Skin changes in sarcoidosis

Overview

Sarcoidosis - multisystem granulomatous disease; skin involved in ~25-35% of cases
Cutaneous lesions divided into specific (contain non-caseating granulomas) and non-specific (reactive, no granulomas)

Presentation

Lupus pernio - violaceous (purple-red), indurated plaques on nose, cheeks, ears, lips; strongly associated with chronic systemic sarcoidosis (pulmonary fibrosis, bone cysts, uveitis); specific lesion
Maculopapular eruption - most common specific lesion; small red-brown papules around periorbital skin, nasolabial folds, mouth
Scar sarcoidosis - pre-existing scar or tattoo becomes raised, indurated, and purple; highly specific clinical clue
Subcutaneous nodules (Darier-Roussy) - deep, painless, flesh-coloured nodules on trunk and extremities
Erythema nodosum - tender, warm, erythematous nodules on shins (anterior); bilateral; do not ulcerate; fade to bruise-like hue over 4-6 weeks; non-specific lesion
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Löfgren syndrome - erythema nodosum + bilateral hilar lymphadenopathy + uveitis + polyarthralgia (ankle arthritis) = acute sarcoidosis; excellent prognosis, remission rate ~80-90% without treatment.

Investigations

🥇 First-line

Skin biopsy of specific lesion - non-caseating epithelioid granulomas, no central caseation (gold standard)
Chest X-ray - bilateral hilar lymphadenopathy (most common systemic finding)
Serum ACE - elevated in ~60% of active disease; useful for monitoring, not diagnostic alone
Serum/urinary calcium - hypercalcaemia from granuloma-mediated 1-alpha-hydroxylase activity

🥈 Second-line

CT chest, ophthalmology slit-lamp (uveitis), pulmonary function tests
⚠️
Always exclude TB before starting immunosuppression - both cause granulomas and erythema nodosum. Key histological difference: caseating necrosis in TB vs no necrosis in sarcoidosis. Perform tuberculin skin test and/or IGRA first.

Differential diagnosis

Key differentials for facial/granulomatous skin lesions
ConditionKey featuresDistinguishing point
Discoid lupus erythematosusErythematous plaques, central atrophy, follicular plugging, scarring on sun-exposed areasPhotosensitivity; positive ANA/anti-dsDNA; no granulomas
RosaceaFacial erythema, papules, pustules, telangiectasia on nose and cheeksNo granulomas on biopsy; no systemic disease
Cutaneous TB (lupus vulgaris)Granulomatous skin lesionsCaseating granulomas; positive IGRA/tuberculin test
Granuloma annulareAnnular skin-coloured papules on hands/feetNo systemic involvement; palisading granulomas on biopsy

Management

First-line (localised specific lesions): Topical or intralesional triamcinolone - reduces granulomatous inflammation in lupus pernio, plaques, papules
First-line (erythema nodosum/Löfgren): Ibuprofen (NSAIDs) + rest - self-limiting, resolves within ~6 weeks

🥈 Second-line

Oral prednisolone 20-40 mg daily, tapered over months - for extensive/symptomatic specific disease or systemic involvement
Hydroxychloroquine - for skin-predominant disease; requires regular ophthalmological monitoring

🥉 Third-line

Methotrexate or azathioprine (steroid-sparing); infliximab (anti-TNF-α) for refractory disease