Cutaneous variants of Langerhans cell histiocytosis in early childhood

The term Langerhans cell histiocytosis (LCH) has been endorsed to describe a group of ra re entities, a highly variable spectrum of clinical presentations which are all characterized by localized or generalized proliferation of pathological Langerhans cells. We describe two infants with LCH who had very distinct cutaneous lesions. A two month-old infant developed discrete, disseminated red-brown nodules and plaques. In contrast, a second child at the age of nine months presented with brown aggregated scaling, crusted and ulcero-necrotic papules mainly restricted to the abdomen. In both patients, dermatohistopathology showed characteristic proliferation of pathologic Langerhans cells,and staging procedures revealed involvement of lung and bones. These two cases illustrate the marked variability of clinical presentation and disease course of LCH. In addition, important aspects and current concepts of LCH are discussed reviewing relevant and recent literature.