Erdheim-Chester disease.
A 38-year-old man presented with numerous dermal nodules, similar to xanthoma disseminatum, that were histologically consistent with his diagnosis of Erdheim-Chester disease, a non-Langerhans cell histiocytosis. Other cutaneous manifestations of this disease include eyelid xanthelasma, pretibial dermopathy and pigmented lesions of the lips and buccal mucosa. The histological diagnosis of Erdheim-Chester disease was originally made on the patient's retroperitoneal tissue, obtained at a laparotomy for surgical treatment of a presumed phaeochromocytoma, and confirmed by the pathognomonic long bone X-ray findings of this disease.
Opie
Kimble M
KM
Department of Dermatology, Royal Perth Hospital, Perth, Western Australia, Australia.
Kaye
Joey
J
Vinciullo
Carl
C
D000328:Adult; D001706:Biopsy; D031249:Erdheim-Chester Disease / Q000175:diagnosis* / Q000601:surgery; D005269:Femur / Q000000981:diagnostic imaging; D006801:Humans; D008297:Male; D011859:Radiography; D013977:Tibia / Q000000981:diagnostic imaging; D016896:Treatment Outcome