TENSION PNEUMOTHROAX AS INITIAL MANIFESTATION OF LUNG INVOLVEMENT IN CUTANEOUS SARCOIDOSIS

Abstract

TOPIC: Lung Pathology TYPE: Medical Student/Resident Case Reports INTRODUCTION: Bullae formation resulting in pneumothorax typically occurs in the late stages of pulmonary sarcoidosis. CASE PRESENTATION: A 56-year-old female with past medical history of hypertension and cutaneous sarcoidosis diagnosed two years ago via skin biopsy. The patient presented with symptoms of heaviness on the right side on the chest, cough, and shortness of breath which started the day before. On examination, the patient was tachypneic, tachycardiac with decreased breath sounds on the right upper and middle lung fields. Chest x-ray showed a right-sided pneumothorax (Figure #1). Computed tomography of the chest (CT) showed a large right pneumothorax measuring approximately 7.5 cm between the visceral and parietal pleura. There were also multiple right upper lobe pulmonary micronodules and biapical bullae, worse on the right lung (figure #2). A Chest tube thoracostomy was performed and patient monitored in the ICU till pneumothorax resolved. Following resolution, a video-assisted thoracoscopic surgery (VATS) with right apical wedge resection of blebs, mechanical and chemical pleurodesis, and chest tube placement was performed. Histology of the right lung apex confirmed non-necrotizing granulomatous reaction from sarcoidosis. The patient improved following surgery and discharged with outpatient follow-up. DISCUSSION: Bullae formation typically presents in advanced pulmonary sarcoidosis with fibrotic changes. In rare cases, bullous disease and subsequent pneumothorax have been reported in the early stage of pulmonary sarcoidosis [1]. Several mechanisms have been proposed for the formation of such bullae; these include bronchial obstruction by sarcoid lesions, causing peripheral air trapping and alveolar distension and rupture, retraction and collapse of surrounding disease lung, and the presence of inflammatory alveolitis causing tissue destruction [2]. Pneumothorax is caused by rupture of these subpleural bullae or necrotic granulomas. Management is by chest tube drainage, bullectomy by thoracoscopic surgery may be required in cases of recurrent pneumothorax [3]. A high index of suspicion should be maintained as pneumothorax can be the presenting feature of early or advanced pulmonary sarcoidosis and predisposes to increased morbidity and mortality. CONCLUSIONS: Bullous disease and pneumothorax are rare presentations of sarcoidosis. Pneumothorax should be suspected in all stages of sarcoidosis in patients with pulmonary symptoms due to high mortality associated with late detection and management. REFERENCE #1: Froudarakis, Marios E., et al. "Pneumothorax as a first manifestation of sarcoidosis." Chest 112.1 (1997): 278-280. REFERENCE #2: Manika, Katerina, et al. "Pneumothorax in sarcoidosis." Journal of thoracic disease 6.Suppl 4 (2014): S466. REFERENCE #3: Keller, Cesar A., et al. "Histopathologic diagnosis made in lung tissue resected from patients with severe emphysema undergoing lung volume reduction surgery." Chest 111.4 (1997): 941-947. DISCLOSURES: No relevant relationships by Ramez Alyacoub, source=Web Response No relevant relationships by Ifunanya Ejikeme, source=Web Response No relevant relationships by CHIDINMA EJIKEME, source=Web Response