391 SKIN LESIONS IN LANGERHANS CELL HISTIOCYTOSIS

Abstract

In considering the clinical features of Langerhans cell histiocytosis (LCH), a proliferation of Langerhans cells, an entire spectrum of findings may be encountered, from minor skin lesions to the diffuse infiltration of abdominal organs associated with continuous fever, weight loss and organ dysfunction. Cutaneous lesions are regarded as a common finding and, not infrequently, the first symptom in LCH. The lesions may have a variety of appearances; most common are brown to red papules which may show vesiculation, ulceration, crusting, or a hemorrhagic appearance. From 1975 to 1993 we reviewed 79 patients with LCH at the Sophia Children's Hospital in Rotterdam and at the Emma Children's Hospital/Academical Medical Center in Amsterdam. Eight patients were diagnosed with pure cutaneous LCH. Four patients were just observed and regressed spontaneously, the other four received a form of treatment. In a later stage one patient progressed to disseminated LCH with lung involvement. Clinical features at presentation of this patient did not distinguish from the other 7 patients. Of a group of 71 patients with other forms of LCH 28 patients developed skin involvement as part of the disease. The lesions resolved in the patients who were treated with chemotherapy. In those patients who did not receive chemotherapy the skin lesions were either observed or treated locally. We conclude that all patients with LCH apparently localized to the skin should initially be observed, however they need careful staging to assess whether it is part of a disseminated disease.