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Morbo di Boeck.
1.5.1 Sarcoidosi
Grading & Level of Importance: B
Sinonimi
Definizione
Malattia sistemica granulomatosa con coinvolgimento cutaneo in 1/3 dei casi.
Eziologia; Patogenesi
Eziopatogenesi non chiara, malattia diffusa in tutto il mondo, colpisce più le femmine dei maschi.
Sintomi
Grande varietà di manifestazioni cliniche: noduli sottocutanei, placche, papule, macule violacee infiltrate. Spesso associata all'eritema nodoso.
Coinvolgimento sistemico (polmoni, occhi, reni, ossa, fegato,tratto gastrointestinale).
Classificazione
Varianti particolari:
- Sindrome di Löfgren
- Sindrome di Heerfordt
- Lupus pernio
- Scar sarcoidosis
Laboratorio
Enzima convertente l' Angiotensina (ACE) a volte elevato, ipercalcemia, linfopenia; Ves elevata, ridotta reazione da ipersensibilità ritardata. Test TB cutaneo. Rx torace.
Dermatopathology
Granulomi a cellule epitelioidi senza caseosi, pochi linfociti (granulomi nudi).
Course
Cronico, remissione spontanea (25%)
Diagnosi
Caratteristiche cliniche, istologia, reperti di laboratorio.
Diagnosi differenziale
Tubercolosi, lebbra, granuloma anulare, necrobiosi lipoidica, linfomi.
Terapia
Corticosteroidi (topici, sistemici, intralesionali) clorochina, PUVA.
Diagnosi differenziale
Tests
- 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?
- True or false?
- 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?
- 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?
- 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?
- This young lady has increasing dyspnea and disseminated brown papules. The biopsy is compatible with sarcoidosis, What other tests are indicated?
Further Images / DOIA
Review Articles
- 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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