Holl-Ulrich K, Rose C
Konsultations- und Referenzzentrum für Vaskulitis-Diagnostik, Pathologie - Hamburg, Labor Lademannbogen MVZ GmbH, Hamburg, Deutschland.
, Dermatopathologie Lübeck, Deutschland.
Pathologe. 2020 Jul;41(4):355-363. doi: 10.1007/s00292-020-00786-9.
The skin is one of the most frequently involved organs in primary systemic and secondary vasculitis; moreover, a vasculitis can occur as single organ vasculitis, limited to the skin. For most types of vasculitis, the lower extremities constitute common sites with clinical symptoms of palpable purpura, nodules, and ulcers. In histopathology of cutaneous vasculitis, it is of utmost importance to correctly identify the vessel types involved and to discriminate between vasculitic vessel damage, unspecific reactive vessel changes, vascular occlusive diseases, noninflammatory purpura, or perivascular infiltrates due to other inflammatory skin diseases. Small-vessel vasculitis is the most frequent type of cutaneous vasculitis; during florid phases, a dermal leukocytoclastic vasculitis is found regardless of etiology. Additional extravascular changes may give etiological clues, however, a correlation with the clinical picture, radiology, and serology is essential. The biopsy type and technique need to be adjusted to the suspected diagnosis and site of the vessels involved. Polyarteritis nodosa and nodular vasculitis can be diagnosed only in biopsies with sufficient subcutaneous tissue. Especially in cutaneous ulcers, a lateral rim of vital skin and subcutaneous tissue is indispensable for a correct diagnosis. Large-vessel vasculitis is not found in skin biopsies of the lower extremities.
皮肤是原发性系统性血管炎和继发性血管炎中最常受累的器官之一;此外,血管炎可表现为单一器官血管炎,仅限于皮肤。对于大多数类型的血管炎而言,下肢是出现可触及性紫癜、结节和溃疡等临床症状的常见部位。在皮肤血管炎的组织病理学检查中,正确识别受累血管类型以及区分血管炎性血管损伤、非特异性反应性血管改变、血管闭塞性疾病、非炎性紫癜或其他炎性皮肤病所致的血管周围浸润至关重要。小血管炎是最常见的皮肤血管炎类型;在急性期,无论病因如何,均可发现真皮白细胞破碎性血管炎。然而,额外的血管外改变可能提供病因线索,与临床表现、放射学和血清学的相关性至关重要。活检类型和技术需要根据疑似诊断和受累血管部位进行调整。结节性多动脉炎和结节性血管炎仅在含有足够皮下组织的活检标本中才能确诊。特别是在皮肤溃疡中,为了做出正确诊断,至关重要的是要有一圈存活的皮肤和皮下组织边缘。下肢皮肤活检中未发现大血管炎。