A 48-year-old African-American woman presents with a 2-year history of slowly enlarging violaceous-red, indurated plaques on the nose, cheeks, and earlobes. Diascopy reveals an "apple-jelly" yellow-brown infiltrate. She reports chronic nasal congestion and hoarseness. Chest imaging shows bilateral hilar lymphadenopathy and restrictive lung disease (FVC 62%). Serum ACE level is elevated at 78 U/L. Skin biopsy reveals non-caseating epithelioid cell granulomas in the dermis with sparse lymphocytic infiltration. AFB and fungal stains are negative; cultures are sterile. The lesions marked **B** in the diagram are consistent with which diagnosis, and what is the most appropriate systemic treatment for this condition?
A. Rosacea with phymatous changes; oral tetracyclines and topical metronidazole
B. Discoid lupus erythematosus; hydroxychloroquine and topical corticosteroids alone
C. Cutaneous tuberculosis (lupus vulgaris); isoniazid and rifampicin monotherapy
D. Lupus pernio of sarcoidosis; systemic corticosteroids with TNF-alpha inhibitors (infliximab/adalimumab) for refractory disease
Explanation
Why "Lupus pernio of sarcoidosis; systemic corticosteroids with TNF-alpha inhibitors (infliximab/adalimumab) for refractory disease" is right
The clinical presentation—violaceous indurated plaques on the nose, cheeks, and earlobes with "apple-jelly" diascopy findings, non-caseating granulomas on biopsy, elevated ACE, bilateral hilar lymphadenopathy, and restrictive lung disease—is pathognomonic for lupus pernio, the most distinctive and prognostically severe cutaneous manifestation of systemic sarcoidosis. The structure marked B represents lupus pernio, which is associated with chronic progressive pulmonary fibrosis, upper respiratory tract sarcoidosis (SURT), and bone cysts. Lupus pernio is notoriously refractory to topical and intralesional steroids alone and requires systemic immunosuppression. Systemic corticosteroids are first-line for active disease, but TNF-alpha inhibitors (infliximab and adalimumab) have the best evidence for refractory lupus pernio and are essential steroid-sparing agents (per ATS/ERS/WASOG Statement on Sarcoidosis and Mañá et al.).
Why each distractor is wrong
Cutaneous tuberculosis (lupus vulgaris); isoniazid and rifampicin monotherapy: While lupus vulgaris also presents with nodules and "apple-jelly" appearance on diascopy, it shows CASEATING granulomas and positive AFB stains. This patient's AFB stains are negative, cultures are sterile, and biopsy shows non-caseating granulomas—ruling out tuberculosis. Lupus vulgaris is marked D, not B.
Discoid lupus erythematosus; hydroxychloroquine and topical corticosteroids alone: Discoid lupus erythematosus (marked A) presents with atrophic scarring and follicular plugging, not violaceous indurated plaques. DLE is an autoimmune condition with different histology (interface dermatitis, not granulomas) and does not cause systemic sarcoidosis features (ACE elevation, hilar adenopathy, restrictive lung disease). Topical steroids alone are insufficient for lupus pernio.
Rosacea with phymatous changes; oral tetracyclines and topical metronidazole: Rosacea (marked C) is a vascular inflammatory condition characterized by telangiectasias and flushing, not granulomatous disease. It does not produce non-caseating granulomas on biopsy, does not elevate ACE, and does not cause systemic manifestations like hilar lymphadenopathy or restrictive lung disease. Tetracyclines and metronidazole are ineffective for sarcoidosis.
High-YieldNEET PG
Lupus pernio = non-caseating granulomas + violaceous plaques on face + chronic systemic disease + refractory to topical steroids → requires TNF-alpha inhibitors for control.
Mañá J et al. Sarcoidosis Vasc Diffuse Lung Dis; ATS/ERS/WASOG Statement on Sarcoidosis
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