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September 1978

Neurofibromatosis and Interstitial Lung Disease

Author Affiliations

From the Departments of Dermatology (Dr Davis) and Medicine (Dr Kaplan), Veterans Administration Hospital and the University of California, San Francisco. Dr Kaplan is now at the University of Colorado Medical Center, Denver.

Arch Dermatol. 1978;114(9):1368-1369. doi:10.1001/archderm.1978.01640210063020

• The literature suggests that 10% to 20% of adult patients with neurofibromatosis have associated interstitial lung disease. Characteristics of such involvement, as present in the case reported herein, include bilateral lower lobe fibrosis and may include bullous and cystic changes in advanced cases. In addition to pulmonary fibrosis, neurofibromatosis may have other intrathoracic associations; including "dumbbell" neurofibromas, intercostal neurofibromas, and intrathoracic meningoceles.

(Arch Dermatol 114:1368-1369, 1978)