Department of Dermatology, St Marianna University School of Medicine, Kawasaki, Japan.
J Dermatol. 2010 Feb;37(2):113-24. doi: 10.1111/j.1346-8138.2009.00761.x.
Palpable purpura tends to indicate involvement of small vessel vasculitis in the upper dermis. Livedo racemosa, nodular lesion and skin ulceration are indicative of involvement of small to medium-sized vessel vasculitis in the lower dermis to subcutaneous fat. We set out to establish a new algorithm (KAWAKAMI algorithm) for primary cutaneous vasculitis based on the Chapel Hill Consensus Conference classification and our research results, and apply to the diagnosis. The first step is to measure serum antineutrophil cytoplasmic antibodies (ANCA) levels. If myeloperoxidase-ANCA is positive, Churg-Strauss syndrome or microscopic polyangiitis can be suspected, and if the patient is positive for proteinase 3-ANCA, Wegener's granulomatosis is most likely. Next, if cryoglobulin is positive, cryoglobulinemic vasculitis should be suspected. Third, if direct immunofluorescence of the skin biopsy specimen reveals immunoglobulin A deposition within the affected vessels, Henoch-Schönlein purpura is indicated. Finally, the presence of anti-phosphatidylserine-prothrombin complex antibodies and/or lupus anticoagulant and histopathological necrotizing vasculitis in the upper to middle dermis (leukocytoclastic vasculitis) indicates cutaneous leukocytoclastic angiitis, whereas if necrotizing vasculitis exists in the lower dermis and/or is associated with the subcutaneous fat, cutaneous polyarteritis nodosa is indicated. The KAWAKAMI algorithm may allow us to refine our earlier diagnostic strategies and allow for efficacious treatment of primary cutaneous vasculitis. In cutaneous polyarteritis nodosa, warfarin or clopidogrel therapies should be administrated, and in cases that have associated active inflammatory lesions, corticosteroids or mizoribine (mycophenolate mofetil) therapy should be added. We further propose prophylactic treatment of renal complications in patients with Henoch-Schönlein purpura.
可触及性紫癜往往表明小血管血管炎累及真皮上层。匐行性斑状血管病、结节性病变和皮肤溃疡提示小至中等血管血管炎累及真皮至皮下脂肪。我们旨在根据 Chapel Hill 共识会议分类和我们的研究结果，为原发性皮肤血管炎建立一种新的算法（KAWAKAMI 算法），并将其应用于诊断。第一步是测量血清抗中性粒细胞胞质抗体（ANCA）水平。如果髓过氧化物酶-ANCA 阳性，则可怀疑为 Churg-Strauss 综合征或显微镜下多血管炎，如果患者的蛋白酶 3-ANCA 阳性，则最有可能为 Wegener 肉芽肿。接下来，如果存在冷球蛋白，则应怀疑为冷球蛋白血症性血管炎。第三，如果皮肤活检标本的直接免疫荧光显示受影响的血管内有免疫球蛋白 A 沉积，则提示为过敏性紫癜。最后，如果存在抗磷脂酰丝氨酸-凝血酶原复合物抗体和/或狼疮抗凝剂以及中上层真皮的组织病理学坏死性血管炎（白细胞碎裂性血管炎），则表明为皮肤白细胞碎裂性血管炎，而如果坏死性血管炎存在于真皮下层和/或与皮下脂肪相关，则表明为皮肤多发性动脉炎。KAWAKAMI 算法可以使我们改进早期的诊断策略，并对原发性皮肤血管炎进行有效的治疗。在皮肤多发性动脉炎中，应给予华法林或氯吡格雷治疗，如果存在与活动性炎症病变相关的病例，则应添加皮质类固醇或吗替麦考酚酯（麦考酚酸酯）治疗。我们进一步提出预防过敏性紫癜患者肾脏并发症的治疗方案。
