[Epidermodysplasia verruciformis associeted with congenital osseous dysplasia: Fortuitous association or common genetic determinism?]

Epidermodysplasia verruciformis [EV] is a rare recessive autosomal genetic disorder of the immune system characterized by increased susceptibility to cutaneous Human papilloma virus infection. We report a case of an EV associated with a congenital osseous dysplasia and complicated by an epidermoid carcinoma. EV could be associated with others genetics diseases as the chondrodysplasia and the neurofibromatosis. No case has been reported in the literature, associating EV and congenital osseous dysplasia

[ epidemio clinical of lichen sclerosus about 31 cases]

Lichen sclerosus is a chronic inflammatory mucocutaneous disease. Through a retrospective study of all patients with Lichen sclerosus followed between 1997 and 2005 at the dermatology department of La Rabta hospital, we outline the epidemio-clinical aspects of this entity. There were 29 female and two male. Two patients were child. The mean age at the occurrence of adult lichen sclerosus was 51, 5 years. The site of the lesions was perineum and genital in 26 cases [one male and 25 female]. Extragenital involvement was present in 10 patients. All patients underwent topical corticosteroid therapy. Architectural changes of vulva were noted in 7 cases. An epidermoid carcinoma occurred in five patients. The male Lichen sclerosus seem to be rare in our study. It is probably explained by the protective role of the circumcision which is practised in all male children in our country. In our study, the risk of malignant transformation of genital Lichen sclerosus is estimated at 19, 2%. It is lightly more than mentioned in the literature. This may be related to the cofactor role of l-IPV and to an insufficient treatment adherence