Cutaneous sarcoidosis
Sarcoidosis of skin; cutaneous sarcoid; lupus pernio (variant)
Cutaneous sarcoidosis affects 20β35% of patients with systemic sarcoidosis and is a recognised diagnostic mimic in skin oncology β particularly when presenting as a non-healing reddish-brown plaque on the face mistaken for BCC, an indurated nodule mistaken for cSCC or a violaceous plaque suggesting cutaneous lymphoma. Lupus pernio (a destructive cutaneous variant on the nose, ears or cheeks) carries the strongest association with chronic systemic disease and pulmonary fibrosis. Diagnosis requires histological demonstration of non-caseating granulomas with exclusion of infection (tuberculosis, fungi) and foreign-body reaction, alongside systemic workup.
Clinical features
- Papules β small (2β5 mm) red-brown to violaceous, often grouped on the face (periorbital, nasolabial), neck, and upper trunk.
- Plaques β red-brown indurated plaques, often annular; cheeks, scalp, back.
- Nodules β subcutaneous (Darier-Roussy variant); usually limbs.
- Lupus pernio β violaceous, indurated, often disfiguring plaque on the nose, cheek or ear; strongly associated with chronic systemic disease and pulmonary fibrosis.
- Scar sarcoidosis β granulomatous infiltration of pre-existing scars (vaccinations, tattoos, surgical wounds).
- Erythema nodosum β reactive subcutaneous nodules; usually accompanies acute sarcoidosis (LΓΆfgren syndrome) and is a different histological entity.
Histology
- Non-caseating epithelioid granulomas with sparse surrounding lymphocytic infiltrate ("naked" granulomas).
- Multinucleated giant cells, occasionally with asteroid bodies or Schaumann bodies.
- No caseous necrosis (key distinguishing feature from tuberculosis).
- Special stains for AFB and fungi to exclude infection.
- Polarised light examination to exclude foreign-body reaction.
- Histological diagnosis must be integrated with clinical and systemic findings β granulomatous skin reactions to infection, foreign bodies, malignancy and beryllium can mimic sarcoidosis histologically.
Differential β the cancer mimics
- BCC β pearly papule with telangiectasias; sarcoidal papules lack pearly translucency and have telangiectasias only in late or scarred disease.
- cSCC β keratotic, ulcerated; sarcoidal nodules are firm but lack keratin.
- Cutaneous lymphoma β particularly granulomatous variants; biopsy and T-cell receptor / B-cell receptor clonality help.
- Discoid lupus erythematosus β atrophic, scarring, immunofluorescence-positive.
- Cutaneous tuberculosis β caseating granulomas, AFB-positive.
- Necrobiosis lipoidica, granuloma annulare, foreign-body granuloma, leishmaniasis, secondary syphilis.
- Biopsy is the rule β cumulative cosmetic morbidity from repeated biopsies in a patient with multiple sarcoidal plaques is unavoidable but worthwhile.
Systemic workup
- Bloods β FBC, U&E, calcium (hypercalcaemia), serum ACE (limited specificity), 25-OH-vitamin D.
- Chest imaging β chest X-ray Β± high-resolution CT for pulmonary involvement.
- Pulmonary function tests, including diffusing capacity.
- ECG Β± Holter / cardiac MRI for cardiac sarcoidosis.
- Ophthalmology review β uveitis common.
- 24-h urinary calcium where serum calcium is elevated.
- Bronchoscopy with biopsy / endobronchial ultrasound if pulmonary disease β generally led by respiratory medicine.
Management
- Limited cutaneous disease β topical corticosteroids (potent), topical tacrolimus, intralesional triamcinolone.
- More extensive cutaneous disease β hydroxychloroquine, methotrexate, oral corticosteroids.
- Lupus pernio β often refractory; oral steroid + steroid-sparing agent (methotrexate, mycophenolate), TNF-Ξ± inhibitors (infliximab, adalimumab) in refractory disease.
- Surgery is generally avoided β sarcoidosis demonstrates the KΓΆbner phenomenon, with recurrence in scars; reserve for selected disfiguring lesions after disease control.
- Multidisciplinary β respiratory, rheumatology, ophthalmology, cardiology.
References
- MaΓ±Γ‘ J et al. Cutaneous sarcoidosis β review. J Am Acad Dermatol; 2020.
- Marchell R, Judson MA. Sarcoidosis β cutaneous manifestations. Clin Chest Med; 2010.
- British Thoracic Society. BTS Clinical Statement on pulmonary sarcoidosis. Thorax. 2021;76(1):4-20.
- British Association of Dermatologists. Sarcoidosis patient information leaflet. BAD, accessed 18 May 2026.
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