[Bullous congenital ichthyosiform erythroderma in a mother and son]

The occurrence of two cases of bullous congenital ichthyosiform erythroderma (BCIE) in a mother and son is reported. Clinical diagnosis was confirmed by histological and ultrastructural findings, which demonstrated marked changes in the cyto-skeleton of the keratinocytes of the Malpighian layer and areas of cytolysis and hypoplasia of the tonofilament-hemidesmosome complexes in the cells of the granulosa layer. These results and the possible aetiopathogenic mechanisms are discussed in the light of the most recent data in the literature. Treatment with oral etretinate proved to be helpful, but not long lasting.