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Keywords:

  • Genodermatoses;
  • skin disease;
  • nursing practice;
  • gene;
  • autosomal dominant;
  • autosomal recessive;
  • X-linked

Abstract

  1. Top of page
  2. Abstract
  3. Genetics of Skin Disease
  4. Multifactorial or Complex Condition Exemplars
  5. Conclusions
  6. Acknowledgements
  7. Clinical Resources
  8. References

Purpose: Some forms of genetic skin disease are highly prevalent and others are exceedingly rare, but collectively, genetic skin disorders (or genodermatoses) are often poorly understood. The purpose of this article, therefore, is to increase nurses’ awareness and understanding of some of the physical, psychological, social, and ethical issues facing patients with inherited skin disorders.

Organizing Construct: This article offers an overview of genetic skin diseases; highlights the complexity and prevalence of the genodermatoses; describes inheritance patterns, genetics, and treatment for six genodermatoses; and reviews some of the ethical, privacy, technological, and resource issues nurses should consider when caring for patients with genetic skin disorders.

Conclusions: Because genodermatoses are found in all age groups, across all populations, and within all healthcare settings, nurses are uniquely positioned to address the educational and healthcare needs of patients and families with inherited skin disorders.

Clinical Relevance: Over the past two decades, genetics has evolved from a niche specialty into general practice. To ensure that patients and their families receive appropriate services and resources, nurses must have a working knowledge of genetic concepts. This article reinforces key genetic concepts while discussing many of the issues and concerns important to caring for patients with genetic skin disease.

Skin plays a vital role in protecting the body, serving as the first line of defense between internal and external environments. Healthy skin guards against pathogen invasion, protects against excessive water loss, regulates body temperature, and plays important roles in sensation, vitamin D synthesis, and protection of vitamin B and folic acid (Long, McMillan, Qiao, Akiyama, & Shimizu, 2009; Madison, 2003). (To learn more about the structure and function of normal skin, the reader is referred to the National Cancer Institute PDQ Genetics at http://www.cancer.gov/cancertopics/pdq/genetics/skin/HealthProfessional.) The term genodermatosis is used to describe any of the genetic mutations that alter the skin's ability to function normally. As a group, the genodermatoses are unusual in that while many of them pose minimal risk to longevity, they frequently have a significant negative impact on quality of life because skin disease is visible and often associated with significant social stigma (Smith & McLean, 2011). Patients with psoriasis, for example, report reduced physical and mental well-being compared to people living with cancer, arthritis, or depression, and these reductions in quality of life were not limited to the disfiguring skin lesions but were attributed to discomfort (itch and pain), functional limitations (bleeding, infections), treatment costs, and side effects (Sebaratnam, McMillan, Werth, & Murrell, 2012). Early recognition and prompt initiation of screening and appropriate therapy can minimize the cosmetic impact of many genodermatoses while improving health outcomes through early identification of associated but otherwise invisible abnormalities.

Caring for patients with a genetic skin condition can be challenging and multifaceted because visible skin changes are usually only one of many disease manifestations. Clinicians must treat the skin lesions, prepare patients and families for the social and emotional stigma that may accompany a skin disorder, treat nondermatologic manifestations (seizures, tumors), initiate screening to monitor the progression of internal disease, educate patients and families about the genetics of the disorder, and refer families to local and online resources. Until recently, patients and families with skin disorders often spent years and thousands of dollars searching for a diagnosis, only to find there was no effective treatment, little research, or very few other individuals with whom to share experiences. Advances in computing power and genomic information over the past two decades have rapidly changed the landscape for these patients and their families. Gene sequencing has made the diagnosis of rare genetic disorders more likely, and the Internet has connected families and communities across the globe, facilitating exchange of information and experiences. New organizations, like the European support group Together Against Genodermatoses (http://www.tag-eu.org) have emerged, offering virtual support to individuals and families living with the physical, social, and psychological impact of severe skin disease. Because nurses interact with patients in virtually every healthcare setting, it is important that they have a working knowledge of genetic concepts, have some familiarity with the genodermatoses, and can quickly access reputable healthcare resources to quickly learn more about an unfamiliar condition.

Genetics of Skin Disease

  1. Top of page
  2. Abstract
  3. Genetics of Skin Disease
  4. Multifactorial or Complex Condition Exemplars
  5. Conclusions
  6. Acknowledgements
  7. Clinical Resources
  8. References

Over 500 gene mutations are known to cause more than 560 distinct skin disorders (Feramisco, Tsao, & Siegel, 2010), 400 of which can be traced to a specific gene locus. Because there is significant overlap in many of these disorders, a number of different classification systems have been developed in an attempt to describe and categorize them. Recent genetic and biologic advances have (a) improved the understanding of normal skin homeostasis, (b) are offering new insight into the mechanisms involved in skin disease, (c) are driving the development of new treatment options, and (d) have changed the way dermatologic disorders are classified (Betz, Cabral, Christiano, & Sprecher, 2011). In this article, the genodermatoses have been classified into one of 12 categories (Table 1) based on the type of skin lesion, and further subdivided by inheritance patterns (Table 2). Readers may be surprised to see a number of familiar conditions listed as genodermatoses, possibly because when introduced to these disorders, nurses learn more about the disease's impact on internal organ systems than on its dermatologic features.

Table 1.  Genodermatoses Categories, Number of Conditions in Each, and Description of Condition
Category n Description
Keratinizing25Extensive, often generalized skin scaling
Palmoplantar keratodermas20Painful blistering on palms and soles of feet
Other keratinizing 7Often associated with cardiac abnormalities
Bullous27All result in some form of skin blistering
Ectodermal dysplasia32Significant overlap with other disorders
Connective tissue50Many are multisystem disorders
Pigmentation54Melanin production or regulation defects
Vascularization26Vascular manifestations
Malignant potential42Associated with an increased for cancer
Porphyria 7Enzyme deficiencies in heme synthesis
Immunodeficiency11Multisystem disorders involving skin
Hair11Pigmentation, hair loss, structural defects
Miscellaneous99 
Table 2.  Selected Genodermatoses by Inheritance Pattern, Gene, and Disorder Category
ConditionGene(s)Disorder category
  1. Note. Data on selected disorders are from tables in Smith and McLean (2011).

Selected autosomal dominant disorders
 Ichthyosis vulgaris FLG Keratinization
 Epidermolytic palmoplantar keratoderma KRT9, KRT1 Palmoplantar keratoderma
 Epidermolysis bullosa simplex KRT5, KRT14 Bullous
 Osteogenesis imperfecta I COL1A1, COL1A2 Connective tissue
 Neurofibromatosis NF1 Pigmentation
 Apert syndrome FGFR2 Vascularization
 Cowden syndrome PTEN Malignant potential
 Porphyria cutanea tarda UROD Porphyria
 Hypertriglyceridemia, familial APOA5, LIPI Miscellaneous
Selected autosomal recessive disorders
 Congenital ichthyosis ABCA12 Keratinization
 Lethal acantholytic epidermal bullosa DSP/PKP1 Epidermal bullosa simplex
 Hemochromatosis HFE Pigmentation
 Ataxia-telangiectasia ATM Vascularization
 Bloom syndrome RECQL3 Malignant potential
 Nijmegan breakage syndrome NBS1 Immunodeficiency
 Congenital insensitivity to pain NTRK1 Miscellaneous
Selected X-linked disorders
 X-linked ichthyosis STS Keratinization
 Keratosis follicularis spinulosa decalvans SAT1 Desmosomes
 Incontinentia pigmentiNEMO (IKBKG)Ectodermal dysplasia
 Fabry disease (dominant & recessive) GLA Connective tissue
 Congenital adrenal hypoplasia NROB1 Pigmentation
 Androgen insensitivity syndrome AR Malignant potential
 Chronic granulomatous disease CYBB Immunodeficiency
 Alport syndromeCOL4A5Miscellaneous
Selected multifactorial disorders
 Atopic dermatitis & psoriasisMultiple genesKeratinization
 Ectodermal dysplasia 1 EDA Ectodermal dysplasia
 Systemic lupus erythematosisMultiple genesConnective tissue

Many skin disorders have long been recognized as “genetic” because affected individuals tend to cluster within families. Teasing out the contribution of individual genes is complicated, however, because disease severity often varies widely between family members despite shared environments, diets, and behavioral patterns. Recent scientific advances in whole genome sequencing and genome-wide association studies are offering important insight into the genetics of many disorders, including complex skin conditions. It is now known that mutations causing dermatologic diseases can occur through virtually every inheritance pattern. Some disorders involve multiple genes and are strongly influenced by environmental exposures and individual behaviors, clearly placing these disorders in the “complex disease” category. Others are single gene disorders inherited in the familiar autosomal dominant, recessive, and X-linked inheritance patterns. Genodermatoses may also develop as a result of chromosomal deletions and rearrangements, methylation abnormalities, and mitochondrial mutations. Some individuals also develop mosaic genodermatoses, in which a mutation is acquired during embryogenesis, altering some, but not all, cell lines. Mosaicism is often more easily seen in skin disease because abnormal skin may appear in patches against a backdrop of normal-appearing skin, often appearing along Blaschko lines (areas of skin that correspond to embryonic migration patterns; Smith & McLean, 2011). Genodermatoses can be particularly challenging to diagnose and treat because clinical and genetic heterogeneity is common and a number of modifier genes and environmental factors influence how skin disease presents (Smith & McLean, 2011). For more information on genetic variation, the reader is referred to http://ghr.nlm.nih.gov/handbook/illustrations.

Multifactorial or Complex Condition Exemplars

  1. Top of page
  2. Abstract
  3. Genetics of Skin Disease
  4. Multifactorial or Complex Condition Exemplars
  5. Conclusions
  6. Acknowledgements
  7. Clinical Resources
  8. References

Multifactorial disorders are diseases that are influenced by the interaction of a number of different genes scattered throughout the genome. These genes work in concert with one another, and are often regulated by environmental or behavioral factors. Multifactorial genetic conditions like acne, eczema, alopecia, and psoriasis affect as many as one in four individuals in some communities, virtually guaranteeing that all nurses will encounter patients with an inherited skin disease at some point.

Atopic Dermatitis

Atopic dermatitis, or eczema, is very common in some communities, affecting nearly 15% of the children in industrialized countries (Hoffjan & Epplen, 2005). Symptoms usually appear in childhood, often disappearing over time, but occasionally persisting, or manifesting in adulthood. At least five genes have been associated with atopic dermatitis, but mutations in the filaggrin (FLG) gene are known to play a significant role in atopic dermatitis, other forms of eczema, ichthyosis vulgaris, and other severe dry skin conditions (Smith & McLean, 2011; Weidinger et al., 2006). The FLG mutation produces an abnormal keratin-filament aggregating protein causes skin corneodesmosomes to break down prematurely, impairing the epidermal barrier. When exposed to irritants like soap and detergents or proteases produced by dust mites or Staphylococcus aureus, allergens may penetrate the already damaged skin barrier, inciting an inflammatory cascade and exacerbating the disease. Atopic dermatitis is a good example of the role that the environment can play in disease exacerbations. It also highlights the variability of disease manifestations, because not all severely affected patients have an FLG mutation, and not all people with an FLG mutation develop eczema.

Psoriasis

It is estimated that 2% to 10% of people worldwide have psoriasis, an autoimmune disorder characterized by patches of itchy, scaly skin. Psoriasis symptoms vary widely, and individuals with severe disease report health-related quality-of-life degradation similar to individuals with chronic diseases, such as depression, hypertension, congestive heart failure, or type 2 diabetes (Sampogna et al., 2006). Although the exact cause is unknown, environmental triggers and genetic predisposition have both been found to play major roles in the development of psoriasis. In a recent twin study, approximately two thirds of the risk for psoriasis could be explained by genetics and the remaining one third could be attributed to shared environmental risk factors (Grjibovski, Olsen, Magnus, & Harris, 2007).

Genetic susceptibility to psoriasis appears to be primarily mediated through genes controlling T cell–mediated immune response. In normal skin, a cell matures and is shed in 28 to 30 days, but in psoriasis, skin cells mature in as little as 3 to 4 days and scaly patches of thick skin rapidly build up, forming psoriatic lesions, triggering further immune responses. The most significant psoriasis gene is in the human leukocyte antigen (HLA) gene complex, specifically the HLA-Cw*0602 allele. Individuals with one copy of the HLA-Cw*0602 allele are at increased risk for developing psoriasis when exposed to an environmental trigger (Liu et al., 2008). Other genes, such has interleukin (IL)-12B and IL-23R have also been implicated in the development of psoriasis, and interestingly, although neither is in the HLA family, both are involved in immune modulation. In addition to its influence on autoimmune inflammatory disease, IL-23 is suspected to play an important role in tumorogenesis (Gene Cards, 2011).

Monogenic (Single Gene) Inheritance Patterns

While some genodermatoses have high de novo (spontaneous) mutation rates, most skin diseases are inherited in an autosomal dominant, recessive, or X-linked manner, and a detailed family history should be collected to inform genetic counseling and family planning decisions. A comprehensive discussion of ethical issues involved in genetic counseling is beyond the scope of this manuscript.

Autosomal Dominant Genodermatoses Exemplar

Peutz-Jeghers syndrome Peutz-Jeghers syndrome (PJS) is an autosomal dominant cancer syndrome caused by mutations in the STK11/LKB1 tumor suppressor gene, on chromosome 19 (Amos, Frazier, Wei, & McGarrity, 2001). Because PJS patients are at extremely high risk for developing cancers (lifetime risk 93%), clinicians should take advantage of the distinct skin manifestations to identify these patients early in life to make cancer screening and prevention more effective in this high-risk population (Amos et al., 2001). PJS is usually diagnosed when patients present with characteristic skin manifestations and are found to have hamartomatous gastrointestinal polyps. The skin lesions usually emerge in childhood, appearing as dark blue to dark brown macules on the fingers, face, and perianal area. Hamartomatous polyps typically develop in the small intestine but may also appear in the stomach, large bowel, and nasal passages. While the polyps themselves are rarely cancerous, they are associated with chronic bleeding and anemia as well as bowel obstruction and intussusception, where one portion of bowel invaginates into the next. Affected patients are also at significantly increased risk for epithelial cancers, including colorectal, gastric, pancreatic, breast, and ovarian cancer (Amos et al., 2001).

There is still some uncertainty about how frequently the mutant STK11/LKB1 gene is inherited, how often it arises as a de novo mutation, and whether it is the only explanation for PJC. In one study of 170 patients with PJS from 46 unrelated families, genetic testing identified a pathogenic STK11/LKB1 gene mutation in just over half (59%) of the patients. The other 41% of patients had virtually identical clinical manifestations but no PJS mutation, making the possibility of PJS genetic heterogeneity plausible (Mehenni et al., 2007). As molecular and genetic testing become more sophisticated, de novo mutation rates, PJS heterogeneity, and PJS overall incidence/prevalence will become better understood. Until then, counseling for PJS inheritance risk is the same as for any autosomal dominant condition; the risk to offspring of a PJS affected individual is 50%, and prenatal testing is available for families in which the mutation is known.

Autosomal Recessive Genodermatoses Exemplars

Autosomal recessive skin disorders are rare, but when they do occur, they are often severe and may be lethal in utero or immediately after birth. In most autosomal recessive disorders, both parents carry one copy of the deleterious mutation and the risk to offspring is 25% for inheriting the mutation from each parent.

Albinism Albinism is an autosomal recessive disorder characterized by defects in the synthesis or transport of melanin. While relatively uncommon in the United States (1:37,000), albinism affects approximately 1 in 17,000 individuals worldwide, with the highest prevalence in sub-Saharan Africa, where approximately 1 in 4,000 Zimbabweans and 1 in 1,429 Tanzanians are born with the disorder (Cruz-Inigo, Ladizinski, & Sethi, 2011). Oculocutaneous albinism (OCA) is the most prevalent form of albinism and has been categorized into one of four subtypes depending on the mutations involved (tyrosinase or p-protein) and the amount of melanin the individual's skin can produce (King, 2004). People who cannot synthesize or who make only a limited amount of melanin suffer from a number of deleterious visual changes and are at significantly increased risk for sunburn and skin cancers because melanin offers protection from the damaging effects of ultraviolet radiation while giving skin, hair, and eyes their color.

Melanin is critical for eye development and skin protection. When melanin is absent, or present in very low amounts, eye movements may be abnormal and visual acuity is often poor. As a result, affected individuals often have difficulty learning to read and, without intervention, perform poorly in school. Prior to the advent of sun screen and protective clothing, nearly 50% of affected individuals had some form of early skin cancer by early adulthood. When sun screen and protective clothing are worn, however, skin cancer is rare, even among OCA patients who make no melanin at all (Cruz-Inigo et al., 2011).

Children with OCA are at particularly high risk for social stigma because they look different with their very pale skin and pink eyes; they may struggle in school; they avoid the sun by staying indoors; and they may wear unusual clothing. In the most extreme example of social stigma, some albino families living in South Africa must also worry about being hunted for their body parts, which are highly valued as amulets or good luck charms. Many albinos in these communities will leave their families, seeking the relative safety of large, anonymous urban centers, further increasing their social isolation and marginalization (Cruz-Inigo et al., 2011). Nurses play several key roles in helping affected patients and families manage the disease and the social stigma that often accompanies it. Nurses provide education, help families locate appropriate healthcare services and resources, and offer suggestions about ways to cope with social stigma. Nurses also help patients and families navigate the significant physical, emotional, and social consequences, including school experiences, employment opportunities, recreational activities, and intimate relationships (National Institutes of Health Clinical Center, 2006).

X-linked Genodermatoses

Many health professionals can describe X-linked inheritance patterns and identify at least a few X-linked disorders, but may not be aware that the inheritance pattern they are describing is for an X-linked recessive disorder. In an X-linked recessive disorder, women usually do not manifest symptoms because one of their X chromosomes can produce enough normal protein that they remain asymptomatic. If a male inherits the X with the mutation from his mother, he will express symptoms because he does not have a second X producing normal protein. This is an important characteristic of X-linked disorders; 50% of males born to a carrier mother will be affected, and 50% will be unaffected. Females can manifest classic X-linked recessive symptoms if their father is affected and their mother is a carrier, and they inherit two X mutations, but this is uncommon (Smith & McLean, 2011). In an X-linked dominant disorder, only one X mutation is needed for disease to manifest. X dominant disorders are usually severe; affected males are likely to die in utero, and while symptomatic, females are more likely to survive because they have one X that produces at least some functional gene product. Examples of X-linked dominant dermatologic disorders include incontinentia pigmenti (IP), Rett syndrome, and Aicardi syndrome (Pagon, Bird, Dolan, Stephens, & Adam, 2012).

Incontinentia Pigmenti IP is lethal most males, and the disease is rare, affecting approximately 700 females worldwide. The diagnosis is usually made based on clinical features but can be confirmed by skin biopsy or by gene testing, and genetic counseling is challenging because disease expression varies widely (Pettigrew et al., 2000). The earliest IP manifestations are skin lesions that evolve predictably through four stages: blistering from birth to approximately 4 months, a wart-like rash for several months, hyperpigmentation from approximately 6 months into adulthood, and finally linear hypopigmentation. Excess melanin is deposited in the skin, appearing on the trunk and extremities as slate gray, blue, or brown marbled or wavy lines. Other features of IP include alopecia, hypodontia, abnormal tooth shape, and dystrophic nails. Other health problems, including cataracts, retinal detachment, and severe vision loss; cognitive delay and intellectual disability and severe skeletal and structural abnormalities, including hemivertebrae, scoliosis, spina bifida, syndactyly, and acheiria (congenital absence of the hands) may also be seen.

Nursing Implications

Nurses are directly engaged with patients and their families in every life event, are present in all healthcare settings, and work with all population groups. As a result, patients rely heavily on nurses for advice, support, navigation assistance, and accurate information about health and healthcare. The public expects nurses to understand how genetic conditions are inherited, understand the genetic underpinnings for common conditions, help patients recognize and navigate the ethical issues associated with genetic information, and be familiar with genetic resources so they can help locate genetic information when it is needed. To prepare the nursing workforce for genomic healthcare, the second edition of the Essentials of Genetic and Genomic Nursing: Competencies, Curricula Guidelines, and Outcome Indicators was published in 2009 (Consensus Panel on Genetic/Genomic Nursing Competencies, 2009), followed 3 years later by the Essential Genetic and Genomic Competencies for Nurses With Graduate Degrees (Greco, Tinley, & Seibert, 2012). Together, these two documents establish the essential knowledge, education, and practice competencies across the nursing spectrum.

Although nurses are not expected to know every detail of all genetic conditions, they should be familiar with genetic disorders commonly seen in their communities and be prepared to develop individualized care plans for patients and families with genetic concerns. If a genetic referral culminates in a genetic test, nurses should be prepared to discuss the ethical issues that often arise when genetic testing is done. For example, when genetic testing is done to confirm the existence of a suspected genodermatosis, it is not uncommon for another deleterious gene to be identified, which may indicate that the individual is at increased risk for developing another completely unrelated genetic disease, such as Alzheimer's disease. Several ethical issues arise involving disclosure of this incidental genetic information, such as: should the incidental finding be disclosed to the patient or family, and if the findings are disclosed, who does that, and when should it be done?

Ethical issues also emerge when considering gene therapy. There are three primary types of gene therapies: (a) cell therapy involves replacing diseased cells with healthy ones (organ transplant, blood transfusion, and in vitro fertilization); (b) protein therapy involves replacing normal proteins by injecting them into tissues with missing or nonfunctional proteins (less commonly used); and (c) gene therapy (largely experimental) involves the replacement or repair of missing or broken genes (Long et al., 2009). While each has unique ethical issues, overarching concerns include who is offered gene-based therapy, who decides when the therapy is offered, what disorders will be treated, and whether gene therapy will be used to cure disease or create more attractive, intelligent, or athletic human beings (Long et al., 2009).

There are some significant privacy concerns in genomic health care as well. While amazing advances in genomic science are revealing more each day about the underlying mechanisms in health and illness, an individual's personal genetic code also holds information about his or her risk for developing diseases far into the future while simultaneously revealing risks shared by closely related family members. Genomic information could easily be used to discriminate against individuals, their families, and communities. Nurses must therefore be familiar with genetic privacy issues and understand the scope and limitations of the protections offered by the Genetic Information Non-discrimination Act, passed in the spring of 2008. Nurses should be comfortable discussing the concepts of genetic information security, identifiability, consent, and access to stored samples.

Accurate, reliable, up-to-date information on genetic disorders has been made infinitely easier with the advent of the Internet. Over the past two decades, many genetic, scientific, and family advocacy organizations have taken the time to develop detailed, useful, robust, and continually updated Web sites, and concurrent advances in search technologies have made locating these Web sites infinitely faster and easier. Finding information can be an exercise in frustration, however, if the disease name is misspelled or is confused with another, similar disorder. Nurses should therefore ask patients whenever possible to write the disorder down or recommend a reliable site so that learning about an unfamiliar disorder is optimized. Some helpful resources focusing exclusively on skin include the Foundation for Ichthyosis and Related Skin Types (http://www.firstskinfoundation.org/), GeneSkin (http://www.geneskin.org/), and the Human Intermediate Filament Database (http://www.interfil.org).

Conclusions

  1. Top of page
  2. Abstract
  3. Genetics of Skin Disease
  4. Multifactorial or Complex Condition Exemplars
  5. Conclusions
  6. Acknowledgements
  7. Clinical Resources
  8. References

Skin diseases affect millions of people worldwide and cause significant morbidity, decreased quality of life, and social stigma. Despite the ubiquitous nature of the genodermatoses, they are often poorly understood outside the dermatology community. Patients and families living with severe skin conditions will grapple with the familiar ethical and legal issues of fairness, privacy, reproductive concerns, and health professional lack of preparedness, but must also cope with the often profound social stigma and isolation associated with visible physical differences in skin integrity, texture, and color (U.S. Department of Energy Genome Programs, 2012). Because nurses often serve as the link between the science of genetics and the human experience of health and illness, nurses can make an enormous difference in healthcare outcomes for individuals, families, and communities by being comfortable with and prepared to identify, educate, and refer patients and families with dermatologic conditions suspected of having a hereditary basis.

Acknowledgements

  1. Top of page
  2. Abstract
  3. Genetics of Skin Disease
  4. Multifactorial or Complex Condition Exemplars
  5. Conclusions
  6. Acknowledgements
  7. Clinical Resources
  8. References

The views expressed in this article are those of the author and do not necessarily reflect the official policy or position of the Department of the Navy, Army, Department of Defense, nor the United States government.

Clinical Resources

  1. Top of page
  2. Abstract
  3. Genetics of Skin Disease
  4. Multifactorial or Complex Condition Exemplars
  5. Conclusions
  6. Acknowledgements
  7. Clinical Resources
  8. References

References

  1. Top of page
  2. Abstract
  3. Genetics of Skin Disease
  4. Multifactorial or Complex Condition Exemplars
  5. Conclusions
  6. Acknowledgements
  7. Clinical Resources
  8. References
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