疱疹样皮炎的诊断治疗进展

TheDiagnosis and Treatment Progress of Dermatitis Herpetiformis

疱疹样皮炎(dermatitis herpetiformis,DH)是一种罕见的慢性复发性自身免疫性大疱病。其典型特征是瘙痒性多形皮损和真皮乳头IgA颗粒样沉积。由于其存在血清循环IgA抗体、表皮型转谷氨酰胺酶抗体及与小肠绒毛萎缩类似的典型组织学特征,被认为是乳糜泄的特异性皮肤表现。近年来多项DH研究表明该病在国人中并不罕见,且中国汉族DH存在独特的风险基因HLA-B*08:01和HLA-DRB1*03:01,该风险基因的检测可在临床协助诊断或排除DH。无谷胶饮食是治疗DH的基础,氨苯砜为治疗DH的特效药物,其他可使用的替代治疗包括柳氮磺胺吡啶、秋水仙碱、环孢素、吗替麦考酚酯、硫唑嘌呤、四环素联合烟酰胺等。

Dermatitis herpetiformis(DH)is a rare type of chronic recurrent autoimmune bullous disease.It is characterized by pruritus polymorphic lesions and granular deposition of IgA in the dermal papilla.Because of the presenee of serum circulating IgA antibody,epidermal transglutaminase antibody and typical histological features similar to villous atrophy of small intestine,it is considered to be a specific skin manifestation of celiac disease.A number of studies on DH in recent years have shown that this disease is not rare in Chinese people.There are unique risk genes HLA-B*08:01 and HLA-DRB1*03:01 in Chinese Han people for DH,which can assist in clinical diagnosis or exclude DH.The gluten-free diet is the basis for the treatment of DH.Dapsone is the effective drug for the treatment of DH.Other alternative therapies that can be used include salazopyridine,colchicine,cyclosporine,mortimecofenate,azathioprine,tetracycline combined with nicotinamide.