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Peter Helmbold, MD; Bernd Hegemann, MD; Hans-Jürgen Holzhausen, MD; Thomas Klapperstück; Wolfgang Ch. Marsch, MD

Arch Dermatol. 1998;134:1275-1278.

Background  The purpose of this study was to test the disease-controlling effect of low-dose oral etoposide monotherapy in adult-onset multisystem Langerhans cell histiocytosis. There are no previous reports of low-dose etoposide monotherapy for this condition.

Observations  A 27-year-old man with a 7-year history of multifocal chronic Langerhans cell histiocytosis presented with severe disabling ulcers in intertriginous areas. He had previously been treated with 2 different regimens of antitumoral chemotherapy; one had to be discontinued due to myelosuppression and the other had proved ineffective. We treated with oral etoposide monotherapy at 50 mg/d (22 mg/m² per day) for 21 days. The treatment was repeated at 28-day intervals for a total of 6 cycles. A rapid initial response with subtotal diminution of the involved skin area was found. No adverse effects were observed. The clinical picture has remained stable during the 7 months following cessation of therapy.

Conclusion  Low-dose oral etoposide treatment is an adequate therapeutic measure for prolonged disease control in adult-type Langerhans cell histiocytosis.

From the Department of Dermatology (Drs Helmbold, Hegemann, and Marsch and Mr Klapperstück) and the Institute of Pathology (Dr Holzhausen), Martin-Luther-Universität Halle-Wittenberg, Halle, Germany.

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