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Lymphoplasmacytic plaque in children has been proposed as a rare, emerging clinicopathologic entity characterized by solitary, extratruncal, asymptomatic papules and plaques that are typically found in healthy young Caucasian females. Biopsy of these lesions reveals a dermal lymphoplasmacytic infiltrate with or without epithelioid granulomas. Two unique patients with lymphoplasmacytic plaque in children are presented in this report, including a 26-month-old female with a lesion on her finger, who represents both the youngest described patient and the first documented with a finger lesion, as well as a 17-year-old young woman with a left thigh lesion, who represents the patient with the longest clinically and histopathologically observed lesion to date. These two additional patients corroborate the experience of lymphoplasmacytic plaque in children in the six previously reported cases and further expand the clinicopathologic spectrum of the disease. Recognition of lymphoplasmacytic plaque in children is important to facilitate distinction from potential differential considerations, including lymphoproliferative disorders and infectious conditions, particularly as the experience to date appears to suggest that lymphoplasmacytic plaque in children represent a reactive (pseudolymphomatous) condition.
‘Pretibial lymphoplasmacytic plaque in children’ was the term recently proposed for an emerging clinicopathologic entity by Fried et al. On the basis of the six reported cases to date, affected patients are typically healthy Caucasian girls or adolescents with a solitary extratruncal plaque or papules. In each reported case, the plaque had typically been stable and asymptomatic. A dermal lymphoplasmacytic infiltrate, with or without epithelioid granulomas, is noted histopathologically. Significantly, no hypergammaglobulinemia, lymphadenopathy or hepatosplenomegaly has been reported in these patients. Presented herein are two patients who show clinical and histopathologic features that both corroborate the previous characterization of this condition as a pseudolymphomatous state, expand its clinicopathologic spectrum to lesions occurring on the thigh and digits and document lesions occurring in younger children, thereby lending support to adoption of the broader term ‘lymphoplasmacytic plaque in children’.
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Lymphoplasmacytic plaque in children represents an emerging diagnosis whose clinicopathologic characterization continues to evolve. Born of the recognition that indolent, localized plaques exhibiting a prominent lymphoplasmacytic infiltrate can occur in children lacking evidence of systemic involvement, lymphoplasmacytic plaque was originally termed ‘isolated benign primary cutaneous plasmacytosis in children’ by Gilliam et al. In retrospect, however, a patient who also likely would meet criteria for this condition had been previously reported under the label of ‘primary cutaneous plasmacytosis in a child’, distinct from the classic primary cutaneous plasmcytosis seen in adults with many systemic symptoms. Gilliam et al. acknowledged this and reported two additional healthy young patients with plasmacytic plaques overlying the tibia. A subsequent patient with similar features was then documented by Fried et al. who in their analysis suggested the term ‘pretibial lymphoplasmacytic plaque in children’ to emphasize the absence of systemic plasmacytosis in these individuals. Since these reports, two additional patients have been reported in the literature (Table 1), including one 7-year-old Asian girl whose lesion arose on the buttock and a 9-year-old girl with a pretibial lesion exhibiting the epithelioid granulomatous component on histopathology.
Table 1. Reported cases of lymphoplasmacytic plaque in children
| ||Age (years)||Gender||Ethnicity||Location||Morphology||Duration by history (years)||Hematoxylin–eosin staining||Clinical course/treatment|
|Aricò and Bongiorno||7||F||Caucasian||Trunk||10 cm plaque with red–brown papules||4||Dermal lymphoplasmacytic infiltrate||Intermittent treatment with topical corticosteroids was unsuccessful|
|Gilliam et al.||15||F||Caucasian||Pretibial||4.5 cm red–brown violaceous plaque||11||Dermal lymphoplasmacytic infiltrate||Pulse-dye laser was used to diminish the color of the lesion|
| ||7||M||Caucasian||Pretibial||Several centimeters scaled violaceous papulonodules||2||Dermal lymphoplasmacytic infiltrate||N/A|
|Fried et al.||11||F||Caucasian||Pretibial||4 cm red–brown plaque||5||Dermal lymphoplasmacytic infiltrate ± small granulomas||Partial, short-term remission was achieved with intralesional steroid injection|
|Ahn et al.||7||F||Asian||Buttock||3 cm erythematous scaly plaque||7||Dermal lymphoplasmacytic infiltrate||Partial remission was achieved with a combination of tapered prednisolone, intralesional steroids and topical steroids. Excision was eventually performed|
|Moulonguet et al.||9||F||Caucasian||Pretibial||3.5 cm red–brown plaque||5||Dermal lymphoplasmacytic infiltrate ± epithelioid granulomas||Local and topical steroids resulted in slight improvement|
|Present report||17||F||Caucasian||Thigh||2 cm pink serpiginous plaque||>10*||Lichenoid dermal lymphoplasmacytic infiltrate with small epithelioid granulomas||The lesion was excised and the patient was recurrence free at 11 months|
| ||2 years and 2 months||F||Caucasian||3rd Finger||8 mm erythematous scaled papule||2 years and 6 weeks||Lichenoid dermal lymphoplasmacytic infiltrate||The patient's family has elected to observe the lesion while weighing options for further management|
Clinically, lymphoplasmacytic plaque in children is characterized by minimally symptomatic, solitary, pink to violaceous erythematous papulonodular plaques up to a few centimeters in dimension with a variably smooth to lobulated surface, and variable scaling. Affected individuals to date have ranged from age 7 to 15, with predilection for females. As indicated by the earlier term pretibial lymphoplasmacytic plaque, the anterior shins appear the favored site; however, more recent experience has showed occurrence of lesions at other locations, including the buttock. This clinical picture can raise differential consideration for vascular lesions, spitzoid proliferations, histiocytic tumors, infectious processes or lichenoid conditions. Significantly, none of these individuals evidenced any signs of systemic involvement related to their lymphocytic plaques, and all the patients accordingly have experienced a favorable course, with treatment for the lesions including observation, topical steroids and excision.
Histopathologically, lymphoplasmacytic plaque shows a dense, mixed infiltrate featuring lymphocytes with admixed plasma cells. Recent reports have highlighted that an epithelioid granulomatous infiltrate can also be seen. Additionally, some lesions have been noted to exhibit a strikingly lichenoid pattern. The presence of variably robust lymphocytic and plasmacytic populations can raise suspicion for a primary lymphoproliferative disorder or for an infectious process. With regard to the former category, particular differential consideration can be given to cutaneous plasmacytoma, primary cutaneous plasmacytosis, end-stage multiple myeloma or marginal zone B-cell lymphoma. In contrast to this group of disorders, lymphoplasmacytic plaque in children does not feature lymphoid or plasmacytic atypia, and clonality has not been showed. With respect to the latter category of infectious disease, syphilis and other treponemal diseases need to be excluded by appropriate clinical and serologic evaluation. The presence of a granulomatous infiltrate obliges work-up for mycobacterial or deep fungal infection. Additional possible conditions which may enter into the pathologic differential diagnosis of lymphoplasmacytic plaque include nodular amyloidosis and even Kaposi's sarcoma.
Consideration of the pathologic differential possibilities that are raised by the histopathologic features of lymphoplasmacytic plaque in children not only highlights the importance of distinction of indolent lymphoplasmacytic plaque from potentially aggressive alternatives but also offers intriguing insight into the possible nosologic position of the entity on the continuum of lymphoid–plasmacytic disorders. Primary cutaneous plasmacytosis, in particular, represents a significant entity with overlapping and contrasting features with lymphoplasmacytic plaque. Primary cutaneous plasmacytosis typically presents as multiple truncal red–brown plaques in Asian individuals with onset in adulthood and typically with associated hypergammaglobulinemia. Furthermore, patients with primary cutaneous plasmacytosis often have lymphadenopathy, hepatosplenomegaly and an elevated erythrocyte sedimentation rate, features not found in lymphoplasmacytic plaque. One additional entity meriting consideration in the differential diagnosis of lymphoplasmacytic plaque is acral pseudolymphomatous angiokeratoma of children (APACHE). APACHE is a rare clinical entity characterized clinically by multiple unilateral angiomatous papules located on hands and feet of children and microscopically by a lymphoplasmacytic infiltrate with long, thick-walled blood vessels and prominent endothelial cells. Given the observation that some reported patients with APACHE clinically and microscopically appear to closely resemble patients with lymphoplasmacytic plaque. Moulonguet et al. suggested that these disease entities may both reside on the spectrum of pseudolymphoma.
The two patients presented here, representing the seventh and eighth cases of lymphoplasmacytic plaque reported to date, offer an additional experience with this provisional entity and support the conclusion that this condition may represent a pseudolymphomatous state. Both the patients detailed here appear to fulfill criteria for a diagnosis of a lymphoplasmacytic plaque while at the same time showing novel, previously undocumented features. To our knowledge, patient 1 is the first to show stability, clinically and histopathologically, for greater than 5 years. In addition, the localization of the plaque on the thigh in the case of patient 1 and on the finger in the case of patient 2, rather than in the pretibial area, is unique (Table 1). These two patients further corroborate previous observations that these plaques arise in childhood, often in Caucasian females, and are extratruncal, solitary and asymptomatic. To our knowledge, these two patients are the oldest and youngest reported with lymphomplasmacytic plaque, respectively. Histopathologically, these cases are consistent with past reports of a dermal polyclonal lymphoplasmacytic infiltrate. The biopsy in the case of patient 1 is also notable histopathologically for the presence of small epithelioid granulomas, a finding that was only also reported by Fried et al. and Moulonguet et al. The fact that patient 1 developed a lesion that was stable in size for over 10 years supports the conclusion that conservative therapy is appropriate for these lesions. Additional investigation is necessary to answer why this indolent condition occurs in childhood and thereby elaborate where on the lymphoplasmacytic spectrum lymphoplasmacytic plaque of children truly exists.