TITLE

Usual Interstitial Pneumonia Complicating Dyskeratosis Congenita

AUTHOR(S)
Utz, James P.; Ryu, Jay H.; Myers, Jeffrey L.; Michels, Virginia V.
PUB. DATE
June 2005
SOURCE
Mayo Clinic Proceedings;Jun2005, Vol. 80 Issue 6, p817
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
ABSTRACT
Dyskeratosis congenita (DC) )s a rare disorder characterized by skin hyperpigmentation, nail dystrophy, and leukoplakia of mucous membranes. Pulmonary complications occur in approximately 20% of patients, although the specific histopathologic features, the temporal relationship between the diagnosis of DC and the development of pulmonary fibrosis, and the response to specific treatment are largely undefined. We describe 2 patients with DC who developed usual interstitial pneumonia. Pulmonary fibrosis developed 18 and 38 years after the original manifestations of DC. Both patients died of respiratory failure, 4 and 6 months after lung biopsy. Pulmonary fibrosis in patients with DC may be linked to underlying abnormalities of fibroblast function.
ACCESSION #
17341386

 

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