[Sarcoidosis associated with lupus pernio and acute pulmonary cavitation]

Abstract

Lupus pernio, an uncommon skin condition due to sarcoidosis in Japan, is a chronically persistent, violaceous skin lesion primarily involving the nose, cheek, ears, and fingers. It has often been associated with chronic fibrotic non-resolving pulmonary sarcoidosis. We reported a case of sarcoidosis associated with lupus pernio and primary pulmonary cavitation as a rare manifestation in the lung. A 44-year-old man visited our hospital in January 1986 because of a 3-year history of swollen and violaceous cheeks (lupus pernio) and an 8-year history of erythema in both upper and lower limbs. The biopsy specimen obtained from the skin lesion revealed epithelioid cell granulomas without any evidence of Mycobacterium or fungal growth. Serum ACE (45.4 U/ml) was elevated. Chest X-ray films and computed tomographic (CT) scans showed bilateral hilar lymphadenopathy, nodules, multiple small opacities, and reticulo-linear opacities. Gallium scintigraphy demonstrated abnormal uptake in the mediastinal and hilar lymph nodes and lung fields. The patient had not received corticosteroids. Chest X-ray films and CT scans in July 1989 showed increased opacities and cavitation. Because pyogenic bacteria and acid-fast bacilli were not detected by repeated sputum examinations or bronchial washing, we concluded that the cavitary lesions were manifestations of primary pulmonary cavitation due to sarcoidosis. Prednisolone (40 mg daily) was prescribed and the dosage was gradually tapered. The lung lesions gradually resolved and have not recurred.