[Congenital histiocytosis X. Papulo-nodal necrotic form].

A child with congenital cutaneous form of Histiocytosis X - the Letterer-Siwe disease - is reported. Since birth there is a papulo-nodular and necrotic eruption, involving the entire skin. No changes in internal organs were discovered. The histologic investigation of skin lesions revealed infiltrates of histiocytes with considerable polymorphism. In spite of the treatment with antibiotics, the skin lesions deteriorated with an enlargement of the necrosis. On the 19th day after delivery a corticosteroid treatment was started, which continued for 60 days. The skin lesions improved considerably and gradually disappeared completely. The child was followed up for 7 1/2 years. No relapses or new skin lesions were observed.