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Sarcoidosis as a rare cause for symmetrical giant bullous disease


Jungraithmayr, Wolfgang; Leggeri, Elisa; Weder, Walter; Vrugt, Bart (2017). Sarcoidosis as a rare cause for symmetrical giant bullous disease. BMC Pulmonary Medicine, 17(1):1-3.

Abstract

BACKGROUND: Sarcoidosis presents with typical clinic-radiological findings and shows histologically non-caseating granulomas. Pulmonary manifestations of sarcoidosis can be diverse, involving the intrathoracic lymph nodes and pulmonary parenchyma.
CASE PRESENTATION: We here describe a case of a 35-year-old patient who presented with a history of exertion dyspnoea and coughing for the past 20 years. At the age of 15, she was exposed to smoke emanating from a fire. Later, she had exposure to mold for two years, and during her childhood, she had animals such as a cockatiel, dog, cat, gecko, and turtle. Computed tomography of the chest revealed symmetrical apical giant bullous lesions. Histology of the resected bullae showed prominent peribronchial fibrosis with non-necrotizing, non-caseating granulomas and collaps of pulmonary lobules adjacent to the bulla. The absence of granulomatous infection and a markedly elevated CD4:CD8 ratio in bronchoalveolar lavage analysis suggested that the underlying process was sarcoidosis.
CONCLUSION: In very rare cases, sarcoidosis can be associated with bilateral symmetrical apical giant bullous disease due to fibrotic and granulomatous changes resulting in a restriction of lung tissue.

Abstract

BACKGROUND: Sarcoidosis presents with typical clinic-radiological findings and shows histologically non-caseating granulomas. Pulmonary manifestations of sarcoidosis can be diverse, involving the intrathoracic lymph nodes and pulmonary parenchyma.
CASE PRESENTATION: We here describe a case of a 35-year-old patient who presented with a history of exertion dyspnoea and coughing for the past 20 years. At the age of 15, she was exposed to smoke emanating from a fire. Later, she had exposure to mold for two years, and during her childhood, she had animals such as a cockatiel, dog, cat, gecko, and turtle. Computed tomography of the chest revealed symmetrical apical giant bullous lesions. Histology of the resected bullae showed prominent peribronchial fibrosis with non-necrotizing, non-caseating granulomas and collaps of pulmonary lobules adjacent to the bulla. The absence of granulomatous infection and a markedly elevated CD4:CD8 ratio in bronchoalveolar lavage analysis suggested that the underlying process was sarcoidosis.
CONCLUSION: In very rare cases, sarcoidosis can be associated with bilateral symmetrical apical giant bullous disease due to fibrotic and granulomatous changes resulting in a restriction of lung tissue.

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Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Institute of Pathology and Molecular Pathology
04 Faculty of Medicine > University Hospital Zurich > Clinic for Thoracic Surgery
Dewey Decimal Classification:610 Medicine & health
Scopus Subject Areas:Health Sciences > Pulmonary and Respiratory Medicine
Language:English
Date:30 May 2017
Deposited On:13 Jun 2017 13:05
Last Modified:21 Nov 2023 08:05
Publisher:BioMed Central
ISSN:1471-2466
OA Status:Gold
Free access at:PubMed ID. An embargo period may apply.
Publisher DOI:https://doi.org/10.1186/s12890-017-0429-z
PubMed ID:28558711
  • Content: Published Version
  • Licence: Creative Commons: Attribution 4.0 International (CC BY 4.0)