Congenital Letterer-Siwe disease associated with protein losing enteropathy

A case of congenital Letterer-Siwe disease associated with protein losing enteropathy is presented. The patient was born at term after an uneventful pregnancy, and generalized eczematous skin eruptions were observed at birth. Excessive protein loss from the intestine was recognized. A cutaneous biopsy from typical lesions revealed histiocytic infiltrations and Langerhans' cell granules in the cytoplasm of the histiocytes.