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Boeck病
1.5.1 肉样瘤病
Grading & Level of Importance: B
同义词
定义
一种系统性肉芽肿性疾病，三分之一病例累及皮肤。
病因和发病机理
病因和发病机制尚不清楚。见于世界各地，女性发病率高于男性。
症状
皮疹为多形性：如结节、斑块、丘疹和浸润性乌青色斑片。常与结节性红斑相关。
系统受累（如肺、眼、肾、骨、肝和消化道）。
分类
特殊类型：
¤ Löfgren综合征
¤ Heerfordt综合征
¤ 冻疮样狼疮型
¤ 瘢痕样肉样瘤病
实验室检查
血管紧张素转化酶（ACE）有时增高，血钙升高，淋巴细胞减少；血沉加快，迟发性过敏减弱。需做皮肤结核菌素试验和胸部X-光片。
组织病理
上皮样细胞肉芽肿，不伴干酪坏死，可有极少的淋巴细胞侵润（裸结节）。
病程
慢性，25％病例可自行消退。
诊断
依据临床特点、组织病理和实验室检查诊断。
治疗
糖皮质激素（包括外用、皮损内注射和系统应用）、氯喹和PUVA治疗。
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?
- 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?
- True or false?
- 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?
- 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?
更多图片/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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