A patient with diabetes insipidus and periorbital swellings; Erdheim-Chester disease.
Erdheim-Chester disease is a rare multisystem disease in which a progressive xanthogranulomatous infiltration of several tissues can be seen. We describe a woman, known to have diabetes insipidus for ten years, with periorbital, retroperitoneal, mediastinal, axillar and inguinal involvement. On histological examination a granulomatous infiltration of fatty tissue and striated muscle was seen, consisting of Touton giant cells, histiocytes with foamy cytoplasm and lymphocytes. Immunohistochemical staining with CD-1a and S-100 was negative and on electron microscopy no Langerhans granules were seen. These findings led to the diagnosis of Erdheim-Chester disease. She had a good response on steroids. Because of some similar clinical features of Langerhans cell histiocytosis and Erdheim-Chester disease, a histiocyte disorder seems the most probable cause.
van der Lee
I
I
Department of Internal Medicine, St. Antonius Hospital, CM Nieuwegein, The Netherlands.
Slee
P H
PH
Elbers
J R
JR
D001365:Axilla / Q000473:pathology; D001706:Biopsy; D003919:Diabetes Insipidus / Q000150:complications; D003937:Diagnosis, Differential; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006099:Granuloma / Q000150:complications / Q000473:pathology*; D015614:Histiocytosis / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D008875:Middle Aged; D009915:Orbit / Q000473:pathology; D011241:Prednisone / Q000627:therapeutic use; D012074:Remission Induction; D014973:Xanthomatosis / Q000150:complications / Q000473:pathology*
D005938:Glucocorticoids; D011241:Prednisone