Maillard H, Szczesniak S, Martin L, Garot D, Machet M C, Machet L, Lorette G, Vaillant L
Service de Dermatologie, CHU, Tours.
Ann Dermatol Venereol. 1999 Feb;126(2):125-9.
Cutaneous periarteritis nodosa (PAN) is distinguished from systemic PAN by the lack of visceral involvement. The aim of this study was to describe the clinical presentation, laboratory findings, clinical course, and treatment in cutaneous PAN.
We retrospectively reviewed the files of patients hospitalized for vasculitis in our Dermatology unit where approximately 20 cases of vasculitis are seen each year. Inclusion criteria were skin signs suggestive of PAN and a histological image of leukocytoclastic vasculitis of an arteriole.
Nine cases of cutaneous PAN were treated in our unit between 1976 and 1997. Follow-up ranged from 32 months to 22 years. No cases of systemic PAN had been diagnosed during this period. These 9 cases of cutaneous PAN all had the same clinical presentation: nodules on the lower limbs in all cases associated with nodules on the upper limbs in half of the cases. Neuropathy was found in 3 of the 9 cases. No systemic involvement was observed. The most frequently used treatment protocol was general corticosteroid therapy (0.5 mg/kg/d prednisone or prednisolone). Immunosuppressive drugs, colchicine, dapsone, non-steroidal anti-inflammatory drugs and intravenous immunoglobulins were also used with efficacy.
Cutaneous PAN is a particular form of vasculitis associating skin signs with locoregional neuromuscular involvement. The differential diagnosis with other types of vasculitis is sometimes a difficult task. The clinical course is the fundamental diagnostic clue in cutaneous PAN. A benign course and the absence of visceral involvement allow initiating a symptomatic treatment such as colchicine. The development of neuromuscular signs may warrant the use of general corticosteroid therapy.
皮肤型结节性多动脉炎(PAN)与系统性PAN的区别在于无内脏受累。本研究的目的是描述皮肤型PAN的临床表现、实验室检查结果、临床病程及治疗情况。
我们回顾性分析了在我院皮肤科住院的血管炎患者病历,我院每年约有20例血管炎患者。纳入标准为提示PAN的皮肤体征以及小动脉白细胞破碎性血管炎的组织学图像。
1976年至1997年间,我院共治疗了9例皮肤型PAN患者。随访时间为32个月至22年。在此期间,未诊断出系统性PAN病例。这9例皮肤型PAN患者临床表现均相同:所有患者下肢均有结节,半数患者上肢也有结节。9例中有3例出现神经病变。未观察到系统性受累情况。最常用的治疗方案是全身使用糖皮质激素(泼尼松或泼尼松龙0.5mg/kg/d)。免疫抑制剂、秋水仙碱、氨苯砜、非甾体抗炎药及静脉注射免疫球蛋白也有疗效。
皮肤型PAN是一种特殊类型的血管炎,伴有皮肤体征及局部神经肌肉受累。与其他类型血管炎的鉴别诊断有时较为困难。临床病程是皮肤型PAN的基本诊断线索。病程良性且无内脏受累可启动对症治疗,如使用秋水仙碱。神经肌肉体征的出现可能需要使用全身糖皮质激素治疗。