A case of epidermodysplasia verruciformis (EV) from India1 has created considerable interest, as it affected 14 individuals from three generations of one family. This genetic disease often runs in families and the mode of inheritance is usually, with a few exceptions, autosomal recessive. 2 Contrary to that, the inheritance was mostly dominant in the described family. One explanation might be consanguinity in some former generations. However, the family tree is nonconclusive. Typical features of EV are widespread plane warts and pityriasis versicolor-like lesions with a highly characteristic cytopathic effect: clarification of epidermal cells starting from the suprabasal layer; more pronounced in the upper layers. In the described case such lesions appeared at the age of 5 years and carcinoma developed at the age of 27 years. Malignancies and premalignancies (actinic keratoses, Bowen’s type carcinoma in situ) originating from benign lesions usually start to appear in the third to fourth decades, ie 20–30 years after the onset of the disease. 3 Thus the malignant transformation is a very long-lasting process, similar to the progression of cervical intraepithelial neoplasia into invasive tumors. One characteristic is preferential localization of malignancies at the sun-exposed areas as a result of the immunosuppressive and DNA-damaging effects of ultraviolet irradiation. In spite of the almost generalized skin involvement, genital and buccal mucosa are not involved. The general condition of the patients remains satisfactory. The profound defect of cell-mediated immunity (CMI) is specific towards the causative viruses. The immunotolerance, ie inability to recognize one’s HPVs and reject the viralinduced lesions, leads to a life-long disease. Causative HPVs differ from all other (almost 100) characterized cutaneous and genital HPVs, and are referred to as

EV-specific viruses. Among more than 20 known EV-specific HPVs, ie HPVs found in lesions of EV, 4–6 only some have oncogenic potential, mainly HPV5 and 8, and much more rarely HPV14, 17, 20 and 47. However, in EV cancers diverse EV HPVs may be detected in addition to HPV5 and 8. Moreover, a remarkable heterogeneity of oncogenic EV HPVs within the same HPV type is a frequent phenomenon. 5, 7 The characteristic feature of oncogenic EV HPVs is that–in contrast to oncogenic genital HPVs8–viral DNA is not integrated into the host DNA, E6 oncoprotein does not degrade antioncogene p53, and oncoprotein E7 has very low transforming activity. 9, 10 Thus the mechanism of EV carcinogenesis is still not fully established and differs substantially from the much better explored genital oncogenesis. Besides EV HPVs, a not infrequent finding in EV is the presence of HPV3 or HPV10; viruses responsible for flat warts in the general population. These HPVs are often detected also in widespread warts in the immunosuppressed population, and a life-long infection with generalized plane warts has clinical and histologic characteristics of EV associated with HPV3. 11, 12 From following such cases for several years, in some we have seen the appearance of EV HPVs simultaneously with malignant transformation. The Indian authors have not provided virological data, however, the development of cancer is, as a rule, associated with oncogenic EV HPVs, which are identical in all continents.