Usual interstitial pneumonia complicating dyskeratosis congenita.

摘要: Dyskeratosis congenita (DC) is a rare disorder characterized by skin hyperpigmentation, nail dystrophy, and leukoplakia of mucous membranes. Pulmonary complications occur in approximately 20% patients, although the specific histopathologic features, temporal relationship between diagnosis DC development pulmonary fibrosis, response to treatment are largely undefined. We describe 2 patients with who developed usual interstitial pneumonia. fibrosis 18 38 years after original manifestations DC. Both died respiratory failure, 4 6 months lung biopsy. may be linked underlying abnormalities fibroblast function.