皮肤结节性多动脉炎（cPAN）与黄斑淋巴细胞性动脉炎（MLA）的关系：35 例 cPAN 病例的盲法组织学评估。
Relationship between cutaneous polyarteritis nodosa (cPAN) and macular lymphocytic arteritis (MLA): Blinded histologic assessment of 35 cPAN cases.
机构信息
University Paris Diderot, Sorbonne Paris Cité, Assistance Publique des Hôpitaux de Paris (AP-HP), Dermatology, Pathology, and Internal Medicine Department, Saint Louis Hospital, Paris, France.
University Paris Diderot, Sorbonne Paris Cité, Assistance Publique des Hôpitaux de Paris (AP-HP), Dermatology, Pathology, and Internal Medicine Department, Saint Louis Hospital, Paris, France.
出版信息
J Am Acad Dermatol. 2015 Dec;73(6):1013-20. doi: 10.1016/j.jaad.2015.09.010. Epub 2015 Oct 20.
BACKGROUND
Cutaneous polyarteritis nodosa (cPAN) is a skin medium vessel neutrophilic arteritis with livedo, nodules, and ulcerations. Macular lymphocytic arteritis (MLA) is a small arteritis with erythematous or pigmented macules and typical histologic features (a lymphocytic infiltrate, concentric fibrin ring, no disruption of the internal elastic lamina).
OBJECTIVE
We sought to assess the frequency of clinical and histologic features of MLA in patients with cPAN.
METHODS
This was a monocentric retrospective analysis of patients given the diagnosis of cPAN with blinded assessment of skin biopsy specimens.
RESULTS
All 35 patients included had an infiltrated livedo, nodules, or both. Ulceration was rare. Erythematous or pigmented lesions were present in 54% of patients. Predominantly lymphocytic arteritis, a paucity of neutrophils, concentric fibrin ring, and absence of internal lamina elastic disruption were present in 60%, 20%, 18%, and 23% of patients, respectively. Median follow-up was 11 years. None of the patients had systemic involvement, and 57% had a complete remission. The incidence of complete remission was not different between patients having a predominant lymphocyte infiltrate or few neutrophils.
LIMITATIONS
This was a retrospective, monocentric study without a control group of patients with MLA.
CONCLUSIONS
Our data do not favor the classification of cPAN and MLA as distinct entities.
背景
皮肤多发性动脉炎（cPAN）是一种皮肤中型血管中性粒细胞性动脉炎，伴有皮肤淤斑、结节和溃疡。斑状淋巴细胞性动脉炎（MLA）是一种小动脉炎，表现为红斑或色素斑，具有典型的组织学特征（淋巴细胞浸润、同心纤维蛋白环、内弹性膜无破坏）。
目的
我们旨在评估 MLA 的临床和组织学特征在 cPAN 患者中的出现频率。
方法
这是一项对 cPAN 患者进行的单中心回顾性分析，对皮肤活检标本进行了盲法评估。
结果
所有纳入的 35 例患者均存在浸润性淤斑、结节或两者兼有。溃疡罕见。54%的患者存在红斑或色素斑。主要为淋巴细胞性动脉炎、中性粒细胞缺乏、同心纤维蛋白环和内弹性膜无破坏分别见于 60%、20%、18%和 23%的患者。中位随访时间为 11 年。无患者有系统性受累，57%的患者达到完全缓解。具有主要淋巴细胞浸润或少量中性粒细胞的患者之间，完全缓解的发生率没有差异。
局限性
这是一项回顾性、单中心研究，没有 MLA 患者的对照组。
结论
我们的数据不支持将 cPAN 和 MLA 分类为不同的实体。
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