Kawakami Tamihiro, Kawanabe Takeshi, Saito Chihiro, Kannari Maya, Mizoguchi Masako, Nagafuchi Hiroko, Okazaki Takahiro, Ozaki Shoichi, Kimura Kenjiro, Soma Yoshinao
Department of Dermatology, St Marianna University School of Medicine, Kanagawa, Japan.
J Am Acad Dermatol. 2007 Nov;57(5):840-8. doi: 10.1016/j.jaad.2007.05.012.
Microscopic polyangiitis (MPA) is a systemic antineutrophil cytoplasmic autoantibody-associated vasculitis associated with necrotizing and crescentic glomerulonephritis and pulmonary capillaritis. MPA generally has a rapidly progressive clinical course, but there have been recent reports of slowly progressive cases.
To evaluate the typical cutaneous findings of MPA, we recorded the clinical and histopathologic features of the cutaneous manifestations.
Eight patients with MPA, who had presented with cutaneous manifestations between 2001 and 2005 in our department, were retrospectively reviewed. They had necrotizing vasculitis in their cutaneous lesions as confirmed by skin biopsy specimens. Patients with other known connective tissue diseases were not included in the study.
All 8 patients with MPA presented cutaneously with erythematous macules on their extremities. Livedo reticularis (5/8, 68%) was also observed. Six of the 8 patients with MPA were given the diagnosis within 3 months of their initial manifestation. In skin biopsy specimens, necrotizing vasculitis was noted in the reticular dermis to the subcutaneous fat. In contrast, the other two patients with MPA were given the diagnosis about 10 years after their initial manifestation. Histopathologic findings demonstrated necrotizing vasculitis with moderate neutrophilic infiltrations in the papillary to middle dermis in the latter two patients. Serum myeloperoxidase-antineutrophil cytoplasmic autoantibody levels were only moderlately elevated in the latter two patients and they were given the diagnosis of slowly progressive MPA. Histopathologically, palisading granulomas were present on the elbow of one of them.
The study was based on histopathological analysis in a limited number of patients due to the rareness of the investigated disease.
There appears to be a correlation between a slowly progressive clinical course of MPA and the depth of dermal involvement and the severity of neutrophilic infiltration in biopsy specimens. Based on these results, we believe that these characteristic patterns may help clinicians establish an earlier diagnosis of possible MPA with positive antineutrophil cytoplasmic autoantibody titers.
显微镜下多血管炎（MPA）是一种与抗中性粒细胞胞浆自身抗体相关的系统性血管炎，与坏死性新月体性肾小球肾炎和肺毛细血管炎相关。MPA通常具有快速进展的临床病程，但最近有缓慢进展病例的报道。
为评估MPA的典型皮肤表现，我们记录了皮肤表现的临床和组织病理学特征。
回顾性分析了2001年至2005年间在我科出现皮肤表现的8例MPA患者。皮肤活检标本证实其皮肤病变存在坏死性血管炎。其他已知结缔组织病患者未纳入本研究。
所有8例MPA患者均在四肢出现皮肤红斑。还观察到网状青斑（5/8，68%）。8例MPA患者中有6例在首次出现症状后3个月内确诊。皮肤活检标本中，在网状真皮至皮下脂肪可见坏死性血管炎。相比之下，另外2例MPA患者在首次出现症状约10年后确诊。组织病理学检查发现，后2例患者在乳头层至真皮中层有坏死性血管炎伴中度中性粒细胞浸润。后2例患者血清髓过氧化物酶-抗中性粒细胞胞浆自身抗体水平仅中度升高，被诊断为缓慢进展型MPA。组织病理学上，其中1例患者肘部出现栅栏状肉芽肿。
由于所研究疾病罕见，本研究基于对有限数量患者的组织病理学分析。
MPA缓慢进展的临床病程与活检标本中真皮受累深度及中性粒细胞浸润严重程度之间似乎存在相关性。基于这些结果，我们认为这些特征性模式可能有助于临床医生对自身抗体滴度阳性的疑似MPA患者做出更早诊断。
