Blanco R, Martínez-Taboada V M, Rodríguez-Valverde V, García-Fuentes M
Service of Rheumatology, Hospital Universitario Marqués de Valdecilla, Facultad de Medicina, Universidad de Cantabria, Santander, Spain.
Medicine (Baltimore). 1998 Nov;77(6):403-18. doi: 10.1097/00005792-199811000-00007.
Cutaneous vasculitis (CV), a condition characterized by palpable purpura and nonspecific histopathologic findings, presents a diagnostic and therapeutic challenge because it may be a primary disorder or it may be a cutaneous manifestation of another entity, such as systemic necrotizing vasculitis, connective tissue disease, systemic bacterial infection, or malignancy. We studied 303 unselected patients (172 adults and 131 children) with CV to assess the disease associations and etiologic factors, to identify the frequency of primary and secondary CV in different age-groups, and to characterize features that help to distinguish between primary and secondary CV. Of the 131 children, 130 had primary CV: Henoch-Schönlein purpura (HSP) in 116 and hypersensitivity vasculitis (HV) in 14. In contrast, of the 172 adults, only 120 had primary CV: HSP in 39, HV in 70, and essential mixed cryoglobulinemia in 11. CV was a manifestation of systemic necrotizing vasculitis in 23 adults (polyarteritis nodosa in 17, Wegener granulomatosis in 4, and Churg-Strauss syndrome in 2). CV was secondary to other processes in 29 adults: in 20 patients CV was associated with connective tissue disease or another autoimmune or rheumatic disease, in 5 patients CV was a manifestation of severe bacterial infection, especially bacterial endocarditis (4 cases), and in the other 4 patients CV was the presenting symptom of an underlying malignancy. The patients for whom CV was a manifestation of systemic necrotizing vasculitis or secondary to a connective tissue disease, severe bacterial infection, or malignancy had clinical and laboratory data suggestive of the associated disorder. The clinical picture and outcome of primary CV in both children and adults were benign. By contrast, the prognosis of patients with CV in the context of systemic necrotizing vasculitis or secondary to other entities depended on the primary process. Given the different disease association in children and adults, we propose a simple diagnostic workup in children with CV. By contrast the diagnostic approach in adults with CV should be more cautious and the workup more extensive. The early differentiation between primary CV, secondary CV, and CV presenting as a symptom of systemic necrotizing vasculitis, especially in adults, is of paramount importance for an adequate diagnosis and appropriate treatment.
皮肤血管炎（CV）以可触及的紫癜和非特异性组织病理学表现为特征，因其可能是原发性疾病，也可能是其他疾病（如系统性坏死性血管炎、结缔组织病、全身性细菌感染或恶性肿瘤）的皮肤表现，故而在诊断和治疗方面颇具挑战。我们对303例未经挑选的皮肤血管炎患者（172例成人和131例儿童）进行了研究，以评估疾病关联和病因因素，确定不同年龄组中原发性和继发性皮肤血管炎的发生率，并明确有助于区分原发性和继发性皮肤血管炎的特征。131例儿童中，130例患有原发性皮肤血管炎：116例为过敏性紫癜（HSP），14例为过敏性血管炎（HV）。相比之下，172例成人中，仅有120例患有原发性皮肤血管炎：39例为过敏性紫癜，70例为过敏性血管炎，11例为原发性混合性冷球蛋白血症。23例成人的皮肤血管炎是系统性坏死性血管炎的表现（17例为结节性多动脉炎，4例为韦格纳肉芽肿，2例为变应性肉芽肿性血管炎）。29例成人的皮肤血管炎继发于其他疾病：20例患者的皮肤血管炎与结缔组织病或其他自身免疫性或风湿性疾病相关，5例患者的皮肤血管炎是严重细菌感染的表现，尤其是细菌性心内膜炎（4例），另外4例患者的皮肤血管炎是潜在恶性肿瘤的首发症状。皮肤血管炎是系统性坏死性血管炎的表现或继发于结缔组织病、严重细菌感染或恶性肿瘤的患者，其临床和实验室数据提示存在相关疾病。儿童和成人原发性皮肤血管炎的临床表现和预后均良好。相比之下，系统性坏死性血管炎背景下或继发于其他疾病的皮肤血管炎患者的预后取决于原发疾病。鉴于儿童和成人的疾病关联不同，我们提出了针对儿童皮肤血管炎的简单诊断检查方法。相比之下，成人皮肤血管炎的诊断方法应更为谨慎，检查也应更为全面。尤其是在成人中，早期区分原发性皮肤血管炎、继发性皮肤血管炎以及作为系统性坏死性血管炎症状出现的皮肤血管炎，对于做出充分诊断和进行恰当治疗至关重要。
