Sugino Hitomi, Kawahara Hikaru, Yamamoto Kayo, Okada Etsuko, Sawada Yu
Department of Dermatology University of Occupational and Environmental Health Kitakyushu Japan.
Skin Health Dis. 2024 Feb 6;4(2):e347. doi: 10.1002/ski2.347. eCollection 2024 Apr.
Pyoderma gangrenosum is a rare inflammatory skin disease classified within the group of neutrophilic dermatoses, and clinically characterised by painful, rapidly evolving cutaneous ulcers with undermined, irregular, erythematous-violaceous edges. Underlying diseases include rheumatoid arthritis, inflammatory bowel disease, haematopoietic malignancy, and aortitis syndrome. However, there was a limited number of cases of concomitant pyoderma gangrenosum and IgA vasculitis. Herein, we report a case presenting persistent large skin wounds as a diagnosis of pyoderma gangrenosum in the setting of IgA cutaneous vasculitis, which was successfully treated by a TNF-α inhibitor. A 67-year-old obese female presented palpable purpura on her lower extremities. A skin biopsy taken from the purpuric eruption showed leukocytoclastic vasculitis with IgA and C3 depositions in the vessel walls of the upper dermis, leading to the diagnosis of IgA vasculitis. Small skin ulcers rapidly expanded in several days, eventually developing perforating skin ulcers with irregular erythematous and violaceous edges on both lower extremities following the tapered oral prednisolone at a dose of 25 mg per day. Based on the clinical manifestation and histological analysis, we diagnosed her skin wound as pyoderma gangrenosum. After the adalimumab administration, the spreading ulceration was dampened, leading to the acceleration of wound epithelialisation.
坏疽性脓皮病是一种罕见的炎症性皮肤病,归类于嗜中性皮肤病组,临床特征为疼痛、迅速发展的皮肤溃疡,边缘呈潜行性、不规则、红斑性-紫罗兰色。潜在疾病包括类风湿性关节炎、炎症性肠病、血液系统恶性肿瘤和主动脉炎综合征。然而,坏疽性脓皮病与IgA血管炎同时存在的病例数量有限。在此,我们报告一例在IgA皮肤血管炎背景下以持续性大皮肤伤口为表现诊断为坏疽性脓皮病的病例,该病例通过肿瘤坏死因子-α抑制剂成功治疗。一名67岁肥胖女性双下肢出现可触及的紫癜。从紫癜疹处取皮肤活检显示白细胞破碎性血管炎,在上真皮层血管壁有IgA和C3沉积,从而诊断为IgA血管炎。小皮肤溃疡在数天内迅速扩大,在每天25毫克逐渐减量的口服泼尼松龙治疗后,最终双下肢出现边缘不规则红斑和紫罗兰色的穿孔性皮肤溃疡。基于临床表现和组织学分析,我们将她的皮肤伤口诊断为坏疽性脓皮病。给予阿达木单抗后,溃疡扩散得到抑制,伤口上皮化加速。