Lupus Pernio in Sarcoidosis
A 71-year-old man presented to the rheumatology clinic with an 8-year history of enlarging nodules on his nose. He reported no fever, weight loss, or respiratory symptoms, but he had a history of iritis of unknown cause. On physical examination, he had painless, violaceous, indurated nodules on his nose, ears, fingers, and toes. The lungs were clear on auscultation. Laboratory studies were notable for an angiotensin-converting enzyme level of 102 U per liter (reference range, 18 to 55). Computed tomography of the chest revealed hilar and mediastinal lymphadenopathy, diffuse fibrosis, and small nodules. Microbiologic examination of the bronchoalveolar-lavage fluid was negative for bacteria, fungi, and mycobacteria, including Mycobacterium tuberculosis. A skin-biopsy specimen of the nose and a transbronchial-biopsy specimen of the lung both showed noncaseating granulomas. A diagnosis of sarcoidosis with lupus pernio was made. Lupus pernio is a cutaneous manifestation of sarcoidosis that is typically characterized by red-to-purple nodules and plaques on the face, especially on the nose. Early recognition and treatment are paramount in avoiding irreversible nasal destruction. Systemic glucocorticoids alone may not adequately treat the lesions. In this patient, treatment with systemic glucocorticoid therapy was initiated, and at a follow-up visit 6 months after presentation, the nodules had abated.
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