CONGENITAL SYSTEMIC LANGERHANS CELL HISTIOCYTOSIS (REPORT OF 2 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 mal e) born with necrotic lesions and skin nodules. The diagnosis was conf irmed 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 func tion were normal. In both infants skeletal survey showed no lyric lesi ons but chest X-rays and high resolution computerized tomography (HRCT ) scan revealed diffuse mottling of both lung fields. Bone marrow aspi ration showed the presence of histiocytes in percentages of 6% and 10% , respectively. Both babies were treated with prednisolone 1 mg/kg bod y weight for three months. The first child who is 20 months old, is no w well with resolution of skin and pulmonary lesions occurring within one month of the initiation of steroids, while the second, who present ed spectacular resolution of skin lesions within the first three weeks of therapy, is also in excellent condition five months after completi on of treatment. We conclude that congenital LCH has to be suspected i n neonates with persisting skin lesions. If the disease is systemic bu t without organ dysfunction, treatment with steroids may be beneficial .