Abstract:  Excision of an epibulbar choristoma followed by amniotic membrane grafting was performed on a 3-month-old baby who presented with nevus sebaceous syndrome. Upon histopathologic examination, the excised mass was seen to be a complex choristoma, a rare ocular tumor. Amniotic membrane transplantation is currently the most acceptable modality of treatment of limbal choristoma, with good cosmetic outcome.

Abstract:   Mastocytosis is a broad term used for a group of disorders characterized by accumulation of mast cells in the skin with or without extracutaneous involvement. The clinical spectrum of the disease varies from only cutaneous lesions to highly aggressive systemic involvement such as mast cell leukemia. Mastocytosis can present from birth to adulthood. In children, mastocytosis is usually benign, and there is a good chance of spontaneous regression at puberty, unlike adult-onset disease, which is generally systemic and more severe. Moreover, individuals with systemic mastocytosis may be at risk of developing hematologic malignancies. We describe a girl who presented to us with a solitary mastocytoma at age 5 and later developed maculopapular cutaneous mastocytosis. At age 23, after an episode of anaphylactic shock, a bone marrow examination revealed mast cell leukemia. She ultimately died despite aggressive chemotherapy and bone marrow transplantation.

Abstract:  We report a large infiltrating atypical granular cell tumor in a child with Noonan syndrome. Even though granular cell tumors are rare in childhood, five cases have been reported in children with Noonan syndrome. This study compares these different cases and explores the possibility of activation of the granular cell by the Ras pathway.

Abstract:  High intraocular pressure is a rare ophthalmic condition associated with infantile hemangiomas that involves the orbit, eyelid, or both. Here, we describe a patient with extensive facial and orbital infantile hemangiomas associated with high intraocular pressure in the affected eye. The prompt management of this challenging condition is essential.

Abstract:  Generalized lichen nitidus is a rare disease, and there are only a few reports associating it with a genetic disorder. Here we report a case of generalized lichen nitidus in Russell-Silver syndrome.

Abstract:  Phototherapy using narrowband ultraviolet B (UVB) is considered among the treatments of choice in vitiligo, even in childhood. The objective of the current study was to evaluate the profile of safety and effectiveness of such therapy in a group of children. An open, uncontrolled study was performed on 28 children with vitiligo who were receiving narrow band UVB phototherapy. The children were classified according to vitiligo type and phototype. Family history and presence of thyroid disease were investigated. Eighteen patients had received other treatments that was stopped 3 months before starting phototherapy. Phototherapy was administered twice a week. The mean duration of therapy was 10 ± 3.4 months. The mean total dose administered was 156.12 ± 79.4 J/cm². Photographs of lesions were taken before and at the end of treatment. The response to phototherapy was expressed as percentage of repigmentation. The data were statistically analyzed using SPSS (SPSS Inc., Chicago, IL). Fourteen percent of patients showed excellent response, 28.6% good response, 25% moderate response, and 28.6% mild response; the remaining 3.5% were not responsive. No side effects were observed except mild erythema requiring a decrease in dosage in a few patients. The results of the present study confirm those of other authors, with better results than adults probably related to good adherence of patients and their families. Nevertheless, high cumulative doses are not necessarily related to a good response. Therefore, we suggest stopping treatment after 6 months in nonresponding cases.

Abstract:  Skin disorders are an important problem in children living in developing countries, but only a few epidemiologic investigations on pediatric dermatoses are available in the literature. Our study is an analysis of the range and frequency of skin diseases presenting to the Italian Dermatological Center in a pediatric Ethiopian population. A retrospective analysis was performed on 17,967 medical records of children aged 0 to 18 years attending the Italian Dermatological Centre in Mekele (Ethiopia) from January 2005 to December 2009. Infections and infestations accounted for 47% of the disorders seen; fungal infections were the most common (44.1%), followed by bacterial and parasitic diseases. Dermatitis constituted the second most common diagnostic category (24.7%) of the disorders seen, and contact dermatitis was the most common diagnosis (48.8%). Pigmentary disorders and disorders of skin appendages were more common in girls, whereas fungal and parasitic infections were more common in boys. Bacterial and parasitic infections were more common in children younger than 1 year old, fungal infections in those aged 1 to 5.9, and disorders of skin appendages and pigmentary disorders in those aged 15 to 18. These findings demonstrate that most of the disorders seen could be easily managed in clinical practice with appropriate skill development. It is crucial to ensure that training of medical students and pediatricians focuses on accurate recognition, diagnosis, and management of these common skin diseases and that families, teachers, health workers, and nurses be educated about the most common signs of prevalent skin diseases to help facilitate appropriate care.

Abstract:  Little is known about the characteristics of cutaneous sinus tract (CST) of dental origin in children. 28 cases of CST of dental origin in children were evaluated. Age, sex, site of skin lesion, duration until diagnosis, teeth involved, etiology, treatment, and outcome were recorded. The last dental care visit was also recorded. The mean age was 10.25 years (range 4–16). The male to female ratio was 1:1.74. The mandibular–submandibular area was the most common site of skin lesions. The mandibular first molar was the most involved tooth, followed by the mandibular incisor. Caries was the most common etiology. The mean duration of lesions until correct diagnosis was 6.5 months (range 0.3–12 mos). The treatment was root canal therapy or extraction. After appropriate dental treatment, CSTs resolve rapidly. Surgical revision of the scars were indicated in eight (29%) patients, to provide better cosmetic results. In these patients, the duration of lesions were longer. Preventive dental care, as indicated by last dental care visit, was poor. CST in children is different from that in adults in terms of sites of skin lesions, duration of lesions, and involved tooth and similar to that in adults in terms of etiology and treatment modality. Early treatment of the dental infection may cause healing of the cutaneous lesion spontaneously, without a scar.

Abstract:  Macrocephaly-capillary malformation (OMIM 602501) is a rare overgrowth and asymmetry syndrome. Cardiac arrhythmias were reported to occur in few patients. We present a case in which fetal arrhythmia was the presenting symptom of the syndrome.

Abstract:  Congenital anonychia is rare, particularly when all 10 toenails are absent. When anonychia is associated with absence of distal phalanges, a diagnosis of Cooks syndrome must be considered. We present a case and discussion of a patient with congenital anonychia, absent distal phalanges, and rudimentary hypoplastic middle phalanges and brachydactyly, consistent with Cooks syndrome.

Abstract:  Pilomatricoma is a benign tumor of the hair matrix cell that presents predominantly in childhood. Although pilomatricoma occurs spontaneously, multiple pilomatricomas have been described in association with several inherited syndromes. We report on a 28-year-old man with Kabuki syndrome with three pilomatricomas in his head and thigh. Although several reports describe multiple pilomatricomas associated with Turner syndrome, there are no reports of multiple pilomatricomas combined with Kabuki syndrome. Ectodermal abnormalities such as hair abnormality and hirsutism are symptoms of Kabuki syndrome, and pilomatricomas are frequently associated with the mutations of beta-catenin in hair follicle development. The predisposition of pilomatricomas may be not merely a coincidental finding, but an added association with Kabuki syndrome.

Abstract:  Dyshidrotic eczema is one of the rare cutaneous adverse effects of intravenous immunoglobulin therapy, usually seen in adults. We herein report the first pediatric case of severe dyshidrotic eczema occurring after intravenous immunoglobulin therapy for Kawasaki syndrome.

Abstract:  A 9-year-old male presented to our dermatology clinic with a recent history of developing numerous cutaneous pilomatricomas, and was subsequently discovered to have sustained a recurrence of his glioblastoma multiforme. Immunohistochemical staining of a representative pilomatricoma and his original brain tumor revealed upregulation and nuclear localization of beta-catenin, a sign associated with poor prognosis in glioblastoma. We hypothesize that the development of multiple pilomatricomas may have been a hallmark of this patient's tumor recurrence and provide support for a recent report of an association between multiple pilomatricomas and gliomatosis cerebri.

Abstract:  There are several reports of the collision of vascular and pigmentary anomalies (e.g., phakomatosis pigmentovascularis) and the association between congenital melanocytic nevi and infantile hemangiomas. We report a case of Spitz nevus arising in skin overlying a congenital plaque-like glomuvenous malformation (GVM). This is the first report of a Spitz nevus arising in direct contiguity to a GVM.

Abstract:  Systemic manifestations of allergic contact dermatitis due to consumption of foods containing balsam of Peru (Myroxylon pereirae)-associated chemicals have recently been reported in children. We present seven children with widespread, recalcitrant dermatitis who experienced 60–80% clearance after initiating a diet low in balsam of Peru, specifically the tomato product ketchup. Furthermore, because we have observed a high prevalence of ketchup in our pediatric patients’ diets, we recommend consideration of moderate consumption of this product in patients with recalcitrant widespread dermatitis.

Abstract:  Onychomadesis, or nail shedding, is rarely seen in children and can be due to stress, systemic illnesses, trauma, and drug therapy or may be idiopathic. Fungal infection of the nail is only rarely reported as a cause of onychomadesis. We present here a case of Trichophyton tonsurans–induced onychomycosis and resultant onychomadesis in a 9-month-old boy with onset of lesions at 2 weeks of life.

Abstract:  The sight of needles and surgical equipment can often cause anxiety in children. We describe the use of “Magic Goggles,” a distraction technique of use in pediatric dermatology procedures.

Abstract:  The aim of this case report was to determine the method and basic principles of immediate tissue expansion using a Foley catheter in the forehead region for treating a medium-sized congenital melanocytic nevus. A 4-year-old child presented with a supraorbitally located forehead congenital melanocytic nevus, close to the midline. Total excision and reconstruction with intraoperatively expanded forehead skin below the hairline was performed. Intraoperative tissue expansion using a Foley catheter allowed us to obtain expanded and enhanced local tissue, which had tissue characteristics similar to those of the forehead skin, and thus closure of a medium-sized defect without distorting important anatomic structures such as the brow and frontal hairline was possible. Of the two methods of tissue expansion, immediate expansion using a Foley catheter is a good alternative in single-stage reconstruction of head and neck defects because it has the advantage of omnidirectional expansion.

Abstract:  Impetigo is a common cutaneous infection that is especially prevalent in children. The prevalence of colonization and infection with resistant strains is continually increasing, forcing clinicians to reevaluate treatment strategies. Newer topical agents are effective in treating infections with resistant strains and may help minimize resistance and adverse effects from systemic agents. Use of topical disinfectants to decrease colonization is an important adjunctive measure. Physicians should be aware of local resistance patterns in impetigo to help guide therapy.

Abstract:  A 7-year-old boy presented with an annular verrucous plaque on the chin of 5 weeks duration. The lesion occurred after a bicycle accident and was unresponsive to antibiotics. Fungal culture grew Sporothrix schenckii. Sporotrichosis should be considered and fungal culture obtained whenever a nodule or plaque fails to respond to initial treatment.

Abstract:  Mutations in the keratinocyte lipid transporter adenosine triphosphate-binding cassette A12 (ABCA12) are known to cause harlequin ichthyosis. More recently, mutations in this gene have been demonstrated to cause other phenotypes within the spectrum of recessive congenital ichthyosis. We report the case of an infant with novel heterozygous mutations in ABCA12 who exhibited features and a clinical course more consistent with congenital ichthyosiform erythroderma than harlequin ichthyosis.

Abstract:  Nevus spilus, also known as speckled lentiginous nevus, is a nevoid disorder characterized by hyperpigmented macules or papules scattered over a background of tan pigmentation. Although nevus spilus is mainly of cosmetic concern, malignant melanoma may rarely develop in the lesions. Although classically not mentioned as a hairy nevus, a few reports in literature mention overlying hypertrichosis in lesions of nevus spilus. We hereby report four cases of nevus spilus without malignant change with overlying terminal hairs that arose mainly from the background pigmented area.

Abstract:  Acute genital ulcers rarely occur in nonsexually active young girls. When present, they can cause significant physical and emotional distress for the patient and her parents, and prompt an evaluation for sexual abuse and sexually transmitted diseases. With this review, we aim to further characterize acute genital ulcers in nonsexually active young girls by reviewing the medical records of patients with this disorder and to offer an approach to the diagnosis, evaluation, and treatment of acute genital ulcers based on our understanding and knowledge of this condition. We retrospectively review our understanding and knowledge of acute genital ulcers in nonsexually active girls at a pediatric hospital. A review of the recent literature on acute genital ulcers and a multidisciplinary approach to the diagnosis, evaluation, and treatment of acute genital ulcers are also presented. Twelve patients presented with acute genital ulcers, 11 of which were hospitalized for evaluation and pain management. Extensive work-up failed to reveal a specific infectious or autoimmune etiology in all but one patient, who was diagnosed with acute mycoplasma pneumonia. Acute genital ulcers in nonsexually active young girls likely represent a form of idiopathic vulvar aphthosis. Evaluation of a first episode of acute genital ulcers with mild prodromal symptoms should be limited. Treatment consists primarily of supportive care and symptom relief.

Abstract:  We present four cases of children less than 2 years of age, seen in four different practices, with a similar, unique, and specific presentation of diaper dermatitis, all while using Pampers Baby Dry with Drymax™ technology. To date, no reported cases exist of allergic contact dermatitis to Pampers Dryweave™ in medical literature.

Abstract:  Congenital anonychia is an inherited autosomal recessive disorder characterized by complete absence of fingernails or toenails, or both. In the present study, we have described a consanguineous Pakistani family having a family member affected with congenital anonychia. Genotyping using polymorphic microsatellite markers showed linkage of the family to gene RSPO4 encoding R-spondin and mapped on human chromosome 20p13. Deoxyribonucleic acid sequence analysis of the gene identified a novel nonsense mutation (c.18C>A; p.Cys6X) in the affected family member.

Abstract:  Blastic plasmacytoid dendritic cell neoplasm is an uncommon malignancy with a high incidence of cutaneous involvement, risk of leukemic dissemination, and poor prognosis. We report a 15-year-old boy with blastic plasmacytoid dendritic cell neoplasm who was treated with acute myeloid leukemia-based polychemotherapy and subsequent allogenic stem cell transplantation.

Abstract:  Complications of severe forms of epidermolysis bullosa (EB) almost invariably lead to chronic malnutrition, jeopardizing immune status, growth, iron status, bone health, wound healing, and quality of life. Although gastrostomy tube (G-tube) feeding has successfully addressed the difficulties of providing nutrition and medications in some children attending our center, others have developed problems such as abdominal distension, poor feed tolerance, and leakage of gastric contents with persistent localized skin ulceration, posing enormous challenges to skin management and nutritional maintenance. Suspicions that G-tube placement and feeding cause or exacerbate these problems has led to a decline in placements at our center over the last 10 years. We therefore recognized that it should not be rejected without due consideration of why some patients seem more prone to complications than others. Thus, information on selected issues and outcomes of G-tube placement was obtained from records of 66 patients undergoing surgery between 1989 and 2008. The complex interrelationships of the sequelae of severe EB, changes in practice over 20 years and lack of data for patients treated early in the series make it impossible to draw firm conclusions at this stage, however, our scrutiny provides valuable information on which to base debate and future studies. It also offers well as useful insights for fellow professionals involved in nutrition support in children with severe EB.

Abstract:  Milia-like idiopathic calcinosis cutis (MICC) is a rare dermopathy, usually occurring in children with Down syndrome. We report a case of an 8-year-old girl with Down syndrome who presented with histologically verified MICC, briefly review the literature on MICC, discuss the differential diagnosis, and describe the use of dermoscopy in the evaluation of lesions, which we believe has never been published previously with this entity.

Abstract:  We report a case of a solitary infantile myofibroma masquerading as an ulcerated infantile hemangioma. Infantile myofibroma is a rare soft tissue tumor that has a good prognosis in the solitary form. It may be difficult to distinguish clinically from more common tumors of infancy such as an infantile hemangioma or from other rare entities and therefore requires a biopsy for definitive diagnosis.

Abstract:  Neonatal lupus erythematosus is an uncommon transplacentally acquired autoimmune disorder. We report a 7-month-old boy with cutaneous involvement of neonatal lupus erythematosus mimicking an extensive capillary malformation.

Abstract:  Hereditary angioedema (HAE) is a rare inherited disease that is often difficult to diagnose. We report a case of a 9-year-old boy with a spontaneous mutation causing HAE, diagnosed after a life-threatening episode of angioedema of the head and upper respiratory tract after a 5-year history of recurrent skin swellings and abdominal pain leading to several hospital admissions. The aim of this report is to direct focus on this rare disease, which can be treated effectively, to diminish morbidity and mortality of children suffering from undiagnosed HAE.

Abstract:  Neonatal purpura fulminans is rare and may be inherited or acquired. It may ultimately lead to multiorgan failure and death. Purpura fulminans in a premature neonate resulting from Staphylococcus aureus septicemia is illustrated. Unfortunately, the baby succumbed to septicemia.

Abstract:  Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a severe variant of pityriasis lichenoides et varioliformis acuta (PLEVA). We report a case of FUMHD in an 11-year-old boy who subsequently developed neurologic symptoms and was found to have necrotizing vasculitis on brain biopsy. Over 5 years of follow-up, he had one biopsy-proven recurrence of his skin lesions and continued rehabilitation and treatment for residual neurologic complications. This case provides histological evidence of central nervous system vasculitis associated with FUMHD. Our patient’s disease was eventually controlled with cyclophosphamide.

Abstract:  Giant congenital blue nevus (GCBN) is rare and usually occurs on the scalp. Malignant blue nevus (MBN) is also rare and has a poor prognosis. We report a case of MBN arising in a GCBN on the back. There have been three previous reports of MBN associated with GCBN on the trunk; our case had the earliest onset of MBN arising in a GCBN.

Abstract:  Branchial cleft anomalies are rare developmental defects of the neck, with an estimated 2% to 3% being bilateral. Although most are isolated findings, some are associated with syndromes. We report a 2-month-old boy with bilateral branchial cleft anomalies, low-set ears, and hydronephrosis who tested positive for a mutation in the TFAP2A gene (A256V) implicated in branchio-oculo-facial (BOF) syndrome. Magnetic resonance imaging (MRI) revealed a thyroglossal duct cyst at the base of the tongue. To our knowledge, this is the first reported case of BOF syndrome presenting concomitantly with a thyroglossal duct cyst.

Abstract:  The term “ectopic nail” refers to nail tissue found in a location other than in the normal nail bed. Here we report a 9-year-old girl with an asymptomatic keratotic “horn” on the tip of her left fifth finger. Present since the age of 1 month, it was misdiagnosed as a common wart and treated using liquid nitrogen cryotherapy without benefit.

Abstract:  Paraneoplastic pemphigus (PNP) is seen most frequently in the setting of Castleman’s disease (CD) in childhood. We report herein a 10-year-old girl with PNP appearing a few weeks after resection of a recurrent CD. Despite improvement in skin and mucosal lesions with prednisolone and azathioprine, she had severe bronchiolitis obliterans and died from respiratory failure a few months later.

Abstract:  Two infants presented with multiple asymptomatic papules and geographic and annular plaques over the extensor aspect of the upper and lower extremities and trunk. Skin biopsy of both lesions showed plugged and hyperplastic dilated acrosryingia and deep dermal ducts, along with granulomatous inflammatory reaction. These lesions showed self-healing with complete resolution. A previous report described similar clinical and histopathologic features and labeled it giant centrifugal miliaria profunda. Because of the large granulomatous plaques and deep infiltrate, we propose that it was a granulomatous variant of giant centrifugal miliaria profunda. We report these cases for their rarity and self-healing nature.

Abstract:  Microgeodic disease, similar to chilblains (pernio), is characterized by painful, erythematous swelling, as well as small, punched-out erosions found in affected phalanges. Although they share a similar appearance and proposed pathogenesis, chilblains has rarely been diagnosed in children with microgeodic disease, and in those cases has not been confirmed on skin biopsy. This article details a child in Connecticut diagnosed during the winter with chilblains and microgeodic disease of his toe, as supported by biopsy and imaging. These findings further indicate that the two diseases are interrelated, imply a similar pathogenesis, and lead us to suggest similar treatment for problematic forms of both diseases.

Abstract:  Despite aggressive sun protection, most individuals with xeroderma pigmentosum (XP) develop cutaneous neoplasia, including actinic keratoses. We describe the case of a 16-year-old girl with XP type C treated safely with photodynamic therapy (PDT). Although there is little if any evidence in the literature supporting the use of aminolevulinic acid PDT in individuals with XP, they may be the ideal candidates for PDT treatment because the profound post-treatment photosensitivity and strict post-therapy sun avoidance necessitated by PDT treatment is already part of the everyday lifestyle of people with XP.

Abstract:  Adaptation of skin barrier function and interleukin-1α (IL-1α) content in diapered and nondiapered skin are poorly characterized in newborns receiving standard skin care. In a monocentric, prospective pilot study 44 healthy, full-term neonates were randomly assigned to skin care with baby wipes (n = 21) or water-moistened washcloth (n = 23) at each diaper change. Transepidermal water loss (TEWL), skin hydration, skin-pH, IL-1α, and epidermal desquamation were measured on days 2, 14, and 28 postpartum. Microbiological colonization was evaluated at baseline and on day 28. Significantly lower TEWL was found on the buttock in the group using baby wipes compared to water. IL-1α and skin hydration significantly increased and pH decreased independent of skin care regimen. IL-1α was significantly higher in diapered skin compared to nondiapered skin. Although skin care with wipes seems to stabilize TEWL better than using water, the skin condition and microbiological colonization were comparable using both cleansing procedures. Increase of epidermal IL-1α may reflect postnatal skin barrier maturation. These data suggest that neither of the two cleansing procedures harms skin barrier maturation within the first four weeks postpartum. Longer observations on larger populations could provide more insight into postnatal skin barrier maturation.

Abstract:  The diagnosis of atypical Spitz tumor (AST) in a pediatric patient conveys an uncertain potential for malignancy. Although pediatric melanoma is rare, AST may be treated aggressively with sentinel lymph node biopsy (SLNB) and subsequent completion lymphadenectomy. These procedures have unclear therapeutic benefit and potential morbidity. We aimed to collect outcomes on children with AST treated with excision alone to assist in the management of these lesions. We queried our institution’s pathology database for AST specimens submitted between 1994 and 2009. A dermatopathologist reviewed pathology slides to confirm AST diagnosis. Clinical information was obtained from medical records, and outcomes surveys were administered to children with AST. Twenty-nine patients met AST diagnostic criteria and were included in this study. Mean age at first excision was 9.0 ± 4.2 (range 2.3–17.5), and 19 patients underwent more than one excision procedure to achieve clear margins. No patient had SLNB. Fourteen patients (48%) with mean follow-up time of 8.4 years (range 3.5–15.8) completed clinical outcomes surveys. Outcomes with mean follow-up time of 2.8 years (range 0.02–8.1 years) were obtained for 10 additional patients from medical records. There were no reports of recurrence, additional lesions, or metastases in these 24 patients. We report one of the largest series of children with AST treated using excision alone and who remain disease free after a significant follow-up period. Our data suggest that SLNB is not warranted in the routine management of pediatric AST. We recommend complete excision with clear margins and careful clinical follow-up.

Abstract:  Treatment of infantile acute lymphocytic leukemia (ALL) may cause failure to thrive and hypogonadism due to hypopituitarism induced by chemotherapy and whole-brain radiotherapy. We report the case of a 22-year-old girl with a genetic predisposition to pattern hair loss who developed inveterate diffuse alopecia. The patient had onset of ALL at 8 years old and underwent bone marrow transplantation (BMT). Diffuse alopecia gradually advanced over her whole body. Her vellus scalp hair gradually came out, and hair loss progressed again at 8 years, after BMT. She later developed iatrogenic failure of secretion of estrogen and was treated with estrogen substitution therapy for 14 months from the age of 20. There was a small increase in the volume of hair during therapy, but alopecia returned to the former level after the therapy was suspended. Histopathologic examinations of the scalp performed during estrogen substitution therapy and 2 years after suspension of the therapy showed a 60% decrease in the number of hair follicles and prominent development of vellus hair. We conclude that estrogen influenced hair growth in the context of a genetic predisposition for pattern hair loss in this case.

Abstract:  Congenital multiple clustered dermatofibroma (MCDF) is a rare, idiopathic, benign tumor presenting at birth as an asymptomatic hyperpigmented patch that is stable until puberty, at which time it enlarges and develops papules. Ultimately, MCDF appears to follow a stable, benign course. We present a case of a 12-year-old girl with congenital MCDF. To our knowledge, this is only the third reported case of congenital presentation of MCDF and the only case featuring atrophoderma-like depression.

Abstract:  In resource-poor settings, scabies is associated with considerable morbidity. Which factors determine morbidity and how rapidly it recedes after specific treatment is not known. Patients with scabies were recruited in three urban slums in Fortaleza, Northeast Brazil. Diagnosis was established according to dermatoscopy, skin scraping, or adhesive film test. Severity of scabies-associated morbidity was assessed semiquantitatively. Patients and close contacts were treated with oral ivermectin (200 μg/kg, repeated after 7 days) and followed up for 2 weeks. Ninety-five patients were included in the study. Papules were the most common lesion type (98.9%). Excoriations due to scratching were observed in 43.2% and bacterial superinfection in 24.2%. Predilection sites were the arms (82.1%) and the abdomen (81.1%). At baseline, 36.3% of patients complained about intense or severe itching. Intense or severe itch decreased to 6.3% 2 weeks after treatment (p = 0.02). Whereas 37.5% of the patients complained about intense or severe itch-related sleep disturbances at baseline, only 8.8% reported the symptom 2 weeks after treatment (p = 0.35). At baseline, the degree of itching was correlated with the degree of sleep disturbance (ρ = 0.64; p < 0.001). One week after the first dose of ivermectin, the intensity of itching and of sleep disturbance decreased significantly (p < 0.001). In patients living in resource-poor setting, scabies was associated with considerable morbidity. Treatment with ivermectin rapidly reconstituted health in almost all cases.

Abstract:  Congenital erosive and vesicular dermatosis is a rare syndrome first described by Cohen et al in 1985. Most of the 18 cases published have been reported in premature newborns. Affected babies typically present with erosions and vesicles that tend to heal shortly after birth with reticulated scaring. We report an additional case, followed up for 5 years, in which we excluded a pathogenic mutation in the TP63 gene.

Abstract:  Ammonium bifluoride is one of the most corrosive acids that may produce severe chemical burns when in contact with skin. This hazardous chemical is widely used in household products. We report two pediatric cases of irritant contact dermatitis after exposure to a rust remover, which contained ammonium bifluoride.

Abstract:  Basal cell carcinoma is the most common tumor of skin. Its incidence increases with age, although it is relatively infrequent under the age of 50 years, and its occurrence in childhood is exceedingly rare. This article describes an otherwise typical basal cell carcinoma arising in young patient, with emphasis on histologic diagnosis.

Abstract:  Dermatomyositis is an autoimmune inflammatory myopathy characterized by unique cutaneous features. Gottron’s papules are pathognomonic, lichenoid papules that can be found overlying the joints of the dorsal hand. Papules on the palms of the hand are less commonly seen, especially in the pediatric age group. Recognition of these inverse Gottron’s papules as a sign of dermatomyositis is important as they may be the only cutaneous feature and may be a clue of underlying interstitial lung disease.

Abstract:  Lipodystrophia centrifugalis abdominalis infantilis is a localized lipodystrophic disease affecting young children. A total of 168 cases were collected from dermatology literature. Where possible, details of the data were also obtained from the authors. Most of the patients were Japanese, Korean, or Chinese in origin. The Caucasian patients were from England, Italy, France, Germany, Spain, and the United States. The male-to-female ratio was 1:1.6. The depressed lesions developed during the first 4 years of life in the groin or axilla, surrounded by a slight erythematous change. Histologically, subcutaneous fatty tissue was lost or markedly decreased in the depressed areas, and marked or moderate mononuclear cell infiltration was observed in the fatty tissue of the surrounding erythematous area. The depressed lesions extended centrifugally to involve the abdominal or chest walls. However, in most cases, such enlargement spontaneously ceased by the age of 13 years. No drugs used were effective in preventing the enlargement of depressed lesions, but more than 60% of patients showed spontaneous improvement after the cessation of enlargement. Lipodystrophia centrifugalis abdominalis infantilis is a clinically and histologically distinct lipodystrophic disease affecting young children, mostly in Asian countries, but a few in Western countries.

Abstract:  Patients with atopic dermatitis (AD) are predisposed to infection with Staphylococcus aureus, which worsens their skin disease; it has been postulated that the lack of antimicrobial peptides due to aberrant allergic inflammation in skin with AD could mediate this enhanced bacterial susceptibility. We sought to characterize the amounts of S. aureus and biological products found in infected AD lesions and whether treatment with topical corticosteroids and oral cephalexin as the only antimicrobial improved outcomes. Fifty-nine children with clinically and S. aureus–positive impetiginized lesions of AD were enrolled in this study. A lesion was graded clinically using the Eczema Area and Severity Index, and wash fluid was obtained from the lesion for quantitative bacterial culture and antibiotic sensitivities and measurement of bacterial products and cytokines. Subjects were re-evaluated 2 weeks after treatment. Improvement in the clinical and inflammatory characteristics of impetiginized lesions were noted, even in the 15% of lesions infected with Methicillin-resistant S. aureus (MRSA). In a subgroup of subjects whose lesions did not contain S. aureus 2 weeks after initiating treatment, beta-defensin levels were higher at both visits than in normal skin. Treatment of uncomplicated impetiginized pediatric AD with topical corticosteroids and cephalexin results in significant clinical improvement, even in subjects infected with MRSA. We propose that the inhibition of abnormal inflammation by the treatment regimen, resulting in the high levels of defensins, is involved in the improvement of AD and that systemic antibiotics do not appear to be necessary in secondary impetiginized AD.

Abstract:  Wells’ syndrome, otherwise known as eosinophilic cellulitis, is a rare dermatosis seen more commonly in adults than in children. In this article, we present a 5-year-old Caucasian boy who initially presented with pruritic, erythematous macules and papules evolving to bullae formation. Subsequent histology confirmed diagnosis of Wells’ syndrome, and additional blood work also demonstrated positive immunoglobulin (Ig)M and IgG for parvovirus. In many instances, the direct etiology of Wells’ syndrome is unclear, but the link between parvoviral infection and development of Wells’ syndrome could further support an additional cause.

Abstract:  A 3-year-old boy presented with asymptomatic elastomas on the posterior trunk. Radiographic studies revealed osteopoikilosis, confirming the diagnosis of Buschke-Ollendorff syndrome. The patient had a history of bilateral simple cutaneous syndactyly, which has not been previously reported with this condition. Buschke-Ollendorff syndrome is a rare autosomal-dominant disorder characterized by connective tissue nevi and osteopoikilosis. Several associated systemic abnormalities have been reported, but morbidity and mortality are generally not affected.

Abstract:  “Cauliflower ear” is a perichondritis of the auricular pavilion, usually caused by infection or repeated trauma. In children, this entity is considered infrequent. We present the case of a 10-year-old girl living in a child care institution with “cauliflower ear” that was interpreted as a possible sign of child abuse.

Abstract:  Lipodystrophia centrifugalis abdominalis infantilis (LCAI) is a rare lipodystrophic disorder mainly affecting Eastern Asian children. We report the clinicopathologic findings of four Taiwanese children with LCAI in which local induration or ecchymotic changes preceded the skin depressions on the groin or abdomen.

Abstract:  Eruptive vellus hairs cysts are benign papules consisting of small cysts containing multiple vellus hairs. An eruption commonly develops on the chest and sometimes the upper extremities. These papules are asymptomatic or rarely pruritic. Only a few cases of familial association have been described. We report the development of eruptive vellus hair cysts on the trunk of an 8- and 12-year-old sister and brother.

Abstract:  A 19-month-old boy was evaluated for a skin eruption after recent vaccinations. Clinical and histopathologic findings supported a diagnosis of Gianotti–Crosti syndrome (GCS). This case report examines the link between GCS and vaccinations, particularly the diphtheria, tetanus, and pertussis vaccine and the varicella virus live vaccine.

Abstract:  Keratosis pilaris (KP) is a frequent and benign condition in children characterized by the presence of rough, follicular papules and varying degrees of erythema. Different variants have been described, including simple KP and red KP. Between September 2007 and October 2010, 11 children with profuse and precocious KP seen at the department of pediatric dermatology were included. They defined an underemphasized clinical variant of childhood KP: the papular, profuse, and precocious KP characterized by early age of onset (<18 mos), extensive involvement of the limbs and cheeks, and papular nature of lesions. No clinical association has been found. The main complication was episodes of folliculitis. Diagnosis was delayed for all patients. Treatment is difficult, but association between emollient and keratolytic agents can provide some help.

Abstract:  The nonselective beta-adrenergic receptor antagonist propranolol is an effective therapy for infantile hemangiomas. Systemic propranolol therapy shows a rapid therapeutic effect with good drug tolerability. We report on the efficacy of local application of propranolol ointment in superficial hemangiomas of the skin. In our outpatient department, 45 children with 65 hemangiomas were treated with 1% propranolol in a hydrophilic ointment topically applied twice a day. Before start of treatment and at each visit, clinical photographs were taken. If ultrasound did not confirm occult deeper components, children were included in the study. Treatment in the proliferative phase within the first 6 months of life (including seven preterm infants) induced regression in 59% and cessation of growth in 26% of the hemangiomas. No response or proliferation of subcutaneous components was observed in 15%. Clinically, no side effects caused by the beta-receptor blocker were noticed. Treatment of two ulcerated hemangiomas of the perineal region twice using a flash lamp pulsed-dye laser and propranolol ointment in the surrounding lesion led to healing of the ulcers in 3 and 6 weeks, respectively. In six patients, topical therapy was started between the ages of 7 and 33 months. Even in these hemangiomas, improvement was obvious after 2 or 3 months. Propranolol administered topically in 1% ointment could have a beneficial effect on superficial hemangiomas of the skin. The treatment was well tolerated without side effects even in preterm infants and in children with numerous or large lesions.

Abstract:  We describe a case of multiple, discrete, hypopigmented macules in the suprapubic and axillary region in a healthy 3-year-old girl. The lesions first appeared at approximately 9 months of age and increased in number over time. Initial histopathologic examination by an outside dermatopathologist at 1 year of age was reported as showing nonspecific histologic changes. A repeat biopsy at 3 years of age showed large intraepidermal clear cells that expressed CKAE1/CAM5.2, CK7, and BRST2. These findings are diagnostic for clear-cell papulosis, a rare condition that primarily affects children. Without great clinical and pathologic suspicion, this is a diagnosis that can often be overlooked because the histologic findings are virtually identical to those of normal skin.

Abstract:  Subcutaneous panniculitis-like T-cell lymphoma is an uncommon form of CD8-positive cytotoxic T-cell lymphoma of the skin that predominantly affects the subcutaneous tissue and is extremely rare in early childhood (<3 yrs). Here, we present an early pediatric case with an indolent form of subcutaneous panniculitis-like T-cell lymphoma occurring at 12 months old. The subcutaneous nodules gradually disappeared spontaneously, and the girl showed excellent prognosis with no aggressive treatment.

Abstract:  Patterned transient pigmentation along lines of creases occurring in a newborn is rare. A case report of this entity is described, along with a review of all six cases described in the literature so far.

Abstract:  Periorificial dermatitis is a common acneiform eruption that is thought to represent a variant of rosacea. These patients typically present with uniform erythematous papules most commonly located periorally but also occurring around the nose and eyelids. This eruption has been linked to topical corticosteroid use, with only a few reports in the literature associated with systemic steroid use in adults. Here, we report two cases of periorificial dermatitis occurring after systemic corticosteroid use in children.

Abstract:  Hereditary mucoepithelial dysplasia (HMD) is a rare genodermatosis characterized by nonscarring alopecia, fiery red gums, perineal erythema, and visual impairment. Histologically, dyskeratotic keratinocytes and a small number of desmosomes are the hallmark of the disease. We report on two unrelated patients who presented with typical clinical and histologic features of HMD along with other unreported clinical findings.

Abstract:  SCALP syndrome is an acronym describing the coincidence of sebaceous nevus syndrome, central nervous system malformations, aplasia cutis congenita, limbal dermoid, and pigmented nevus (giant congenital melanocytic nevus). We present a fourth case of this syndrome.

Abstract:  The aim of this study was to investigate the prevalence of Pediculus humanus capitis and the coexistence of intestinal parasites in boarding primary schools in Sivas, Turkey. Seven hundred seventy-two students (350 [45.3%] girls, 422 [54.7%] boys) were evaluated with combing for the presence of head lice, collection of fecal samples, and examination of the perianal region for intestinal parasites using the cellophane tape method. The overall infestation rate for head lice was 6% (n = 46). Nine children had evidence of nits only (1.2%), whereas living lice and nits or eggs were found in 37 children (4.8%). Girls were significantly more commonly infested (12.9%) than boys (0.2%). Of the parameters evaluated, socioeconomic level, number of rooms per family, and size and weight of the children were statistically significantly different between the children with and without lice. Although the infestation rate of children with intestinal parasites was higher in the head louse-infested group (23.9%) than in the group of children without lice (17.6%), the differences were not statistically significant.

Abstract:  Severe cases of atopic dermatitis (AD) may require systemic immunosuppression to achieve disease control. Unfortunately, some cases continue to be refractory to management or develop unacceptable adverse effects. There are limited reports of the use of intravenous immunoglobulin (IVIg) in the treatment of severe AD, but results are inconsistent. In a retrospective study, we report 10 children with severe AD refractory to systemic immunosuppression and maximal topical therapy who were treated using IVIg. The children received monthly IVIg for an average of 24 months. This resulted in a significant improvement in symptoms, with fewer infection-related exacerbations and hospitalizations, allowing systemic immunosuppression to be tapered. The effect was associated with a significant decrease in serum immunoglobulin E and was sustained after cessation of IVIg in 50% of cases. No significant side effects attributable to the IVIg infusions were noted. In this cohort of children with severe AD and recurrent cutaneous infections, IVIg provided an effective treatment with minimal side effects and significant benefits in school attendance and quality of life.

Abstract:  Subcutaneous granuloma annulare (SGA) is a rarely reported subtype of granuloma annulare that occurs almost exclusively in children. The etiology of these lesions is unknown, although a possible relationship to insulin-dependent diabetes mellitus (IDDM) has been proposed in the literature. Here we present an unusual case of SGA on the right forearm in a 3-year-old girl. Unlike the typical lesions noted on the extremities, on magnetic resonance imaging her lesions involved the subcutaneous tissue and adjacent muscles in multiple locations. Histopathologically, the case was consistent with SGA but was unusual in its distribution involving multiple muscles, a finding that has not been previously reported. Weeks after incisional biopsy, she was readmitted with diabetic ketoacidosis (DKA) secondary to IDDM. After treatment of her DKA and control of her glycemia, the forearm SGA vanished, which supported the pathologic diagnosis and alleviated our concerns secondary to the unusual distribution.

Abstract:  Porokeratosis is a rare disorder of epidermal keratinization that is regarded as a precancerous. Recipients of hematopoietic stem cell transplantation (HSCT) have a greater risk of skin cancer; chronic graft versus host disease (GVHD) is an additional risk factor. A 16-year-old boy who had received HSCT for acute myelogenous leukemia was referred to us for sclerodermoid chronic cutaneous GVHD. Two years later, he developed disseminated porokeratosis with a few atypical lesions. Despite cryotherapy, numerous lesions of porokeratosis recurred rapidly. Acitretin resulted in good clinical response and reduced the rate of onset of new lesions.

Abstract:  We present two pediatric patients with pityriasis lichenoides et varioliformis acuta fulminans whose admitting diagnosis was Stevens Johnson Syndrome. The patients were successfully treated with methotrexate and prednisone. These cases highlight the importance of early recognition and treatment of this disease to prevent further morbidity and a potentially fatal prognosis.

Abstract:  We report a case of ulcerated lupus vulgaris occurring in 1.5-year-old boy at the Bacille Calmette-Guérin vaccination site within 6 months, which was diagnosed using histology and polymerase chain reaction. The lesion resolved with isoniazid and rifampicin therapy.

Abstract:  The objective of the study was to catalog hair shaft abnormalities in individuals with ectodermal dysplasia (ED) syndromes using light microscopy and to compare findings with those in unaffected controls. Light microscopy was performed in a nonblinded manner on hair shafts from 65 participants with seven types of ED (hypohidrotic ED, ED–ectrodactyly–cleft lip or palate, ankyloblepharon–ectodermal defects–cleft lip and palate, Clouston syndrome, Goltz syndrome, Schopf-Schulz Passarge syndrome, and oculodentodigital dysplasia) and 41 unaffected controls. Hair donations were collected at the 28th Annual National Family Conference held by the National Foundation for Ectodermal Dysplasia. Control participants were recruited from a private dermatology practice and an academic children’s hospital outpatient dermatology clinic. Sixty-five affected participants and 41 unaffected controls were included in the analysis. We assessed the hair shafts of ED and control participants for abnormalities visible using LM. Light microscopy identified various pathologic hair shaft abnormalities in each type of ED, although none of the findings were statistically significantly different from those of the control group. Light microscopy is a poor adjuvant tool in the diagnosis of ED syndromes. Most findings are nonspecific and not sufficiently sensitive.

Abstract:  Erythema dyschromicum perstans (EDP) is asymptomatic slate-gray to blue-brown macules. Idiopathic eruptive macular pigmentation is asymptomatic brown nonconfluent macules. We describe electron microscopic studies of a 9-year-old Japanese girl with EDP. The ultrastructural figures indicated that the production of immature, small, irregular-shaped melanosomes in melanocytes and peripheral localization of melanosomes in keratinocytes caused the clinical appearance of EDP. The ultrastructural evidence distinguishes EDP from idiopathic eruptive macular pigmentation and suggests a distinct pathogenesis of the disease.

Abstract:  Melanotic neuroectodermal tumor of infancy (MNTI) is an uncommon lesion with remarkably consistent histopathologic features that arises primarily in the pediatric population. We describe a MNTI arising in the anterior maxilla of a 6-month-old boy.

Abstract:  We report three infants who developed agminated pyogenic granulomas over congenital vascular malformations, all of which had an aggressive growth pattern. There were no precipitating events such as laser therapy or surgery. Lesions were excised.

Abstract:  Acral peeling skin syndrome (APSS) is a rare, autosomal, recessive genodermatosis characterized by painless spontaneous exfoliation of the skin of the hands and feet at a subcorneal or intracorneal level. It usually presents at birth or appears later in childhood or early adulthood. Some cases result from mutations in the TGM5 gene that encodes transglutaminase 5, which has an important role in cross-linking cornified cell envelope proteins. We report a new APSS pedigree from Jordan that contains at least 10 affected family members, although sequencing of the TGM5 gene failed to disclose any pathogenic mutation(s). On the basis of probable consanguinity, we performed homozygosity mapping and identified areas of homozygosity on chromosomes 1, 6, 10, 13, and 16, although none of the intervals contained genes of clear relevance to cornification. APSS is a clinically and genetically heterogeneous disorder, and this Jordanian pedigree underscores the likelihood of still further heterogeneity.

Abstract:  Rat bite fever is a rare but potentially fatal Gram-negative infection that predominantly affects populations with exposure to rats, notably children. The clinical presentation is nonspecific and requires a high threshold of suspicion to elicit a history of rat exposure. We report here a case of a child whose diagnosis was made retrospectively but with good outcome.

Abstract:  Atypical presentations of molluscum contagiosum require histophathologic examination and may show pleomorphic lymphocytic infiltrates of a reactive nature, mimicking cutaneous lymphoproliferative diseases. Serial sections of specimens or polymerase chain reactions to show T-cell receptor clonality may be helpful for differential diagnosis. We report a case of atypical molluscum contagiosum accompanied by atypical lymphocytic infiltration showing CD30 positivity.

Abstract:  Becker’s nevus otherwise known as Becker’s pigmented hairy nevus, is a common melanotic epidermal hypermelanosis. It usually presents as a large unilateral patch of hyperpigmentation and hypertrichosis on the shoulder, chest, or back of an adult man. It can be bilateral or multiple and affect areas other than the usual sites. We report a 16-year-old boy with two Becker’s nevi on the left side of the face in a segmental distribution with extension onto the oral mucosa. This case is being reported for its rare presentation.

Abstract:  Hematuria is defined as the abnormal presence of red blood cells in the urine and is commonly divided into gross and microscopic hematuria. Terminal hematuria occurs at the end of the urine stream and may have a prostatic, bladder, or trigonal cause. Here, we present a 16-year-old boy who developed terminal hematuria after 1 month of treatment with isotretinoin for acne vulgaris. This side effect of isotretinoin is not reported in the literature. This is the first case report.

Abstract:  Lupus erythematosus panniculitis (LEP) is a rare finding in children, with only 12 fully reported prior cases in the English literature. We describe three cases of LEP in children younger than 18 and compare them to previous cases reported in the literature. We examine laboratory tests performed, biopsy results, age at onset and diagnosis, presence or absence of systemic symptoms, and outcomes after treatment. It is unknown what the risk is of these patients developing future systemic lupus erythematosus. We also discuss the relevance of subcutaneous panniculitis-like T-cell lymphoma, because the clinical and pathologic pictures are similar in presentation.

Abstract:  We performed a prospective study of 1,000 neonates investigated in the first 72 hours of life in the health area of Ferrol (northwest of Spain) to assess the prevalence of erythema toxicum neonatorum, the anatomical sites most frequently involved, the influence of different maternal and neonatal parameters, day of life of medical examination, and type of delivery (vaginal or Cesarean). Overall prevalence of erythema toxicum neonatorum was 16.7%. Lesions most frequently involved the trunk, buttocks, and proximal areas of limbs. A higher prevalence of this dermatosis was found in Caucasian newborns (p = 0.01) and those with higher birthweight (p < 0.05), greater gestational age (p < 0.05), vaginal delivery (p < 0.05), maternal age of <30 years (p = 0.28), and fewer than two previous pregnancies (p = 0.12).

Abstract:  Aquagenic wrinkling of the palms (AWP) is a condition characterized by excessive wrinkling, palmar edema, and whitish papules accompanied by pain, pruritus, or discomfort after brief immersion of the hands in water. It is well documented to be associated with cystic fibrosis (CF), with several theories regarding the pathogenesis having been proposed. We report a case of two sisters with AWP in whom CF has not been diagnosed and review the literature on AWP and its association with CF and CF carrier status. Because diagnosis of mild forms CF or knowledge of an underlying CF genetic mutation is frequently unknown, identification of AWP may represent the only sign of such mutations. The dermatologist plays an integral role in early detection of AWP, and the importance of genetic testing in such patients cannot be overlooked. We recommend various measures to apply in clinical practice to ensure diagnosis and decrease morbidity and mortality in patients.

Abstract:  Systemic drug-related intertriginous and flexural exanthema (SDRIFE), also known as Baboon syndrome, is an uncommon, cutaneous reaction that occurs after the systemic administration of drug-related allergens. We report the case of a 5-year-old boy with SDRIFE after systemic administration of amoxicillin-clavulanate.

Abstract:  Montelukast was effective in treating refractory abdominal and urinary symptoms in a child with systemic mastocytosis.

Abstract:  Melanonychia is a black, tan, or brown streak within the nail plate subsequent to activation of melanocytes in the nail matrix. We present a case of a Haitian girl who presented with transverse melanonychia involving all 10 fingernails in the setting of hyperthyroidism and acute liver injury. Melanonychia has been described only one time in the literature in the setting of hyperthyroidism though this patient also underwent radium treatment which could have led to nail changes.

Abstract:  Chemotherapy used in the treatment of malignancies produces multiple mucocutaneous adverse reactions that may be clinically challenging. These mucocutaneous reactions are common and sometimes not diagnosed. The objective of this study was to determine the clinical patterns of the mucocutaneous manifestations during and after chemotherapy in children with a hematologic malignancy and to determine whether nutritional status influences the clinical presentation. We recruited patients aged 6 months to 16 years diagnosed with leukemia and lymphoma from a pediatric hematology outpatient clinic between November 2008 and May 2010. The patients were divided into two groups: Group 1, recently diagnosed patients, included in the study before receiving chemotherapy, and Group 2, patients in surveillance who had not had chemotherapy for at least 3 months. A dermatologic examination was performed, and biopsy and mycological and bacteriological tests were conducted if necessary, with 6 months of follow-up. We evaluated 89 patients and included 65 in the study: 40 boys and 25 girls with an average age of 8.3 years. All patients had skin lesions at some time during their baseline assessment or follow-up. The manifestations found were anagen effluvium, xerosis, and acral hyperpigmentation. To our knowledge, this is the first comparative study of skin manifestations associated with chemotherapy in a Mexican pediatric population. The mucocutaneous manifestations associated with chemotherapy are important causes of morbidity. All of the children in our study had skin lesions on assessment. We did not find an association between skin manifestations and nutritional status.

Abstract:  Diagnosis of tinea capitis (TC) can be challenging for dermatologists, especially in noninflammatory TC caused by anthropophilic dermatophytes and in black patients, in whom erythema of the scalp is difficult to appreciate. The finding of a typical TC dermoscopic pattern may lead more quickly to a correct diagnosis.

Abstract:  Sweet’s syndrome (SS) is an uncommon condition characterized by recurrent painful cutaneous inflammatory eruptions. It is rare in childhood and has a broad range of extracutaneous manifestations. We describe a child presenting with SS and postinflammatory elastolysis who subsequently developed aortitis complicated by aortic dilatation requiring surgical intervention. Histologic features of the aorta were consistent with Takayasu arteritis (TA). Our case and previously reported cases of pediatric SS complicated by aortitis all demonstrate striking clinical similarities in that all have been associated with postinflammatory elastolysis of involved skin and aneurysmal dilation of the thoracic aorta. We propose that TA should be considered one of the disease associations of SS when complicated by postinflammatory elastolysis and that early referral for cardiovascular screening be considered in this group of patients.

Abstract:  A healthy 10-year-old girl was referred for evaluation of patchy hair loss on the scalp of longer than 6 months duration. She had been diagnosed and treated for head lice approximately 1 month before onset of the hair loss. Examination of the scalp showed discrete ovoid patches of hair loss at the vertex scalp. A scrape of the area of hair loss was performed, and a nit was visible on microscopic examination. Focal hair loss may represent an atypical manifestation of ongoing pediculosis capitis.

Abstract:  Dermatophytoses are frequent in children, but involvement of the facial skin has peculiar aspects that should be considered a separate entity: tinea faciei. Microsporum canis infection in tinea faciei has not been widely documented. To review cases of tinea faciei due to M. canis in children diagnosed at the Dermatology Clinic, University of Cagliari. Between 1990 and 2009, all children with dermatophyte infections of the facial skin were recruited for the study after parental consent. Diagnosis was made through direct microscopic and cultural examination. Age, sex, clinical form, illness duration, identified dermatophyte, source of infection, and treatment were recorded. Forty-six cases of tinea faciei due to M. canis in children aged 11 months to 15 years (29 male/17 female) were diagnosed. In 42 (91.3%) children, the illness was the result of contact with pets, and 4 (8.7%) cases resulted from contact with children affected by tinea capitis due to M. canis. Clinical manifestations were typical ringworm in 34 (74%) patients, whereas in 12 (26%) cases, atypical forms mimicking atopic dermatitis, impetigo, lupus erythematosus, and periorificial dermatitis were observed. In 18 (39%) cases, involvement of the vellus hair follicle was documented as ectothrix invasion. Topical or systemic antifungal therapy was effective in all patients. Tinea faciei shows a complex spectrum of differential diagnosis and age-related variations with respect to other superficial dermatophytosis. M. canis is the main organism responsible in children residing in Cagliari, capitol city of Sardinia, Italy. Close collaboration with veterinary and educational programs within infant communities are required for adequate prevention.

Abstract:  Vitiligo is an acquired disorder in which the loss of melanocytes results in depigmented patches. Topical tacalcitol, a vitamin D₃ analog, is occasionally used to treat vitiligo. We report a case of a 7-year-old Japanese boy who showed moderate repigmentation and worsened freckles after using topical tacalcitol to treat a vitiligo lesion on and around the left eyelid. Topical tacalcitol might have induced and worsened the freckles, because the changes occurred during treatment. Dermatologists should be aware that topical application of tacalcitol to the cheeks can cause freckling and worsen existing freckles, in predisposed children, as described here.

Abstract:  A 14-year-old girl presented with a 3-week history of mucosal erosions, injected conjunctiva, dehydration, and respiratory distress. She had been treated with intravenous acyclovir for herpes simplex infection with positive herpes simplex virus immunoglobulin M and immunoglobulin G. Physical examination and imaging revealed a large abdominal mass. Incisional biopsy was obtained, and pathology demonstrated angiofollicular hyperplasia with hyalinized germinal centers and Castleman’s syndrome-like features. Based on the mucosal erosions, herpes simplex virus serology and positive herpes simplex virus-1 direct fluorescent antibody, Castleman’s disease secondary to overwhelming herpes simplex virus infection was the initial impression. The poor response to antivirals and subsequent development of a bullous eruption on the hands resulted in dermatology consultation. Skin biopsy was obtained from a bullae and revealed suprabasilar acantholysis with necrosis as well as upper dermal, perivascular, and interface infiltrate of lymphocytes and eosinophils. No viropathic changes were present. Direct immunofluorescence was significant for immunoglobulin G deposition intercellularly and along the dermoepidermal junction and focal trace C3 deposition along the dermoepidermal junction consistent with paraneoplastic pemphigus, later confirmed by indirect immunofluorescence. We report this case of paraneoplastic pemphigus secondary to Castleman’s syndrome confounded by herpes simplex virus-1 positive mucosal erosions.

Abstract:  Bullous pemphigoid is an autoimmune blistering disorder rarely seen in the pediatric population. We report a case of trauma-associated bullous pemphigoid presenting in a 15-year-old boy with Crohn’s disease while on immunosuppressive therapy.

Abstract:  Blue rubber bleb nevus syndrome, or Bean syndrome, is a rare sporadic disorder of venous malformations of the skin and internal viscera. We report a rare case of blue rubber bleb nevus syndrome with gastrointestinal and central nervous system involvement.

Abstract:  Genital herpes simplex virus (HSV) is a sexually transmitted infection that can be transmitted from mother to child in utero, perinatally, or postnatally. Cutaneous infection with HSV commonly presents as vesicles affecting the skin, eyes, or mouth. In our case, we report a well child with cutaneous hypopigmented patches at birth that preceded typical blistering.

Abstract:  PHACE syndrome is characterized by posterior fossa malformations (P), large facial hemangiomas (H), arterial anomalies (A), cardiac anomalies or coarctation of aorta (C), and eye anomalies (E) and has striking female predominance. Endocrine abnormalities have recently been described in these patients, involving the thyroid and pituitary glands. We report the case of a 2-year-old girl with the clinical features of PHACE with absence of bilateral internal carotid arteries and isolated growth hormone deficiency.

Abstract:  We describe the case of a 7-year-old boy with urticaria, fever, and arthritis that appeared 10 days after starting cefditoren therapy for acute tonsillopharyngitis, which was diagnosed as a serum sickness-like reaction due to this medication.

Abstract:  Keratosis lichenoides chronica (KLC) is an acquired keratinization disorder that is rare in childhood. We report a case of sporadic pediatric-onset KLC with seborrheic dermatitis-like lesions on the forehead, papules in a retiform pattern in the axillae and mons pubis, and eye and oral mucosal involvement,with additional features of premature canities and a transient photosensitive eruption.

Abstract:  P-tert-butylphenol formaldehyde resin (PTBPFR) is recognized as a significant source of allergic contact dermatitis in adults and children in association with athletic gear, shoes, and neoprene. To our knowledge, this is the first case report of allergic contact dermatitis with PTBPFR associated with padded foam bras.

Abstract:  Keratitis-ichthyosis-deafness (KID) syndrome is a rare ectodermal dysplasia, characterized mainly by the presence of hyperkeratotic skin lesions, neurosensory hearing loss, and vascularizing keratitis. Most mutations that have been discovered as a cause of KID syndrome are autosomal dominant, found in exon 2 of the Connexin (Cx) 26 gene. A G12R (p.Gly12Arg) is a GJB2 mutation reported in only two patients with KID syndrome to date. This article describes a patient with the G12R mutation and KID syndrome with interesting additional features, which include a porokeratotic eccrine ostial and dermal duct nevus, follicular occlusion triad, and unusual persistent oral mucosal papules. We compare this patient’s phenotype with the only two other patients described with the same (G12R) mutation. The phenotypic heterogeneity of KID syndrome, inexplicable according to our current understanding of these proteins, speaks to the complexity of the connexin system and its overlapping expression patterns in different tissues.

Abstract:  This report describes a novel missense mutation in the interferon regulation factor 6 (IRF6) gene associated to facial asymmetry. This new feature widens the phenotype spectrum of Van der Woude syndrome (VWS).

Abstract:  A 6-year-old Hispanic boy presented to the ophthalmology department with complaints of pain, photophobia, and blurry vision of both eyes. He was found to have bilateral granulomatous panuveitis, cataracts, and high intraocular pressures. He later developed multiple asymptomatic, ovoid, hypopigmented patches over the mid-lumbosacral back. Biopsy of lesional skin was significant for low melanocyte counts and a mild lymphocytic infiltrate. The patient was diagnosed with Vogt–Koyanagi–Harada syndrome (VKH). This article reviews the literature regarding the cutaneous presentation of VKH.

Abstract:  Tinea faciei (TF) is a common clinical form of tinea in children that is frequently misdiagnosed and treated with corticosteroids. No large case series of TF focusing on children have been published. The aim of this study was to analyze the main epidemiologic, clinical, and microbiologic features of TF in children over a period of 30 years and compare these features with those of other tineas. We undertook a retrospective study of 818 cases of tinea in children at a referral hospital in southern Spain, diagnosed between 1977 and 2006, concentrating for this study on TF. Of the 73 cases of TF diagnosed, 50.7% were in girls. Most children (46.6%) were 4 to 9 years old. At the time of diagnosis, 29.2% of the cases had been treated with topical steroids. The most frequently isolated dermatophyte was Trichophyton mentagrophytes, which was isolated significantly more frequently in TF than in the other tineas. Cases of TF in children were not extremely unusual, emphasizing that TF must be considered in children with inflammatory facial eruptions. This consideration and the more-frequent use of mycologic tests can help achieve the correct diagnosis, when present.

Abstract:  Although anti-tumor necrosis factor alpha (TNF-α) agents are commonly used to treat psoriasis and other inflammatory diseases in adults and children, numerous reports have documented new-onset or flaring psoriasis in adults treated for the other conditions. Individual case reports have documented similar observations in three children. We report a series of anti-TNF-α-induced psoriasis in children with juvenile idiopathic arthritis or inflammatory bowel disease treated at a large children’s hospital. All five patients presented with severe scalp involvement. One child was treated with adalimumab for juvenile idiopathic arthritis, and four received infliximab for inflammatory bowel disease. The five patients developed psoriasis 2 to 10 months after initiating anti-TNF-α therapy. They presented with erythematous, scaly, crusted scalp lesions. Three of the five patients were initially treated with griseofulvin for presumed tinea capitis. The anti-TNF-α agent was discontinued at the time of diagnosis in two cases. Topical steroids were the mainstay of psoriasis therapy, with improvement in four of five patients. Anti-TNF-α agents have been associated with the onset or worsening of psoriasis in adults, but this has rarely been reported in children. We describe five pediatric cases of anti-TNF-α-induced psoriasis presenting with severe scalp involvement and review their subsequent management. We hope that clinicians caring for patients receiving anti-TNF-α agents will consider psoriasis from the onset of cutaneous symptoms and institute appropriate therapy or referral.

Abstract:  Lindane is an organochloride pesticide, first introduced as a scabicide for human use in the 1950s (1). Because of its low cost and efficacy, it quickly became a first-line treatment for scabies and head lice, but not long after its use became widespread, its safety was called into question, primarily regarding its neurotoxic effects. We intend to show through review of the literature and databases that lindane has been associated with numerous severe and fatal adverse reactions. This review will summarize 67 cases of adverse reactions and deaths associated with the medical use of lindane. Many of the most serious events and fatalities occurred in pediatric and geriatric populations. We concede that toxicity frequently arises from misuse, but labeled usage of lindane caused 43% of the serious adverse reactions of this review. Moreover, the numerous instances of lindane misuse elucidate the importance of clear labeling and instructions, especially with a product with a narrow therapeutic index. Therefore, this report attempts to elucidate the dangers of lindane as a medical treatment for scabies and head lice by providing a comprehensive review of all documented adverse drug reactions since its introduction.

Abstract:  We present two case reports of CH in which severe bleeding episodes occurred during the first weeks of life and report the use of topical tranexamic acid to control bleeding in this setting. Patient 1 was a full-term female infant who presented at birth with a large 7- by 6-cm CH of the forehead showing a few millimeter-sized crusts. No active treatment except close follow-up was advised. At 10 weeks of age, the size of the lesion having spontaneously decreased more than 50%, she presented with severe bleeding from a small crusted area. The bleeding was controlled using topical tranexamic acid, and except for a few minor, easily controlled bleeding episodes in the following weeks, the lesion regressed more than 80%. Patient 2 was a full-term male infant seen at 1 day of life for a 14- by 10-cm CH of the right knee with a few small, dark, superficial crusts. At 3 weeks of age, he was hospitalized after severe bleeding from one of the crusted areas, with a drop in hemoglobin from 131 to 114 g/L. Bleeding was controlled using topical tranexamic acid, and compressive dressing. Because the lesion was clinically a rapidly involuting CH, there was no need for embolization or surgery. The presence of crusting in CH, even in the absence of frank ulceration, is an ominous sign and can precede serious bleeding. Tranexamic acid, an antifibrinolytic agent that helps stabilize the clot, has proved useful topically in controlling bleeding in CH.

Abstract:  Progressive macular hypomelanosis (PMH) is a condition of unknown etiology characterized by asymptomatic, hypopigmented macules located predominantly on the trunk. We recorded 12 adolescents with PMH over a 6-month period. Ten were female, and the mean age was 16.6 years. The average time from the patients first noticing pigment change to diagnosis was 15 months. PMH is probably an underdiagnosed condition.

Abstract:  Two hundred forty-seven healthy newborns were investigated in a prospective cohort descriptive study. Information on phenotype and obstetric and parental history was collected. A positive association was found between erythema toxicum neonatorum and season of birth (spring and summer), whereas parental history of any skin disease was related to a lower frequency of this eruption.

Abstract:  We present an 11-year-old boy with livedo reticularis (LR) secondary to amantadine treatment for his attention deficit hyperactivity disorder (ADHD). We are unaware of previous reports of amantadine-induced LR associated with treatment for ADHD in the pediatric population.

Abstract:  We report a pediatric patient with focal dermal hypoplasia syndrome who developed painful excessive granulation tissue refractory to traditional medical and surgical therapies. Complete response was achieved rapidly with a combination of photodynamic therapy and intralesional steroid injections. The patient has remained in remission for longer than a year.

Abstract:  Miliaria crystallina (MC) is a self-limiting, superficial obstruction of the eccrine sweat ducts with subsequent extravasation of sweat, resulting in rapidly surfacing, tiny, clear, noninflammatory vesicles. The disease is seen frequently in hot, humid, tropical climates and in the neonatal period, but congenital occurrence is rare. We report a case of congenital MC in a baby born to a mother with chorioamnionitis.

Abstract:  We performed a descriptive survey of 1,000 consecutive newborns cared for in the first 3 days of life in the health area of Ferrol (in northwest Spain) to assess the prevalence and most-frequent locations of oral cysts and milia and to study the influence of different maternal and neonatal parameters in the development of these lesions. Prevalence of palatal, gingival, and cutaneous cysts was 53.7%, 13.4%, and 16.6%, respectively. Milia were predominantly located on the cheeks, chin, and forehead. We found a frequent association between palatal and gingival cysts but not between oral cysts and milia. Neonatal factors such as sex, weight at birth, and gestational age can significantly influence the development of palatal and gingival cysts.

Abstract:  A 10-year-old boy with Hunter syndrome and extensive typical skin lesions underwent 9 months of enzyme replacement therapy, after which the skin lesions disappeared. We believe that treatment with idursulfase probably removes the cutaneous storage of glucosaminoglycans in Hunter syndrome.

Abstract:  Poikiloderma with neutropenia (PN), Clericuzio type (OMIM #604173) is a new, unique genodermatosis first described by Clericuzio et al (Am J Med Genet A, 2011, 155, 337) in Navajo Indian population. This disease is characterized by poikiloderma that usually develops in the first year of life and is associated with nail abnormality, palmoplantar hyperkeratosis, chronic neutropenia, and recurrent infections. The rash typically starts from the extremities and spreads centripetally to involve the trunk, face, and ears. Recently, a homozygous mutation in the C16orf57 gene on chromosome 16q13 was identified as a strong candidate as the gene responsible for PN. We report three cases of PN whose clinical presentations, laboratory investigations, and C16orf57 mutation support the diagnosis of PN. One child has developed multiple painful calcinosis cutis lesions. Early-onset poikiloderma should prompt a complete blood count as a screening test.

Abstract:  New ongoing urticarial lesions confined to former injection sites of vaccination and allergy immunotherapy were observed in an 8-year-old boy. This finding can be best explained as an isotopic response phenomenon.

Abstract:  Isotretinoin for acne vulgaris in the setting of chronic granulomatous disease (CGD) is a controversial therapeutic option. In this inherited immunodeficiency, inefficient tissue responses to bacteria and fungi lead to chronic and recurrent infections. Isotretinoin is known to be associated with several mucocutaneous side effects that could worsen the immune response in individuals with CGD. We report the fourth published case of acne vulgaris treated with isotretinoin in an individual with CGD, with a safe and successful outcome.

Abstract:  Epidermolysis bullosa acquisita (EBA) is a rare, acquired, subepidermal blistering disease characterized by autoantibodies directed against type VII collagen, the major component of anchoring fibrils. We report a 5-year-old Chinese boy who presented with extensive lesions consisting of disseminated pruritic vesicles and tense blisters. The diagnosis of EBA was confirmed by histopathology, immunofluorescence, and immunoblotting analysis. The disease was controlled with a combination of prednisone and dapsone.

Abstract:  Malakoplakia is a rare, chronic disease with pleomorphic presentation. It occurs most frequently in immunocompromised patients. It is believed that this entity represents an unusual response to bacterial infection in which macrophages fail to phagocytose them properly. It involves many organs, particularly the urinary and gastrointestinal tracts. It is uncommon on the skin and less common in children. The diagnosis is usually based on histopathologic findings.

Abstract:  Milia en plaque is a rare variant of miliathat occurs spontaneously on an erythematous base without identifiable causative factors. Approximately 40 cases have been recorded in the literature. Most occurred in the periauricular area, affected middle-aged patients, and showed a predilection for women. Here, we report a case of milia en plaque on the bilateral posterior helices in a 6-year-old Chinese boy.

Abstract:  We report the unusual case of a young child who developed an idiopathic basal cell carcinoma (BCC). The patient was otherwise healthy, with no history of excessive sun or radiation exposure or any predisposing factors for nonmelanoma skin cancer. This is a rare case that exemplifies the need for pediatricians and dermatologists to be aware that BCC may occur in children, even if they have no predisposing factors.

Abstract:  Anaplastic large cell lymphoma (ALCL) accounts for 10% to 30% of all childhood lymphomas and approximately 5% of all non-Hodgkin’s lymphoma. ALCL is considered to be a T-cell non-Hodgkin’s lymphoma that can be divided into two major groups with distinct genetic, immunophenotypic, and clinical behaviors. The first group consists of a spectrum of CD30+ T-cell lymphoproliferative disorders that include primary cutaneous ALCL (C-ALCL) and lymphomatoid papulosis. The second group is systemic ALCL (S-ALCL), which is further divided into two subgroups: anaplastic lymphoma kinase positive (ALK+) and ALK-negative. Between 30% and 60% of S-ALCL express ALK, which is usually the result of a t(2;5) translocation that correlates with onset in the first three decades of life, male predominance, and good prognosis. Although morphologically similar, ALK− ALCL shows varied clinical behaviors and immunophenotypes; is commonly seen in older age groups, with a peak incidence in the sixth decade of life with no preference as to sex; and has an overall poorer prognosis. We present a case of CD30+, ALK− S-ALCL in a 7-year-old girl.

Abstract:  Terra firma-forme dermatosis is an idiopathic condition characterized by acquired, dirtlike plaques despite normal hygiene. A diagnosis can be reached by removing lesions with gentle alcohol swabbing. Although Terra firma-forme dermatosis was first described more than 20 years ago and is thought to be not uncommon in clinical practice, it has never been systematically studied. There are few publications about this condition, including no case series of more than six patients. In particular, little is known about the incidence, peak age groups, and most common locations of Terra firma-forme dermatosis. A retrospective review was conducted to identify cases of Terra firma-forme dermatosis in a single-provider practice consisting of 55% pediatric and 45% adult patients. Thirty-one patients with Terra firma-forme dermatosis were identified, including 10 who presented with Terra firma-forme dermatosis as their primary concern. Only two patients were older than 17 years. The median duration of lesions was 4 months. The most common lesion locations were the neck, ankles, and face. Before presenting to the dermatology clinic, three patients had undergone endocrine evaluations, and four had been prescribed topical corticosteroids. Terra firma-forme dermatosis is relatively common and most often occurs in children on the neck or posterior malleolus. This series exemplifies the importance of recognizing Terra firma-forme dermatosis so as to provide rapid relief for patients and avoid unnecessary tests and treatments.

Abstract:  We present a report of three cases with vesicles containing opalescent fluid grouped over the face and scattered on the trunk and limbs. Culture of the fluid aspirated from the vesicles grew Staphylococcus aureus in two of the three cases. The observation of vesicles grouped on the face in a sick febrile child should prompt the diagnosis of a severe staphylococcal septicemia.

Abstract:  We report the case of a 16-year-old Indian girl who presented with multiple small papular lesions and larger erythema multiforme-like plaques in a Christmas tree pattern over her body for 3 weeks. Histopathologic examination was performed, and she was diagnosed with pityriasis rosea presenting with two atypical morphological variants—generalized papular and erythema multiforme-like. A brief review of literature of these two rare types is also presented.

Abstract:  Neurofibromatosis 2 (NF2) is an autosomal-dominant disease caused by genetic mutations of the NF2 gene on chromosome 22. Patients are often diagnosed according to the presence of bilateral vestibular schwannomas and other tumors in the brain and spinal cord. In children, NF2 can present early with ocular findings and cutaneous tumors. We report here a 9-year-old girl who presented with multiple pigmented, slightly tender plaques on her scalp, face, and back that were revealed by histopathology to be plexiform schwannomas. We suspected NF2 and sent the patient’s blood for genetic testing, which confirmed our diagnosis.

Abstract:  Cutaneous mastocytosis in children has an indolent course and undergoes spontaneous regression. Many triggering factors may cause mast cell degranulation and clinical manifestations. Knowledge of these factors is important for patients and their families. We report a case of exacerbation of urticaria pigmentosa due to mast cell degranulation caused by Enterobius vermicularis, which has not been reported before as a triggering factor.

Abstract:  Androgenetic alopecia (AGA) is the most common type of hair loss in adults, but it has been rarely reported in children. We report two cases of AGA in two siblings, aged 6 and 8, whose mother had the same condition, without any association with other skin diseases or abnormalities in endocrinologic examinations.

Abstract:  A 10-year-old boy presented with fever, stiff neck, and rash over the legs. During the course of his hospital stay, the clinical picture gradually evolved, and he met the criteria for Kawasaki disease (KD) on the seventh day of hospitalization. During this period, he also developed alopecia areata. He was managed with intravenous immunoglobulin, aspirin, and intralesional triamcinolone. This is the first case of alopecia areata with KD in the literature, and it does not appear to be a mere coincidence. We discuss the probable mechanisms of alopecia areata with KD, an association that has not been reported before.

Abstract:  Frontonasal dysplasia (FND) is a rare syndrome characterized by malformations of the central portion of the face, especially of the forehead, nose, and philtrum. FND is associated with hypertelorism, a hidden encephalocele, and a cleft of the nose. Occasionally, affected individuals also experience abnormalities of the brain and craniofacial bones. In such cases, a frontal lipoma or calcification of the falx cerebri suggest the existence of a lipoma of the corpus callosum. We present the case of a male newborn with a congenital lipoma 15 mm in size located in the medial frontal line. Magnetic resonance imaging confirmed complete agenesis of the corpus callosum. While there is controversy about the association of frontal lipoma and lipoma of the corpus callosum and FND, we believe our case supports the concept that the changes are due to the same underlying pathogenic mechanism. Therefore, we recommend imaging of the central nervous system in newborns with a congenital lipoma located in the craniofacial midline.

Abstract:  Panton-Valentine leukocidin (PVL)-producing Staphylococcus aureus results in leukocyte destruction and tissue necrosis (Pediatric Dermatology 2007;24:401). It can be associated with a spectrum of clinical manifestations that range from localized staphylococcal skin infections to sometimes severe necrotizing pneumonia (Clin Infect Dis 1999;29:1128). We report a case of four siblings, three brothers whose atopic dermatitis was complicated by cutaneous lesions and furunculosis, while their 21-month-old sister had a fatal PVL positive staphylococcal pneumonia.

Abstract:  Linear immunoglobulin A bullous dermatosis (LABD) is an autoimmune blistering disease that most commonly presents in preschool-aged children. There have been few neonatal reports, all of which had life-threatening aerodigestive complications requiring mechanical intervention and systemic therapy. We present a case of LABD in a neonate who had an uncomplicated course and was treated conservatively with only low-potency topical corticosteroids and wound care before resolution of his skin lesions.

Abstract:  We report a case of linear immunoglobulin A bullous disease in a 9-year-old boy who presented with rapidly progressive severe disease and could not tolerate dapsone because of high liver enzymes within a week after a low dose of dapsone in association with an underlying fatty liver. He showed remarkable improvement with intravenous immunoglobulins used as monotherapy, with a rapid clearance and a sustained remission after stopping the treatment.

Abstract:  Milia are common, small, keratin-containing cysts frequently seen in all age groups. They may arise spontaneously or develop after a variety of stimuli. They can be found alone or as part of syndromes. We present a female neonate born not only with profuse facial milia, but also with acral bullae and absent dermatoglyphics. Similar features were seen in several members of her family. These findings correspond to the syndrome known as Basan’s syndrome, a rare autosomal-dominant inherited dermatosis characterized by profuse congenital milia, transient neonatal acral bullae, and absence of dermatoglyphics.

Abstract:  A 2-year-old white girl with divided (or kissing) epidermal nevus of the third and fourth fingers of the left hand is described. The possible pathogenesis of this unique lesion is also discussed.

Abstract:  We describe two full-term infants who presented with congenital cutaneous candidiasis (CCC) and compare their clinical presentation and outcome with that of neonatal candidiasis and chronic mucocutaneous candidiasis. Although candidal vulvovaginitis occurs in up to one-third of pregnancies, CCC is uncommon and can be confused with more-serious pustular disorders that present in neonates. Greater awareness of CCC is essential to make an early diagnosis and distinguish it from other infections.

Abstract:  Henoch–Schönlein purpura (HSP) is the most common vasculitis of childhood. It is usually self-limiting and is characterized by an immune complex–mediated vasculitis associated with IgA deposition. We present an unusual case of HSP with mucosal lesions and coronary artery thickening.

Abstract:  We report an unusual occurrence of a primary dural-based malignant melanoma in a 13-year-old boy with neurocutaneous melanosis. The lesion presented with rapid-onset symptoms characterized by raised intracranial pressure and seizures, had an aggressive clinical course, and proved to be fatal despite two surgeries and adjuvant therapy. There should be a high index of suspicion for the occurrence of such a malignant leptomeningeal tumor in patients with congenital melanocytic nevi presenting with neurological symptoms of recent onset.

Abstract:  Although dermatoses affecting the soles of the feet in children are regularly encountered in dermatology clinics, the relationship with allergic contact dermatitis affecting this part of the foot is not well established. The aim of this study was to evaluate the relevance of patch testing children with sole dermatoses. We reviewed the results of all patch tests performed in children (<18 years) presenting with dermatoses involving the soles between 1997 and 2009 from our departmental patch test database. Forty-one children were identified: 27 children with an inflammatory dermatitis affecting the sole and 14 children with juvenile plantar dermatosis (JPD). Seventeen (41%) children had at least one clinically relevant positive patch test reaction. Rubber additives and potassium dichromate were the most frequent allergens identified. Forty-eight percent of children with inflammatory dermatitis affecting the sole and 29% of children with JPD had at least one relevant reaction. Of the children with relevant reactions, 76% had a personal or family history of atopy. Our results demonstrate the importance of patch testing children with dermatoses affecting the soles and indicate that a history of atopy or a diagnosis of JPD should not deter investigation.

Abstract:  We report a new case of postvaccination morphea profunda (MP) in a child and discuss its different clinical presentations, prognosis, and therapy and its relationship with “solitary morphea profunda.” A 2-year-old healthy girl presented with an induration of the anterior aspect of the left thigh of 9 months duration. The lesion had appeared 3 months after a third dose of diphtheria-tetanus-pertussis vaccine. Cutaneous examination showed an induration of 7 × 7 cm with an “orange peel” texture after pinching the skin. Histologic examination confirmed the diagnosis of MP. Systemic steroids (1 mg/kg/day) led to the stabilization of the lesion. After 4 months of treatment, we began the concomitant use of oral methotrexate (10 mg/wk) for 2 months. Methotrexate was then continued alone for 10 months, leading to a significant regression of the induration with no relapse.

Abstract:  We present a 15-year-old female patient with a 6-month history of recurrent painful petechiae on the fingers and feet. Trauma or pressure were denied, but she reported recurrent tonsillitis and urinary tract infections and a single event of bilateral scotoma. Extensive investigations (e.g., echocardiography) for a suspected diagnosis of septic emboli were unremarkable. Routine histopathology, direct and indirect immunofluorescence, and esophagogastroduodenoscopy led to the diagnosis of dermatitis herpetiformis. The therapeutic strategy comprised gluten-free diet and dapsone to alleviate the symptoms. Dermatitis herpetiformis should be included in the differential diagnosis of palmar or plantar petechiae, especially when occurring in children or young adults.

Abstract:  We report on a case of Netherton syndrome showing a new SPINK5 mutation (c.957_960dupTGGT duplication in exon 11), associated with partial defect of biotinidase.

Abstract:  In this study, we report on the case of a newborn boy diagnosed after birth with an accessory scrotum attached to a peduncular type of perineal lipoma without any other associated congenital anomalies. The neonate underwent a simple surgical excision of the lipoma and accessory scrotum in the first month of life, and his postoperative course was uneventful. Histologic examination revealed normal scrotal skin and adipose tissue. Accessory scrotum has a high incidence of association with perineal lipoma (83% of reported cases) and other urogenital and anorectal anomalies, but urogenital or anorectal anomalies were not seen in our patient.

Abstract:  Erythema nodosum (EN) in association with kerion celsi is a rare condition in children, with only 11 cases having been reported in the English literature. We describe a new case in a 7-year-old boy in whom the disorder had begun 2 months before. He had many inflamed, boggy, suppurative nodules over the left occipitoparietal area of the scalp and, 2 weeks later, developed multiple painful, erythematous subcutaneous nodules of the shins, thighs, and upper limbs. EN was confirmed by histologic examination. Our review of the literature of all cases of EN during kerion showed that it usually occurs at or slightly after the height of infection or after drug introduction. Trichophyton mentagrophytes was involved in the majority of cases, and improvement of EN usually occurs with griseofulvin.

Abstract:  An otherwise-healthy 13-year-old girl with previously normal nails developed longitudinal pigmented bands on multiple fingernails. Physical examination revealed faintly pigmented bands on multiple fingernails and on the left fifth toenail. We believed that the cause of the pigmented bands was onychophagia-induced longitudinal melanonychia, a rare phenomenon, which emphasizes the need for dermatologists to question patients with melanonychia about their nail biting habits because they may not be forthcoming with this information.

Abstract:  Circumscribed acral hypokeratosis is a disorder characterized by areas of erythematous depressed skin with distinct histopathological findings typically found on the palmar and plantar surfaces. Most patients are middle-aged women who report a multiyear history. We present an 10-year-old African American boy who had an asymptomatic, irregularly shaped erythematous lesion on his left medial foot that had been present since birth. A biopsy showed an abrupt, well-demarcated decrease in the thickness of the stratum corneum layer, with no parakeratosis, that was consistent with a diagnosis of circumscribed hypokeratosis. This represents the first pediatric and congenital case of circumscribed hypokeratosis reported. We review the literature and discuss the ramifications of a congenital case on understanding the etiology of circumscribed hypokeratosis.

Abstract:  Myoepithelioma is a well-known tumor in the salivary glands and breasts in adults. It is exceptionally rare in soft tissue and in children. We present a case of subcutaneous scalp myoepithelioma in an 11-year-old girl. On clinical examination, it appeared as a dermoid cyst. Myoepithelioma is uncommon in the subcutaneous tissue. Clinically, the neoplasm is nonspecific. Because of the variable appearance of myoepithelial cells and their phenotype, the pathological diagnosis is challenging. We report a case of subcutaneous myoepithelioma in a child and discuss the literature.

Abstract:  We report a case of 1-year-old girl with congenital hypertrichosis terminalis treated using intense pulsed light for hair removal. Repeated sessions were performed every 3 weeks. Facial hair reduction was achieved after 12 sessions and body hair reduction after 15 sessions. Intense pulsed light resulted in 75% reduction of hair in congenital hypertrichosis terminalis.

Abstract:  Neutral lipid storage disease is a nonlysosomal multisystemic triglyceride storage disease. It is characterized by leukocyte vacuolization (Jordans’ anomaly), variable systemic involvement, and ichthyosis. Two of our patients presented with congenital ichthyosis. Lipid vacuoles were demonstrated in granulocytes and monocytes and in basal keratinocytes on skin biopsy. They were diagnosed as Chanarin Dorfman syndrome. In contrast to these cases, the third case presented with progressive symmetric erythrokeratoderma without ichthyosis. Lipid vacuoles were demonstrated in blood cells on peripheral smear and in basal keratinocytes. Only screening of peripheral smear led to the correct diagnosis.

Abstract:  Ecthyma gangrenosum is classically a cutaneous manifestation of a pseudomonal septicemia that presents in a patient with an immunodeficiency or hematologic malignancy. We describe a previously healthy 8-month-old girl who developed transient neutropenia and characteristic ecthyma gangrenosum lesions secondary to methicillin-resistant Staphylococcus aureus. This unique presentation of methicillin-resistant Staphylococcus aureus ecthyma gangrenosum emphasizes the importance of broad empiric coverage and early culturing for microorganism and susceptibilities in any patient presenting with ecthyma gangrenosum.

Abstract:  Livedo reticularis is a lacy mottling of the skin that can have many etiologies. We report a case of an 8-year-old boy with a diagnosis of melanoma who developed persistent livedo reticularis during treatment with interferon alpha-2B. To our knowledge, this is the first case report of livedo reticularis occurring as a side effect of interferon treatment for pediatric melanoma. Given the increasing incidence of pediatric melanoma, it is important that dermatologists be aware of potential side effects of interferon therapy to optimize care and education for these patients.

Abstract:  Large congenital melanocytic nevus rarely presents itself without hairs, with hardened skin and progressive depigmentation. We report a girl who presented with a large congenital melanocytic nevus in the left cheek. Over the years, the nevus became pruriginous, light brown, bumpy, and hard. Histology revealed nevus cells interspersed with dense fibrosclerotic collagen bundles. There are few reported cases of large congenital melanocytic nevus with this evolution, so-called desmoplastic hypopigmented hairless nevus.

Abstract:  A 7-year-old girl presented with a 3-month history of a small blue–brown papule arising within a large sebaceous nevus on her temporal scalp. A punch excision was performed that demonstrated a hybrid follicular cyst. The majority of the cyst wall displayed pilomatrical differentiation consisting of basaloid matrical-type cells with luminal shadow cell keratinization. The matrical epithelium displayed strong membranous, cytoplasmic, and nuclear staining for β-catenin. Only a small component of the wall displayed infundibular differentiation. These findings were consistent with a hybrid follicular cyst predominantly displaying pilomatrical differentiation with only a small component of infundibular lining. The most common tumors arising within sebaceous nevi are the syringocystadenoma papilliferum and trichoblastoma. Follicular cysts with infundibular (sebaceous cyst) and trichilemmal (pilar cyst) differentiation are exceedingly common, although their development within sebaceous nevi appears to be rare. We report a rare case of a hybrid follicular cyst with matrical differentiation occurring within a sebaceous nevus.