Chronic eosinophilic pneumonia associated with neurofibromatosis type 1: An unusual complication

Abstract

Neurofibromatosis type 1 (formerly known as von Recklinghausen's disease) is an autosomal dominant disorder, which results from the proliferation of the neural crest cells, thus affecting any organ system. Several pulmonary manifestations have hitherto been reported, including chest wall deformities, diffuse lung disease, thoracic neoplasms, pulmonary arterial hypertension, central hypoventilation, diaphragmatic paralysis and meningocele. However, eosinophilic lung disorders have not been described. An unusual case of chronic eosinophilic pneumonia in a patient with neurofibromatosis type 1, is reported herein. He had a propitious outcome, following corticosteroid treatment. This is the first well-documented case of chronic eosinophilic pneumonia and neurofibromatosis type 1 in the same patient. These clinical entities might share common pathogenic mechanisms, as suggested by the present study, that could explain their co-existence.