By Gary M. White, MD
Epidermodysplasia verruciformis (EV) is a rare genodermatosis in which the patient is susceptible to cutaneous infection with human beta-papillomavirus. Diffuse persistent flat warts or pityriasis versicolor-like lesions are characteristic. 75% of cases involve mutations in TMC6(EVER1) and TMC8(EVER2) genes. An "acquired EV" occurs and describes an EV-like phenotype in immunocompromised individuals, e.g. HIV infection or chemotherapy.
- There is significant increase risk of SCC in the inherited type. The SCC occurs in sun-exposed areas so sun protection is critical.
- For acquired EV, the risk of SCC is unknown.
- HPV 5 and 8 are the most common virus subtypes found in lesions and are found in 90% of the HPV-associated malignancies.
Flat papules and plaques on the dorsa of the hands and arms which have some resemblance to verruca vulgaris or verruca plana. Hypo and hyperpigmented macules occur on the trunk and may resemble tinea versicolor. In the inherited type, lesions begin in childhood and progressively increase in size and severity. 30-60% of patients experience malignant transformation but the fourth decade.
For the inherited type, sun protection is critical. Patients can die from metastatic SCC if they do not diligently protect themselves from the sun.
- The patient should be monitored closely for skin cancer and educated on sun avoidance.
- Improvement of the immune state should be done if possible, e.g. HAART therapy in the case of HIV infection or reduction of any immunosuppressive agents if possible.
- Genetic counseling should be done for inherited EV.
- Both systemic retinoids and alpha interferon have been used as treatment. For example, a 19-year-old woman was treated with acitretin 0.75 mg/kg/day in combination with recombinant interferon alfa-2a subcutaneously 3 million IU 3 times per week for 6 months [JAAD 2001;45;296]. Her skin lesions were greatly reduced, but not eliminated.
- Imiquimod 5% or ingenol mebutate.
- Glycol acid 15% lotion
- Topical retinoids
Courtesy Steven Goldberg, MD
α- and β-Papillomavirus infection in a young patient with an unclassified primary T-cell immunodeficiency. JAAD July 2014 Volume 71, Issue 1, Pages 108–115.e1
Familial case in a 15-year-old otherwise healthy black girl. JAAD March 2010 Volume 62, Issue 3, Pages e13–e14
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