Demirkesen Cuyan
Department of Pathology, Acıbadem University, Istanbul, Turkey.
Curr Opin Rheumatol. 2017 Jan;29(1):39-44. doi: 10.1097/BOR.0000000000000346.
The present review will focus on recent publications in cutaneous vasculitides.
Some histopathological and clinical features, such as papillary dermal edema, perivascular C3 deposition, clinically evident edema, and lesions above the waist, may point out renal or gastrointestinal involvement in Henoch-Schönlein purpura (HSP). HSP associated with familial Mediterranean fever differs from typical isolated HSP by showing no deposits of IgA, much younger age, and location of the lesions on the face or the trunk. Single-organ cutaneous small vessel vasculitis is a more restricted entity than hypersensitivity vasculitis and HSP. Because cutaneous polyarteritis nodosa and macular lymphocytic arteritis share some clinicopathologic features, the question is raised whether they are not two different entities. Several histopathological features defining IgG4-related disease are found in granuloma faciale and erythema elevatum diutinum, two localized chronic cutaneous vasculitis; however, in a recent series no diagnostic criteria for IgG4-related disease was detected in them.
When a patient presents with skin lesions, in which necrotizing or leukocytoclastic vascuitis is confirmed histologically, irrespective of the size of the affected vessel, the possibility of systemic vasculitis, an infection, medication, or a systemic disease such as systemic lupus erythematosus must be searched before reaching definitive diagnosis.
本综述将聚焦于皮肤血管炎的近期出版物。
一些组织病理学和临床特征,如乳头真皮水肿、血管周围C3沉积、临床上明显的水肿以及腰部以上的病变,可能提示过敏性紫癜(HSP)存在肾脏或胃肠道受累。与家族性地中海热相关的HSP与典型的孤立性HSP不同,表现为无IgA沉积、发病年龄更小且病变位于面部或躯干。单器官皮肤小血管炎是一种比超敏性血管炎和HSP更局限的疾病。由于皮肤结节性多动脉炎和斑状淋巴细胞性动脉炎具有一些临床病理特征,因此有人提出它们是否并非两种不同的疾病。在面部肉芽肿和持久性隆起性红斑这两种局限性慢性皮肤血管炎中发现了一些定义IgG4相关疾病的组织病理学特征;然而,在最近的一系列研究中,未在它们中检测到IgG4相关疾病的诊断标准。
当患者出现皮肤病变且组织学证实为坏死性或白细胞破碎性血管炎时,无论受累血管的大小如何,在做出明确诊断之前,必须排查系统性血管炎、感染、药物或系统性疾病(如系统性红斑狼疮)的可能性。