1.4.1 Sarcoidosis

Grading & Level of Importance: B




Boeck disease, Besnier-Boeck-Schaumann disease, benign lymphogranulomatosis.


Rare disease. Skin involvement in 20-35% of cases; in 11% skin as first manifestation. The estimated prevalence in Europe is around 10/100,000 inhabitants, and is significantly higher in northern countries. 


Systemic granulomatous disease with skin involvement.

Aetiology & Pathogenesis

Unclear, found worldwide, female > male. It occurs most commonly in young and middle-aged individuals of both genders and all races. Afro-Caribbeans and African-Americans more commonly involved.

Signs & Symptoms

Many possible skin findings: brown-violaceous dermal papules, plaques, nodules. Often associated with erythema nodosum. Systemic disease can involve lymph nodes (90%, mostly intrathoracic), lungs (90%), eyes (30%), kidneys, bones, neural system, liver and GI tract.


All skin areas can be involved. 


Special forms:

  • Löfgren's syndrome (acute onset of fever, bilateral hilar lymphadenopathy and erythema nodosum)
  • Lupus pernio (centrofacial skin involvement)
  • Scar and tattoo sarcoidosis

Laboratory & other workups

  • Angiotensin converting enzyme (ACE; indicating cumulative involvement) and interleukin 2 receptor (IL2R; indicating disease activity) in serum sometimes elevated
  • Hypercalcemia
  • Lymphopenia
  • ESR elevated
  • Impaired delayed (type IV) hypersensitivity
  • Tuberculin skin test (negative)
  • Chest x-ray (may show bilateral hilar lymphadenopathy or interstitial changes)


Epithelioid cell granulomas without caseation, few CD4+ T lymphocytes (naked granulomas).


Chronic, spontaneous remission (25%).


Restrictive lung disease, immune suppression (lymphopenia), osteoporosis (in corticosteroid-treated patients; calcium supplementation contraindicated). Cutaneous calcification (calcinosis cutis). 


Clinical features, histology, laboratory findings.

Differential diagnosis

Tuberculosis, leprosy, granuloma annulare, necrobiosis lipoidica, lymphoma, leishmaniasis, amyloidosis, rosacea (lupus pernio).

Prevention & Therapy

Prevention of occupational and individual chronic small particle inhalation:

  • Skin involvement: Corticosteroids (topical, intralesional, systemic; first line), methotrexate (second line), chloroquine/hydroxychloroquine, PUVA
  • Systemic involvement: Corticosteroids (systemic; first line), methotrexate (second line), azathioprine, (leflunomide, mycophenolate mofetil, cyclophosphamide, TNF antagonists. Biologics may induce sarcoidosis.
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