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Erdheim-chester disease.

Myoung-Shin Kim¹, Chae-Hwa Kim, Seok-Joo Choi

¹Department of Dermatology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Annals of Dermatology

|December 18, 2010

Summary

Erdheim-Chester disease (ECD), a rare histiocytosis, presents with multi-organ involvement. This case highlights facial and scalp lesions, bone lesions, and perirenal infiltrates, confirming ECD with extraskeletal manifestations.

Keywords:

Erdheim-Chester disease Histiocytosis Non-Langerhans cell

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Area of Science:

Histiocytosis
Rare diseases
Dermatopathology

Background:

Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis of unknown cause.
It characteristically involves multiple organs, including skeletal and extraskeletal sites.
Diagnosis typically relies on imaging and histopathology.

Observation:

A 68-year-old man presented with facial and scalp lesions, diabetes insipidus, cough, and dyspnea.
Radiological findings included osteosclerotic bone lesions, pericardial effusion, and perirenal infiltrates.
Histopathology revealed foamy histiocytes in skin and perirenal tissues.

Findings:

Skin biopsy showed dermal infiltration of foamy histiocytes and multinuclear giant cells.
Perirenal biopsy confirmed fibrosis with CD68-positive, S100-negative foamy histiocytes.
The patient was diagnosed with Erdheim-Chester disease with extraskeletal involvement.

Implications:

This case underscores the diverse clinical presentations of ECD.
Early diagnosis and recognition of extraskeletal manifestations are crucial for management.
Interferon-alpha was initiated as treatment for this patient.