Department of Pediatrics, University Children's Hospital of Bern, Inselspital, Bern, Switzerland.
Pediatric Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico and Department of Clinical Sciences and Community Health, Università degli Studi di Milano, Milan, Italy.
Clin Rev Allergy Immunol. 2017 Dec;53(3):439-451. doi: 10.1007/s12016-017-8626-3.
In childhood, cutaneous small-vessel vasculitides include Henoch-Schönlein syndrome, a systemic vasculitis, and Finkelstein-Seidlmayer syndrome, a skin-limited vasculitis. Both Henoch-Schönlein and Finkelstein-Seidlmayer syndromes are seen more frequently in white or Asian compared with black children and occur especially in winter and spring with a male-to-female ratio of approximately 2:1. In everyday clinical practice, both conditions are diagnosed on clinical grounds without histological confirmation. The characteristic cutaneous hallmarks of Henoch-Schönlein syndrome include a purpuric rash in all and a subcutaneous edema in approximately every second case, which are often preceded by non-specific red or pink macular elements that mimic a non-itching urticarial rash. Recent data point out that Henoch-Schönlein children often present further cutaneous findings such as Köbnerization, Rumpel-Leede capillary fragility phenomenon, and blistering eruptions. Children with Finkelstein-Seidlmayer syndrome are usually ≤24 months of age and not ill-appearing. They present with (a) large, round, red to purpuric plaques (often with a targetoid appearance) predominantly over the cheeks, ears, and extremities and (b) often tender non-pitting edema of the distal extremities, ears, and face (without pruritus). Both in Henoch-Schönlein syndrome and Finkelstein-Seidlmayer syndrome, there is often scrotal involvement. The cutaneous findings remit without sequelae within 2 months in Henoch-Schönlein and 3 weeks in Finkelstein-Seidlmayer syndrome.
在儿童时期，皮肤小血管血管炎包括过敏性紫癜，一种全身性血管炎，和 Finkelstein-Seidlmayer 综合征，一种皮肤局限性血管炎。与黑人儿童相比，白人或亚洲儿童更常发生过敏性紫癜和 Finkelstein-Seidlmayer 综合征，且尤其多见于冬春季节，男女比例约为 2:1。在日常临床实践中，这两种疾病均基于临床诊断，无需组织学确认。过敏性紫癜的典型皮肤特征包括所有病例的紫癜性皮疹和大约每二例病例的皮下水肿，这些通常先于非特异性红色或粉红色斑疹样元素，类似于无瘙痒的荨麻疹样皮疹。最近的数据表明，过敏性紫癜患儿常出现其他皮肤表现，如 Koebner 现象、Rumpel-Leede 毛细血管脆性现象和水疱性皮疹。Finkelstein-Seidlmayer 综合征患儿通常≤24 个月，且外观无病态。他们表现为 (a) 大而圆的红色至紫癜性斑块（常呈靶形外观），主要位于脸颊、耳朵和四肢；(b) 四肢、耳朵和面部常出现触痛性无凹陷性水肿（无瘙痒）。过敏性紫癜和 Finkelstein-Seidlmayer 综合征中，常伴有阴囊受累。过敏性紫癜的皮肤表现在 2 个月内消退，无后遗症，Finkelstein-Seidlmayer 综合征则在 3 周内消退。
