Unusual cases of Meleda keratoderma treated with aromatic retinoid etretinate

We studied a brother and sister of South Italian origin with palmoplantar keratoderma which, because of the clinical aspects (transgrediens et progrediens), mode of inheritance (autosomal recessive) and evolution, we diagnosed as Meleda keratoderma with particular clinical features (presence of ainhum-like narrowings and concomitant lesions at other sites). Appropriate classical histologic and familial investigations, as well as HLA typing were done, and retinol-binding protein values were determined. The patients were treated with the aromatic retinoid etretinate, and the clinical and histological results after 9 months' treatment are reported.