Primary cutaneous Langerhans cell histiocytosis in an adult
D. J. Lichtenwald, H. R. Jakubovic and D. Rosenthal
Department of Medicine, McMaster University, Hamilton, Ontario, Canada.
An unusual adult case of Langerhans cell histiocytosis is presented. The
case initially mimicked granuloma annulare, both clinically and
histologically. The patient subsequently had development of extensive
cutaneous involvement that pathologically revealed a diffuse infiltrate of
CD1-positive histiocytic cells containing Langerhans granules. Extensive
investigations failed to detect systemic involvement. The patient's
cutaneous eruption did not respond to various therapeutic interventions,
including phototherapy with oral psoralen with long-wave UV radiation in
the A range (PUVA) and systemic vinblastine sulfate. Marked but temporary
clinical and histologic improvement was achieved with total body electron
beam radiotherapy. The nosology of this case is discussed in the context of
the various histiocytic proliferative disorders.
