Aoussar A, Ismaïli N, Berbich L, Tazi Mezalek Z, Aït Ourhrouil M, Senouci K, Mansouri F, Hassam B
Service de Dermatologie, Hôpital Ibn Sina, Rabat, Maroc.
Ann Dermatol Venereol. 2007 Mar;134(3 Pt 1):264-7. doi: 10.1016/s0151-9638(07)91510-0.
Pyoderma gangrenosum is a form of neutrophilic dermatosis often associated with inflammatory disease or blood dyscrasias. We report a case of pyoderma gangrenosum revealing Takayasu's arteritis.
A 26 year-old woman was followed since 2002 for pyoderma gangrenosum occurring during pregnancy and regressing under general corticosteroids. In January 2004, immediately after childbirth, she presented an extensive ulcerated lesion on the right forearm. Histological examination of a biopsy sample from this solution pointed towards pyoderma gangrenosum. Somatic examination showed reduced peripheral pulse on the right side, asymmetric blood pressure between the two arms and vascular murmur in the carotid, femoral and renal arteries. Based on laboratory and x-ray examinations, Takayasu's arteritis was diagnosed and chronic viral hepatitis B detected. Resumption of treatment with prednisone (1 mg/kg/d) resulted in resolution of the wounds within 1 month.
Although pyoderma gangrenosum is a classic complication in Takayasu's arteritis in Japan, this combination is rare in Europe, North America and North Africa. This case was singular in terms of the occurrence of pyoderma gangrenosum with each pregnancy. However, a number of epidemiological and clinical features were consistent with those reported in the literature, namely presentation predominantly in female patients, young age and involvement of the limbs. In 60% of cases, pyoderma gangrenosum precedes Takayasu's arteritis. The recommended mode of treatment comprises oral corticosteroids as first-line therapy followed by cyclosporine as second-line treatment.
坏疽性脓皮病是一种中性粒细胞性皮肤病,常与炎症性疾病或血液系统疾病相关。我们报告一例坏疽性脓皮病并发高安动脉炎的病例。
一名26岁女性自2002年起因孕期出现坏疽性脓皮病并在全身使用糖皮质激素后病情缓解而接受随访。2004年1月,产后不久,她右侧前臂出现广泛的溃疡病变。对该病变活检样本的组织学检查显示为坏疽性脓皮病。体格检查发现右侧外周脉搏减弱,双臂血压不对称,颈动脉、股动脉和肾动脉有血管杂音。基于实验室检查和X线检查,诊断为高安动脉炎,并检测出慢性乙型病毒性肝炎。重新使用泼尼松(1mg/kg/d)治疗后,伤口在1个月内愈合。
尽管在日本坏疽性脓皮病是高安动脉炎的经典并发症,但在欧洲、北美和北非这种组合较为罕见。该病例中坏疽性脓皮病在每次孕期均出现,情况较为特殊。然而,一些流行病学和临床特征与文献报道一致,即主要见于女性患者、年轻且累及四肢。在60%的病例中,坏疽性脓皮病先于高安动脉炎出现。推荐的治疗方式包括口服糖皮质激素作为一线治疗,环孢素作为二线治疗。