Chen K-R, Toyohara A, Suzuki A, Miyakawa S
Department of Dermatology, Kawasaki City Hospital, Kawasaki, Japan.
Br J Dermatol. 2002 Nov;147(5):905-13. doi: 10.1046/j.1365-2133.2002.04933.x.
Cutaneous manifestations are the most frequent, and often the initial feature of extra-articular involvement in patients with rheumatoid vasculitis.
The purpose of the study was to evaluate the clinical and histological spectrum of cutaneous vasculitis and the associated systemic involvement in patients with rheumatoid vasculitis.
Among 525 patients with rheumatoid arthritis, 20 tissue specimens with histologically proven cutaneous necrotizing vasculitis from 11 patients were investigated by studying the types and levels of affected vessels and related clinical features.
Small-vessel vasculitis identified as dermal necrotizing venulitis was found in 10 patients, clinically characterized by palpable purpura, maculopapular erythema, erythema elevatum diutinum and haemorrhagic blisters. Arteritis histologically resembling cutaneous polyarteritis nodosa, clinically characterized by subcutaneous nodules, livedo reticularis, atrophie blanche and deep ulcers was identified in four patients all with systemic complications. Coexistence of venulitis and arteritis was identified in three patients. Different cutaneous vasculitic manifestations often coexisted and recurred in the same patient. Three patients with systemic complications of mononeuritis multiplex (two of three), interstitial pulmonary fibrosis (two of three) and abdominal microaneurysms (one of three) died within 1 year of onset of the cutaneous vasculitis. Immunofluorescence demonstrated vessel wall deposition of IgM and/or complement in six of the seven patients examined.
Features of cutaneous rheumatoid vasculitis overlapping both the characteristics of cutaneous necrotizing venulitis and cutaneous polyarteritis nodosa together with coexistence of these different type of vasculitis in the same or different lesional skin account for the associated diverse cutaneous vasculitic manifestations. Although dermal venulitis (leucocytoclastic vasculitis) was the most common presentation, the presence of leucocytoclastic vasculitis in rheumatoid patients did not necessarily indicate a favourable prognosis. Associations with mononeuritis multiplex and bowel involvement had a fatal prognosis, while patients with superficial dermal venulitis without other extra-articular involvement may follow a favourable prognosis.
皮肤表现是类风湿性血管炎患者关节外受累最常见且往往是最初的特征。
本研究旨在评估类风湿性血管炎患者皮肤血管炎的临床和组织学谱以及相关的全身受累情况。
在525例类风湿关节炎患者中，对11例经组织学证实为皮肤坏死性血管炎的20份组织标本进行研究，观察受累血管的类型和程度以及相关临床特征。
10例患者被诊断为以真皮坏死性静脉炎为特征的小血管炎，临床表现为可触及的紫癜、斑丘疹性红斑、持久性隆起性红斑和出血性水疱。4例患者被诊断为组织学上类似于皮肤结节性多动脉炎的动脉炎，临床表现为皮下结节、网状青斑、白色萎缩和深部溃疡，这4例患者均有全身并发症。3例患者同时存在静脉炎和动脉炎。不同的皮肤血管炎表现常共存且在同一患者中复发。3例有多发性单神经炎（3例中的2例）、间质性肺纤维化（3例中的2例）和腹部微动脉瘤（3例中的1例）等全身并发症的患者在皮肤血管炎发病后1年内死亡。免疫荧光显示，在检查的7例患者中有6例血管壁有IgM和/或补体沉积。
皮肤类风湿性血管炎的特征既重叠了皮肤坏死性静脉炎的特点，又有皮肤结节性多动脉炎的特点，且在同一或不同皮损部位存在这些不同类型的血管炎，这就导致了相关的多种皮肤血管炎表现。虽然真皮静脉炎（白细胞破碎性血管炎）是最常见的表现，但类风湿患者出现白细胞破碎性血管炎并不一定预示预后良好。与多发性单神经炎和肠道受累相关的预后不良，而无其他关节外受累的浅表真皮静脉炎患者可能预后良好。
