Clinical course and therapy of Langerhans cell histiocytosis in children and adults

The Langerhans cell histiocytosis is a rare disease with a clear preference of infancy and an incidence of 0.2 to 1.0/100000 children occurring worldwide. In 60 to 70% of the cases the first manifestation is before the second year of age. The disease prefers the male sex in a male to female relation of 2:1. Occurrence in adult age is very rare. Up to 10% of the cases are congenital. Both the etiology and the pathogenesis of the disease are unknown. The clinical presentation is extremely variable and covers a wide spectrum from localized involvement with a favorable prognosis to disseminated, malignant and foudroyant course. We report on ten patients, six infants and children and four adolescents and adults. We point out the very variable morphologic manifestations,the different disease course and the therapeutic options.