Abstract

Desquamative interstitial pneumonia (DIP) is a rare form of interstitial lung disease (ILD) in children that can be idiopathic but is usually associated with an inborn error of surfactant metabolism. We reported DIP in a 13-year-old girl who was referred to our outpatient clinic because of worsening dyspnea. High-resolution computed tomography showed ground-glass attenuation with honeycombing and intralobular, interstitial septal thickening, suggestive of an ILD. Transbronchial lung biopsy was performed, and histopathology findings were consistent with DIP. The patient was started on steroid therapy with oxygen support. Unfortunately, she died a month after being diagnosed due to disease progression.

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