Source: Annual Congress 2012 - The good clinical practice: useful case report
Disease area: Interstitial lung diseases

Abstract

Erdheim-Chester disease (ECD) is a rare, non-Largerhans histiocytosis with multisystem involvement. Pulmonary involvement is uncommon. We present a 46-year-old woman who presented with chronic chough, dyspnea and yellowish plaques in the periorbital area. She was previously diagnosed as diabetes insipidus. Chest radiographs showed bilateral diffuse interstitial infiltrates.

Symmetric sclerotic bone lesions, dural nevre thickening were defined on imaging studies. Histopathologic examination of the skin lesions revealed infiltration of CD-68 positive foamy histiocytes. She was diagnosed as ECD with pulmonary, pituitary, skeletal, orbita, central nervous system and skin involvement. Following first-line treatment with corticosteroids her syptoms worsened. Treatment with interferon-alpha was started. She had an marked improvemet in symptoms, radiologic findings and skin involvement under this treatment.

Conclusion: ECD should be considered in the differential diagnosis of interstitial lung diseases. As there is no standard treatment for this disorder, interferon-alpha can be effective in the treatment of Erdheim-Chester disease.

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D. Ediger, A. Gorek Dilektasli, A. Ursavas, D. Koprucuoglu, S. Balaban Adim, G. Gokalp, U. Yayla, E. Ege (Bursa, Turkey). The treatment of a non-Largenhans-cell histiocytosis, Erdheim-Chester disease: A-case report. Eur Respir J 2012; 40: Suppl. 56, 3515

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