EPIDERMODYSPLASIA VERRUCIFORMIS

By Gary M. White, MD

Epidermodysplasia verruciformis


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.

Clinical

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.

Treatment

For the inherited type, sun protection is critical. Patients can die from metastatic SCC if they do not diligently protect themselves from the sun.

Additional Pictures

Epidermodysplasia verruciformis

Courtesy Steven Goldberg, MD
Epidermodysplasia verruciformis

References

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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