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Generalized cowpox infection in a patient with Darier disease

ence, both dorsal and ventral aspects can be severely involved and the parallel linearity of the dorsal hands is very specific. The linearly arranged hyperkeratotic papules of the flexures, the sine qua non of the syndrome, are not always present. Hypotrichosis with woolly hair is caused by mutations in either the LIPH or the LPAR6 gene. As the woolliness of the hair may be conspicuous only at an early age, the correct differentiation from isolated autosomal recessive hypotrichosis may be difficult. Whenever a patient with a set of clinical signs and symptoms is examined, we try to look for one unifying diagnosis. In this case, clinical examination of individual II-1 did not reveal an obvious entity. Examination of the extended family disclosed two coexistent disorders, with only a minority of the affected individuals having both. The strength concealed in large families for getting to correct clinical diagnoses, as well as for molecular analysis of hereditary disorders, is reemphasized.