Thirty cases of X-linked ichthyosis (XLI) and 32 cases of ichthyosis vulgaris (IV) were diagnosed by measuring the steroid sulphatase activity of peripheral blood lymphocytes or the electrophoretic mobility of serum LDL or both. The clinical features of the two conditions were then compared. In both diseases 60-66% of patients had a family history of the condition. Ichthyosis was noted at birth or immediately afterwards in 59% of the patients with XLI while it appeared in infancy in 68% of those with IV. Scales were mostly large and brown or dark brown in patients with XLI, while the majority of patients with IV had small brown or light brown scales. The distribution of the ichthyotic lesions differed in the two types of ichthyosis. On the trunk, the abdomen was more severely involved than the back in 63% of the cases with the XLI, whereas the back was more scaly than the abdomen in 44% of those with IV. On the extremities, the extensor surface was more severely affected than the flexor surface in both types. X-linked ichthyosis was characterized by the presence of lesions in the pre-auricular area, which were found in 93% of the cases with XLI, while only 17% of the IV patients had ichthyotic lesions at this site. Involvement of the preauricular area could be an important clinical feature for distinguishing XLI from IV.
Virological studies were performed on 12 patients with epidermodysplasia verruciformis (EV). Three types of lesions were observed: red plaques, pityriasis versicolor (PV)-like macules and plane warts. Human papillomavirus (HPV) 14, 20 and 21 were isolated from the plaques, HPV 3, 14 and 38 from flat warts and HPV 5, 12, 17, 20 and 38 from PV-like lesions. No clear relationship could be established between the different lesions and the types of HPV. Types 17 and 20 have been isolated most frequently from Japanese EV patients and HPV 5, frequently detected in other countries, is less common, whereas HPV 8 has not been isolated. Skin cancers occurred in six of the cases (50%) and all had benign lesions that were PV-like. At least one type of HPV 5, 17 or 20 could be isolated from these benign lesions and HPV 17 or 20 detected in the cancers. These three types of HPV in EV patients appear to be involved in the malignant transformation.
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