Hsiao Jennifer L, Antaya Richard J, Berger Timothy, Maurer Toby, Shinkai Kanade, Leslie Kieron S
Department of Dermatology, University of California at San Francisco, CA, USA.
Arch Dermatol. 2010 Nov;146(11):1265-70. doi: 10.1001/archdermatol.2010.328.
Hidradenitis suppurativa (HS) and pyoderma gangrenosum (PG) are both rare inflammatory skin conditions that are associated with systemic inflammatory diseases. We performed a retrospective medical chart review of patients with an overlap of HS and PG.
We identified 11 cases of PG lesions presenting in patients with HS. Ten of the patients were women, and 9 were obese. All the patients developed HS lesions first, a median of 2.5 years (range, 0-15 years) preceding the appearance of PG lesions. All patients required multiple therapeutic agents because their diseases were often poorly responsive to standard therapies. Two patients received tumor necrosis factor inhibitors; 1 responded to treatment. One patient was treated with anakinra (interleukin-1 receptor antagonist) and had a 75% improvement of her lesions.
We have identified a group of patients who have an overlap of PG and HS. Pyoderma gangrenosum can appear at any point after the development of HS and often has a severe, refractory course. We propose that PG and HS may represent variant manifestations of cytokine dysregulation by the innate immune system with common etiology. New therapeutic agents are eagerly sought, and further investigation with regard to interleukin 1 blockade is warranted.
化脓性汗腺炎(HS)和坏疽性脓皮病(PG)均为罕见的炎症性皮肤病,与全身性炎症性疾病相关。我们对HS和PG重叠的患者进行了回顾性病历审查。
我们确定了11例HS患者出现PG皮损的病例。其中10例患者为女性,9例肥胖。所有患者均先出现HS皮损,在PG皮损出现前中位数为2.5年(范围0 - 15年)。所有患者均需要多种治疗药物,因为他们的疾病对标准治疗往往反应不佳。2例患者接受了肿瘤坏死因子抑制剂治疗;1例对治疗有反应。1例患者接受阿那白滞素(白细胞介素 - 1受体拮抗剂)治疗,皮损改善了75%。
我们发现了一组PG和HS重叠的患者。坏疽性脓皮病可在HS发生后的任何时间出现,且通常病程严重、难治。我们提出PG和HS可能代表先天性免疫系统细胞因子失调的不同表现形式,具有共同病因。迫切需要新的治疗药物,对白介素1阻断的进一步研究是有必要的。