Erdheim-Chester Disease
Open Access
- 1 January 2010
- journal article
- Published by Korean Dermatological Association and The Korean Society for Investigative Dermatology in Annals of Dermatology
- Vol. 22 (4), 439-43
- https://doi.org/10.5021/ad.2010.22.4.439
Abstract
Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis of unknown etiology, characterized by multi-organ involvement. ECD is usually diagnosed on the basis of characteristic radiologic and histopathological findings. Lesions may be skeletal and/or extraskeletal in location, and may include the skin, lung, heart, and central nervous system. We describe here a 68-year-old man with multiple yellowish plaques and a pinkish nodule on his face and scalp. He had been previously diagnosed with diabetes insipidus, and recently complained of coughing and dyspnea. Imaging studies showed multiple osteosclerotic lesions of the bones, a moderate amount of pericardial effusion, and multifocal infiltrative lesions in the perirenal space. Histopathological examination of the skin lesions revealed dermal infiltration of foamy histiocytes with multinuclear giant cells. Moreover, laparoscopic biopsy of the perirenal tissue revealed fibrosis with infiltrating foamy histiocytes being CD68-positive and S100-negative. Based on these findings, he was diagnosed with ECD with extraskeletal manifestations, and treated with interferon-alpha.Keywords
This publication has 9 references indexed in Scilit:
- Erdheim–Chester disease: Multisystem involvement and management with interferon-αLeukemia Research, 2010
- Erdheim-Chester disease with predominant mesenteric localization: Lack of efficacy of interferon alphaJoint Bone Spine, 2009
- Systemic Erdheim–Chester diseaseVirchows Archiv A Pathological Anatomy and Histopathology, 2008
- Variability in the efficacy of interferon‐α in Erdheim‐Chester disease by patient and site of involvement: Results in eight patientsArthritis & Rheumatism, 2006
- Erdheim-Chester disease: case report with multisystemic manifestations including testes, thyroid, and lymph nodes, and a review of literatureJournal of Clinical Pathology, 2004
- Treatment of Erdheim-Chester disease with cladribine: a rational approachBritish Journal of Ophthalmology, 2004
- Erdheim-Chester disease: typical radiological bone features for a rare xanthogranulomatosisAnnals Of The Rheumatic Diseases, 2002
- Erdheim-Chester Disease Clinical and Radiologic Characteristics of 59 CasesMedicine, 1996
- Results of Treatment of 127 Patients with Systemic Histiocytosis (Letterer-Siwe Syndrome, Schuller-Christian Syndrome and Multifocal Eosinophilic Granuloma)Medicine, 1981