34244 Acquired epidermodysplasia verruciformis: A retrospective review of cases

Abstract

Epidermodysplasia verruciformis is a rare genodermatosis characterized by an unusual susceptibility to the beta genus of the human papillomavirus and early development of skin cancers. Acquired epidermodysplasia verruciformis (AEDV) is a distinct rare disorder with similar clinical and histopathologic manifestation, strongly associated with immunosuppression. Clinical course, therapeutic management, and potential for skin cancer development in AEDV is largely unknown. We report 7 patients with AEDV diagnosed between January 1992 and November 2019. Four patients were males (57.1%), with median age of diagnosis of 29 years, range 9-67 years. All patients had underlying immunodeficiency; 5 with acquired immunodeficiencies (HIV/AIDS, heart transplant, kidney transplant, pancreas transplant, systemic lupus erythematosus and Crohn’s disease) and 2 with congenital immunodeficiencies (isolated CD4 lymphopenia and immunodeficiency 71 with inflammatory disease and congenital thrombocytopenia). For patients with acquired immunodeficiencies, the median interval between the onset of immunosuppression and the presentation of AEDV lesions was 5 years, range 3-7 years. Patients were followed for a median of 5.5 years, range 1-10 years. Only 1 patient (14.2%) progressed to develop squamous cell carcinoma of the left forearm. Topical imiquimod (3/7), topical tretinoin (3/7), cryotherapy (2/7), intralesional candida injection (2/7), and topical podophyllotoxin (1/7) were attempted for treatment of AEDV. Partial resolution of AEDV lesions was observed with intralesional candida injection (2/2) and topical retinoid (1/3). Initiation of HAART yielded complete resolution in one patient with HIV/AIDS. In conclusion, we report a series of AEDV with emphasis on therapeutic management, clinical course, and long-term follow up of this rare disorder.