Nonspecific Cell‐Mediated Immunity in Patients with Epidermodysplasia Verruciformis

Abstract

Epidermodysplasia verruciformis (EV) is a rare disease that usually begins in childhood and is characterized by a generalized infection by human papilloma virus (HPV), frequent associations with cutaneous carcinomas, and abnormalities of cell-mediated immunity (CMI). We studied nonspecific CMI in 13 patients with EV by bacterial skin tests, allergic reactions to dinitrochlorobenzene (DNCB), measurement of responses to phytohemagglutinin (PHA), and quantification of T lymphocytes and T lymphocytes subsets in peripheral blood. Impairment of CMI was manifested by the cutaneous anergy to a variety of common skin antigens and, by the reduction of the lymphocyte transformation to PHA. There were no correlation between the severity of cases and abnormalities of CMI in our patients, however; the impairment of CMI was lower in cases of short duration, suggesting that the impairment of CMI in EV might reflect a long period of disease.

Merkel cell polyomavirus in Merkel cell carcinoma from a Brazilian epidermodysplasia verruciformis patient

“…Impairment of cell-mediated immunity was manifested as cutaneous anergy to common skin antigens and by a reduction of the lymphocyte transformation response to phytohemaglutinin. 4 Multiple BCCs and SCCs developed between 25 to 28 years of age, predominantly on sun-exposed skin. These tumors grew rapidly and were highly destructive.…”

Multifocal leiomyosarcomatosis in a 6-year-old child with epidermodysplasia verruciformis and immune defect

“…Although the possibility of the presence of an underlying primary immune deficiency cannot be excluded at this stage, these findings may well be because of the presence of chronic HPV infection and leiomyosarcoma. In fact, impairment of cell-mediated immunity (eg, cutaneous anergy and decreased lmyphoproliferation with mitogens) with EV has been described previously [14,15]. Because our case has been on chemotherapy, we did not repeat immunologic studies.…”

Systemic lupus erythematosus arising in a patient with epidermodysplasia verruciformis

“…Impairment of cell-mediated immunity (CMI) in patients with EV is manifested by the cutaneous anergy to a variety of common skin antigens and by the reduction of the lymphocyte transformation to phytohemagglutinin (PHA). 13 The onset of the clinical manifestation (warts and pityriasis versicolor-like lesions) in our patient and her sister was at the age of 6 and 9 years, respectively. This agrees with the findings of other workers 14 that the onset usually manifests in childhood.…”