Langerhans cell histiocytosis in the elderly

Abstract

Disseminated Langerhans-cell-histiocytosis (LCH) is most frequent in children at the age of 1-3 years, seldomly seen in adults and extremely rare in the elderly. The clinical course may be acute, subacute or chronic, progressive or stationary. Spontaneous remissions are possible, but rare. In elderly patients often the disease is at first limited to the skin before it becomes systemic. A 73-year-old female patient with chronic stationary disease of 3.5 years duration died 4 weeks after the acute dissemination of her LCH. At the beginning, her skin and liver involvement had responded to chemotherapy with etoposide. Six months later cutaneous relapse occurred with a more disseminated pattern involving the external auditory meatus. Treatment with topical nitrogen mustard followed by thalidomide produced marked improvement. As complications an irritation after topical application of nitrogen mustard and a maculo-papular exanthem after thalidomide were noted. No further visceral involvement was documented for one year. Then the patient developed acute disseminated disease and died within four weeks. As LCH may show a highly unpredictable course with progress and spontaneous remission, the prognosis is difficult. Any therapeutical procedures should be based on the actual state of the disease as determined by careful examination of the organs most commonly involved.