Prosty Connor, Guirguis Justina, Chergui May, Afif Waqqas, Netchiporouk Elena
Faculty of Medicine, McGill University, Montreal, QC, Canada.
Division of Dermatology, McGill University Health Centre, Montreal General Hospital, Montreal, QC, Canada.
SAGE Open Med Case Rep. 2022 Apr 14;10:2050313X221091600. doi: 10.1177/2050313X221091600. eCollection 2022.
Epidermolysis bullosa acquisita is a rare autoimmune disease involving cutaneous blistering and scarring associated with collagen VII autoantibodies. Similarly, collagen VII autoantibodies are present in the majority of Crohn's disease patients and approximately a quarter of epidermolysis bullosa acquisita patients have coexisting Crohn's disease. Treatment options for epidermolysis bullosa acquisita are limited and are largely ineffective. Here, we describe a 36-year-old female with a history of Crohn's disease presenting with a 7-year history of severe blistering and scarring of acral surfaces. Diagnostic workup revealed subepidermal cleavage on skin biopsy and elevated serum collagen VII autoantibodies, indicative of epidermolysis bullosa acquisita. She was given ustekinumab for her coexisting Crohn's disease and, afterwards, her epidermolysis bullosa acquisita resolved as evidenced by a lack of new blisters or scarring. Further studies are required to evaluate the effects of ustekinumab on epidermolysis bullosa acquisita.
获得性大疱性表皮松解症是一种罕见的自身免疫性疾病,表现为皮肤水疱和瘢痕形成,与Ⅶ型胶原自身抗体相关。同样,大多数克罗恩病患者体内也存在Ⅶ型胶原自身抗体,约四分之一的获得性大疱性表皮松解症患者同时患有克罗恩病。获得性大疱性表皮松解症的治疗选择有限且大多无效。在此,我们描述一名36岁有克罗恩病病史的女性,其肢端严重水疱和瘢痕形成已有7年病史。诊断性检查显示皮肤活检有表皮下裂隙,血清Ⅶ型胶原自身抗体升高,提示为获得性大疱性表皮松解症。她因同时患有克罗恩病而接受了乌司奴单抗治疗,之后,其获得性大疱性表皮松解症得到缓解,表现为无新的水疱或瘢痕形成。需要进一步研究来评估乌司奴单抗对获得性大疱性表皮松解症的影响。