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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.
Medicina Clinica
|July 17, 2012
Summary
Erdheim-Chester disease (EC), a rare histiocytosis, involves xanthomatous infiltration. This study highlights its heterogeneous nature and the importance of clinical-pathological communication for accurate diagnosis in 12 patients.
Area of Science:
- Histiocytosis
- Pathology
- Rare Diseases
Background:
- Erdheim-Chester disease (EC) is a rare non-Langerhans' cell histiocytosis.
- Characterized by xanthomatous infiltration with foamy CD68+/CD1a- histiocytes.
- A multisystemic and heterogeneous clinicopathological condition.
Purpose of the Study:
- To report a series of 12 patients diagnosed with Erdheim-Chester disease.
- To review clinical, pathological, and therapeutic aspects of EC.
- To emphasize diagnostic challenges and necessary communication.
Main Methods:
- Retrospective review of 12 EC cases from 7 Spanish tertiary hospitals.
- Inclusion criteria: demonstrated histiocyte infiltration (CD68+/CD1a-) in a clinical context.
- Analysis of clinical, pathological, and therapeutic data.
Main Results:
- Twelve patients (7 male) were included, with a median follow-up of 36 months.
- Median age at onset was 49 years; diagnosis at 56 years.
- Neurological involvement was linked to mortality; 9 patients had osteosclerosis.
Conclusions:
- Erdheim-Chester disease is a complex, multisystemic condition.
- Accurate diagnosis requires a high index of suspicion.
- Effective communication between clinicians and pathologists is crucial for diagnosis.
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