Multicystic dysplastic kidney in association with congenital ichthyosiform erythroderma

Multicystic dysplastic kidney is a noninherited congenital disease. Association of this disease with abnormalities of various organs is common. We, however, report a rare case of multicystic dysplastic kidney associated with congenital ichthyosiform erythroderma in an infant. Different developmental origins of the skin and the kidney can explain the scarcity of concurrent congenital skin and kidney abnormalities. Nonetheless, the development of both organs depends on mesenchyme-epithelial interactions for inductive signaling. It seems defects in the production of signaling molecules can explain such an association.