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Updated: May 20, 2026

Minimal Erythema Dose (MED) Testing
Published on: May 28, 2013
[Erdheim-Chester disease: study of 12 cases]
Montserrat Juanós Iborra1, Albert Selva-O'Callaghan, Javier Solanich Moreno
1Servicio de Medicina Interna, Hospital General Universitario Vall d'Hebron, Departamento de Medicina, Universitat Autònoma de Barcelona, Barcelona, Spain.
Background And Objective:
Erdheim-Chester disease (EC) is a rare form of non-Langerhans' cell histiocytosis. It is characterized by the xanthomatous infiltration of tissues with foamy CD68+/CD1a- histiocytes. We report a series of 12 patients diagnosed with EC.
Patients And Methods:
We reviewed the clinical, pathological and therapeutic aspects of 12 cases diagnosed with EC at 7 tertiary teaching hospitals in Spain. Patients were included if tissue infiltration by histiocytes CD68+/CD1a- could be demonstrated in an appropriate clinical setting.
Results:
Twelve patients (7 male) were included. Median follow-up was 36 months (IQR: 20-84). The median age at the time of clinical onset and pathological diagnosis was 49 (IQR: 28-61) and 56 years (IQR: 37-62), respectively. In 6 cases multiples biopsies were performed (skin, muscle, testicular) previous to diagnosis, which was confirmed in 3 cases after a carefully review of pathological tissues. Neurological involvement was independently associated with mortality (P<.05). Characteristic long bone osteosclerosis was detected in 9 patients.
Conclusion:
EC is a multisystemic and heterogeneous clinicopathological condition. A high index of suspicion and fluent communication between clinicians and pathologists is necessary to achieve a correct diagnosis.
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