Photoclinic

A 29-year-old non-smoker woman presented to emergency department of Shariati Hospital with shortness of breath and right-sided chest pain with dry cough since 4 days ago which was acute in onset and progressive in nature. There was no history of fever, paroxysmal nocturnal dyspnea, palpitation or hemoptysis. She had no drug history. On admission to the hospital, the vital signs were stable with decreased breath sounds on the right side of the chest. Cardiovascular and central nervous systems were unremarkable. An electrocardiogram was normal. Chest X-ray showed right-sided pneumothorax. Subsequent Computed Tomography (CT) scan showed diffuse bilateral cystic lesion in both lungs with severe right pneumothorax (Figures 1 and 2). A tube thoracostomy with a 28F tube was placed. Biopsy from apical segment of the upper lobe of the right lung was taken, which showed a portion of lung tissue with marked hemorrhage in alveolar spaces and alveolar walls disruption with thin walled cystic air spaces and patchy, disordered, clustered to nodular proliferation of bland spindled and cuboidal epithelial cell around airways, lymphatic and blood vessels. On Immunohistochemistry (IHC), the proliferating cells were positive for HMB-45/Desmin (Figure 3).