Open AccessPulmonary manifestation of systemic mast cell disease
Author(s) -
Schmidt M.,
Dercken C.,
Loke O.,
Reimann S,
Diederich S,
Blasius S,
Heidenreich S.
Publication year - 2000
Publication title -
european respiratory journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.021
H-Index - 241
eISSN - 1399-3003
pISSN - 0903-1936
DOI - 10.1034/j.1399-3003.2000.15.32.x
Subject(s) - medicine , pathology , mast cell , lymph , systemic disease , systemic mastocytosis , pathological , biopsy , sarcoidosis , infiltration (hvac) , bone marrow , lung , reticular connective tissue , disease , immunology , physics , thermodynamics
Systemic mast cell disease is a rare disease of unknown aetiology. Systemic infiltration and proliferation of mast cells in skin, bone marrow, gastrointestinum and lymph nodes is the central pathological feature. This study reports a patient with mastocytosis of the skin (urticaria pigmentosa) for 10 yrs. The patient was referred to hospital for dyspnoea. Chest radiograph showed moderate reticular infiltration of both lungs, computerized tomography revealed multiple lymph nodes of the mediastinum and faint nodular lesions of middle and upper areas of lungs. Transbronchial biopsy demonstrated mast cell infiltration of the lung with formation of mast cell granuloma. According to the current literature, systemic mast cell disease with pulmonary involvement is a very rare entity. After a treatment with interferon α‐2a over 6 months, the patient′s condition and particularly dyspnoea showed improvement in parallel with an amelioration of the lesions as demonstrated by thorax computed tomography.