Congenital systemic Langerhans cell histiocytosis (report of two cases).

Two cases of congenital systemic Langerhans cell histiocytosis (LCH), diagnosed and treated in our department from June 1995 until May 1996, are described. The cases concern two neonates (one female and one male) born with necrotic lesions and skin nodules. The diagnosis was confirmed by skin biopsy which showed diffuse infiltration by CD1 antigen and S-100 protein positive histiocytes. The babies didn't present with anemia, hepatosplenomegaly or lymphadenopathy. Hepatic and renal function were normal. In both infants skeletal survey showed no lytic lesions but chest X-rays and high resolution computerized tomography (HRCT) scan revealed diffuse mottling of both lung fields. Bone marrow aspiration showed the presence of histiocytes in percentages of 6% and 10%, respectively. Both babies were treated with prednisolone 1 mg/kg body weight for three months. The first child who is 20 months old, is now well with resolution of skin and pulmonary lesions occurring within one month of the initiation of steroids, while the second, who presented spectacular resolution of skin lesions within the first three weeks of therapy, is also in excellent condition five months after completion of treatment. We conclude that congenital LCH has to be suspected in neonates with persisting skin lesions. If the disease is systemic but without organ dysfunction, treatment with steroids may be beneficial.