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.
- 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
- 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.
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.
- This patient with AIDS Stage C3 has developed red-brown facial papules over the past few weeks. A skin biopsy shows dermal granulomas. What should you consider in your differential diagnosis?
- Statement 1 Sarcoidosis not only involves the skin but also the lungs, eyes, salivary glands, kidneys, bones, tonsils and nervous system.
- Sarcoidosis with cutaneous, pulmonary and renal involvement in a 25-year-old woman from the Antilles. The chest x-ray shows parenchymal diseases with micronodular lesions. What treatment do you suggest?
- 55-year-old woman presents with facial dermatosis for several months. Differential diagnostic possibilities include
- True or false?
- This young lady has increasing dyspnea and disseminated brown papules. The biopsy is compatible with sarcoidosis, What other tests are indicated?
- This young lady has numerous red-brown papules which have a yellow color under diascopy. The biopsy shows epithelioid granulomas without central necrosis. The likely diagnosis is?
- Young lady from Finland with fever (39 °C), arthralgias, and painful lesions on legs. The chest x-ray shows perihilar infiltrates with mediastinal lymphadenopathy. What is the diagnosis?
Further Images / DOIA
- M.C. Iannuzzi, B.A. Rybicki, A.S. Teirstein: Sarcoidosis (2017)
- A. Haimovic, M. Sanchez, M.A. Judson, S. Prystowsky: Sarcoidosis: A comprehensive review and update for the dermatologist: Part I. Cutaneous disease (2012)
- A. Haimovic, M. Sanchez, M.A. Judson, S. Prystowsky: Sarcoidosis: A comprehensive review and update for the dermatologist: Part II. Extracutaneous disease (2012)
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