SYSTEMIC XANTHOHISTIOCYTOMA - A VARIANT OF XANTHOMA DISSEMINATUM

We present a 66-year-old man who had maculopapular pigmented lesions o n the skin of the head, neck and trunk suggesting generalized eruptive histiocytoma (GEH), These lesions had a yellowish centre in a target- like pattern that has not been previously described, The patient suffe red from diplopia and had a severe sensorimotor polyneuropathy causing progressive paresis of the limbs, The explorations performed disclose d the presence of specific xanthomatous infiltrates in the skin, lungs , respiratory tract, peripheral nerves and meninges, suggesting xantho ma disseminatum (XD) or juvenile xanthogranuloma, Multiple osteolytic lesions of large bones were also found, The infiltrate was CD68, MAC 3 87 and factor XIIIa positive and S-100 and CD1 negative, Some cells co ntained worm-like bodies visible by electron microscopy. Our patient p resented clinical and immunohistochemical findings suggestive of GEH, juvenile xanthogranuloma or XD, supporting the idea of a wide spectrum of non-langerhans cell histiocytosis. These specific target-like xant homatous lesions seem to be unique for this new variant of XD.