Tay Daniel Zunsheng, Tan Ki-Wei, Tay Yong-Kwang
Department of Medicine, Yong Loo Lin School of Medicine, National University of Singapore, Singapore 119228.
Department of Dermatology, Changi General Hospital Singapore, Singapore 529889.
J Family Med Prim Care. 2014 Oct-Dec;3(4):374-8. doi: 10.4103/2249-4863.148113.
Pyoderma ga ngrenosum (PG) is a rare, inflammatory, destructive neutrophilic dermatosis, which mimics other ulcerative conditions.
In a retrospective study based on patients diagnosed with PG over a 3-year period (2010-2013), we evaluated demographics, anatomical sites, number of lesions, subtypes, histopathology, associated conditions, treatment regimens, healing time, and recurrence.
Of our five patients, there were three males and two females, age ranging between 19 and 58 years (mean age 38 years). Four had single lesions localized to the lower limbs while one had multiple lesions (more than five) over bilateral hands and legs. Ulcerative subtype was observed in all the patients. One exhibited pathergy. Skin biopsies were done in four patients, revealing dense neutrophilic infiltrates in three cases and leukocytoclastic vasculitis in one. Associated systemic diseases were observed in all patients, four having inflammatory bowel disease and one having both systemic lupus erythematosus and anti-phospholipid syndrome. The patients were all treated with systemic corticosteroids either alone or in combination with immunosuppressants (e.g., azathioprine, mycophenolate mofetil, tacrolimus), and wound dressing. Split-thickness skin graft was done in one patient. Complete healing was achieved in all patients, ranging from one to 3 months after diagnosis. No recurrence was reported.
Systemic corticosteroids, either alone or in combination with steroid-sparing agents are the mainstay of treatment. Should family physicians encounter a rapidly progressing ulcer that has poor response to usual wound management, timely referral to dermatology should be made.
坏疽性脓皮病(PG)是一种罕见的、炎症性、破坏性嗜中性皮肤病,可模仿其他溃疡性疾病。
在一项基于2010年至2013年3年间诊断为PG的患者的回顾性研究中,我们评估了人口统计学、解剖部位、皮损数量、亚型、组织病理学、相关疾病、治疗方案、愈合时间和复发情况。
我们的5例患者中,男性3例,女性2例,年龄在19至58岁之间(平均年龄38岁)。4例患者有单个皮损,局限于下肢,而1例患者双手和双腿有多个皮损(超过5个)。所有患者均观察到溃疡性亚型。1例表现为同形反应。4例患者进行了皮肤活检,3例显示密集嗜中性粒细胞浸润,1例显示白细胞破碎性血管炎。所有患者均观察到相关的全身性疾病,4例患有炎症性肠病,1例患有系统性红斑狼疮和抗磷脂综合征。所有患者均接受了全身皮质类固醇单独或联合免疫抑制剂(如硫唑嘌呤、霉酚酸酯、他克莫司)治疗及伤口敷料处理。1例患者进行了中厚皮片移植。所有患者均实现完全愈合,诊断后1至3个月不等。未报告复发情况。
全身皮质类固醇单独或联合类固醇节约剂是主要治疗方法。如果家庭医生遇到对常规伤口处理反应不佳的快速进展性溃疡,应及时转诊至皮肤科。