首次命名淀粉样变型类天疱疮并附一例报道

Amyloidosis pemphigoid:a case report

本文首次提出淀粉样变型类天疱疮的概念,并报道一例。患者中年女性,躯干、四肢苔藓样皮疹伴瘙痒17年,无水疱。组织病理示:真皮乳头大量嗜酸性无定形物质沉积,结晶紫染色:阳性;DIF:真皮乳头及基底膜IgG、IgM颗粒状沉积,C3、IgA阴性;IIF:类天疱疮循环抗体IgG 1∶320阳性。人皮肤盐裂IIF:循环抗体IgG表皮侧阳性;抗BP180抗体0 U/mL,抗BP230抗体169.2 U/mL。

We first proposed the conception of cutaneous amyloidosis pemphigoid and reported a case in this paper.A middle-aged female presented with hypertrophic patches,no blisters,with itching for 17 years on trunk and limbs.Biopsy showed a large number of eosinophilic amorphous substance deposition in dermal papilla and the crystal violet staining was positive.DIF showed the granular deposition of IgG and IgM was found in the basement membrane and dermal papilla,and negitive for C3 and IgA.IIF positivity of antibody IgG was noted with both routine and salt split method.Enzyme-linked immunosorbent assays showed anti BP180 antibody was 0 U/mL and anti BP230 antibody was 169.2 U/mL.