What are the typical cutaneous manifestations of sarcoidosis and their recommended evaluation and management?

Cutaneous Sarcoidosis Manifestations

Clinical Presentation

Cutaneous sarcoidosis occurs in 20-35% of patients and is frequently the initial manifestation of systemic disease. 1, 2, 3

Specific Lesions (Granulomatous)

These lesions demonstrate non-caseating granulomas on biopsy and include:

• Papules and maculopapular lesions - the most common specific manifestation, appearing as red-brown papules that may coalesce 1, 2, 4
• Plaques - violaceous or hyperpigmented indurated lesions 1, 2
• Lupus pernio - violaceous, indurated plaques characteristically affecting the nose, cheeks, and ears; this is a hallmark of chronic, progressive disease 1, 2
• Subcutaneous nodules - deeper lesions that may be palpable beneath normal-appearing skin 1, 2, 4
• Scar sarcoidosis - granulomatous infiltration of pre-existing scars 2, 4
• Cicatricial sarcoidosis - scarring lesions 1

Non-Specific Lesions

• Erythema nodosum - tender, erythematous nodules typically on the lower extremities; when associated with bilateral hilar lymphadenopathy, this constitutes Löfgren's syndrome, which carries an excellent prognosis with spontaneous resolution 2, 3

Prognostic Significance

The type of cutaneous lesion predicts disease course:

• Favorable prognosis: Löfgren's syndrome (erythema nodosum with hilar adenopathy), maculopapular lesions, and subcutaneous nodules are associated with disease remission within two years 2
• Chronic disease: Plaques and especially lupus pernio indicate chronic, progressive systemic involvement requiring long-term management 2, 3

Diagnostic Evaluation

Initial Assessment

• Skin biopsy is essential - punch biopsy has an 81.6% diagnostic yield with only 4% complication rate, making it a simple, non-invasive method to establish diagnosis early 3
• Biopsy of specific lesions reveals non-caseating granulomas without evidence of infection 1, 2

Laboratory Markers

When systemic involvement is suspected:

• Soluble IL-2 receptor - elevated in 76% of patients with systemic disease 4
• Serum ACE - elevated in only 21% of cutaneous cases but useful for monitoring treatment response when initially elevated 1, 4, 3
• C-reactive protein - elevated in 50% of patients 4
• Hypercalcemia - present in 6% of patients 4

Risk Factors for Systemic Progression

• Female sex and age <54 years are significantly associated with systemic manifestations 4
• Specific cutaneous lesions (versus erythema nodosum alone) predict higher risk of progressive disease 3
• 24% of patients with isolated cutaneous sarcoidosis develop additional organ involvement during follow-up, with progression occurring 4-9 years after initial presentation 5

Systemic Screening

For all patients with cutaneous sarcoidosis:

• Chest radiography or CT - to evaluate for pulmonary involvement and hilar lymphadenopathy 3
• Pulmonary function testing - if respiratory symptoms present (occurs in 72% of patients with specific skin lesions) 3
• Ophthalmologic examination - to screen for uveitis 1
• Long-term monitoring is mandatory - even isolated cutaneous disease can progress to systemic involvement years later, requiring surveillance every 1-2 years 1, 5

Management Approach

Localized Disease (Limited Lesions, Single Body Region)

For limited, discrete papules and plaques, start with high-potency topical corticosteroids as first-line therapy. 1, 6

• Clobetasol propionate 0.05% cream or ointment applied once daily to lesional skin, 10-20 grams daily 1, 6
• Halobetasol propionate is an alternative ultra-potent option 7, 6
• Intralesional triamcinolone acetonide may be more effective than topical preparations for discrete lesions 7, 1, 6
• Evidence for topical therapy is limited - one study showed complete resolution in only 25% of patients (5 of 20) with partial resolution in the remainder 7

Critical caveat: Ultra-high potency topical corticosteroids require strict usage time limits due to risk of skin atrophy and systemic absorption; avoid prolonged use 6

Widespread Disease (Multiple Regions, Extensive Lesions)

For widespread cutaneous involvement or cosmetically significant lesions, oral corticosteroids are the first-line systemic treatment. 1, 6

• Prednisone 0.5-0.75 mg/kg/day is the recommended starting dose 6
• Response occurs in approximately two-thirds of patients, though effects are often limited to treatment duration 1
• Begin tapering 15 days after disease control is achieved 6
• Topical or intralesional corticosteroids are impractical for widespread lesions 7, 6

Major pitfall: Recurrences during corticosteroid tapering are common and frequently require additional immunosuppressive therapy 1, 6

Steroid-Refractory or Chronic Disease

For patients with ongoing disease despite glucocorticoids or unacceptable steroid side effects:

Step 1: Add methotrexate as a steroid-sparing agent for symptomatic patients with ongoing disease or glucocorticoid toxicity 1

Step 2: Add infliximab for patients who fail glucocorticoids and/or other immunosuppressives with continued cosmetically important active skin disease 7, 1, 6

• Two randomized controlled trials demonstrated statistically significant improvement in cutaneous lesions with infliximab compared to glucocorticoid alone 7
• This is particularly important for lupus pernio, which represents chronic disease requiring steroid-sparing alternatives 7, 6
• Adalimumab has shown efficacy in prospective studies as an alternative anti-TNF agent 1

Important negative evidence: Golimumab and thalidomide failed to show benefit in randomized trials for cutaneous sarcoidosis 7

Special Considerations

• Most mild cutaneous lesions are minimally symptomatic and do not require treatment 2
• Chronic lesions, particularly lupus pernio, are disfiguring and have strong psychological and social impact, warranting aggressive treatment 2
• Long-term oral corticosteroid use carries substantial side effects, making early consideration of steroid-sparing agents essential 7, 6