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妊娠类天疱疮

Gestational pemphigoid.

作者信息

Huilaja Laura, Mäkikallio Kaarin, Tasanen Kaisa

机构信息

Department of Dermatology, Medical Research Center, University of Oulu, Oulu University Hospital, Oulu, Finland.

出版信息

Orphanet J Rare Dis. 2014 Sep 2;9:136. doi: 10.1186/s13023-014-0136-2.

Abstract

Gestational pemphigoid (pemphigoid gestationis, PG) is a rare autoimmune skin disorder occurring characteristically during pregnancy. Autoantibodies against placental BP180 (also known as BPAG2 or collagen XVII) cause damage to the skin basement membrane, resulting in severe itching and blistering rash over the body and the extremities. The diagnosis of PG is confirmed by immunofluorescence analysis of a skin biopsy, while serum levels of pemphigoid antigen BP180 antibody can be used to assess disease activity. PG with mild symptoms can be treated with topical corticosteroids, while oral corticosteroids are the mainstay in treatment of severe PG. PG usually flares up at the time of delivery, and resolves spontaneously shortly after. However, relapses in subsequent pregnancies are common. As PG has been linked to the risk of prematurity and fetal growth restriction, prenatal monitoring jointly by a dermatologist and an obstetrician is recommended. Mothers should also be informed of the potential risk of re-activation of the disease in subsequent pregnancies and during hormonal contraception.

摘要

妊娠类天疱疮(妊娠疱疹,PG)是一种罕见的自身免疫性皮肤病,典型地发生于孕期。针对胎盘BP180(也称为BPAG2或ⅩⅦ型胶原)的自身抗体可导致皮肤基底膜受损,从而在全身及四肢出现严重瘙痒和水疱性皮疹。PG的诊断通过皮肤活检的免疫荧光分析得以证实,而类天疱疮抗原BP180抗体的血清水平可用于评估疾病活动度。症状较轻的PG可用外用糖皮质激素治疗,而口服糖皮质激素是重度PG治疗的主要手段。PG通常在分娩时发作,并在产后不久自行缓解。然而,在随后的妊娠中复发很常见。由于PG与早产及胎儿生长受限风险相关,建议皮肤科医生和产科医生联合进行产前监测。还应告知母亲们疾病在后续妊娠及激素避孕期间再次激活的潜在风险。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a03b/4154519/dda60dcbabf2/13023_2014_136_Fig1_HTML.jpg

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