Pregnancy associated with pemphigus
Pregnancy associated with pemphigus
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妊娠与天疱疮有关
DOI：
--
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发表时间：
1995
期刊：
影响因子：
--
通讯作者：
J. Lessing
中科院分区：
文献类型：
--
作者：
Y. Daniel;M. Shenhav;A. Botchan;M. Peyser;J. Lessing
病例报告一名24岁健康的德系犹太妇女，两年来主诉间歇性外阴和左腋窝病变。皮损包括植被、糜烂和结壳覆盖的脓疱疹。Vulva 1活检示角化过度、角化不全、基底部以上裂隙形成、棘层松解、乳头状瘤病、真皮和表皮嗜酸性粒细胞渗出。免疫荧光显示免疫球蛋白-G(Ig G)和免疫球蛋白C在表皮细胞间隙中沉淀。电子显微镜下可见桥粒破坏。根据这些发现，天疱疮素食者被诊断出来。血清间接免疫荧光为阴性。组织相容性抗原(HLA)分型为A-11、A-28、B-14、B-35、CW4、DR1、DRW1和BW6等位基因。开始用80毫克氟可的松龙(Ultraalan，先灵公司/柏林)进行类固醇治疗，后来在局部用湿敷料和抗生素治疗的基础上加用硫唑嘌呤(100毫克/天)。逐渐愈合伴随着外阴皮肤角化过度和裂隙的形成。两年后，在她的一次缓解期间，她怀孕了。在一个健康的新生儿足月阴道分娩的情况下，妊娠是平安无事的。在接下来的几年里，她遭受了疾病的间歇性发作，仅限于外阴。在突发事件期间，她接受了类固醇治疗，但后来尝试了顺势疗法。在她第二次怀孕期间，疾病严重恶化，在怀孕26周时，她被送往医院。入院时，她的皮肤和粘膜上覆盖了大量的水泡和硬壳。大阴唇覆盖有腐蚀物，无赘生物或脓疱疹。皮肤活检显示典型的寻常型天疱疮改变，伴有腺泡松解、基底部以上裂隙形成，直接免疫荧光显示表皮细胞间隙内有免疫球蛋白抗体。真皮或表皮未见嗜酸性粒细胞渗出。间接免疫荧光显示抗表皮细胞间隙的血清抗体效价为1：80。根据这些发现，寻常型天疱疮
Case report A 24 year old healthy, Ashkenazi Jewish woman complained of intermittent vulval and left axillary lesions for two years. The lesions included vegetation, erosion and pustules covered with crusts. Vulva1 biopsy demonstrated hyperkeratosis, parakeratosis, suprabasal cleft formation, acantholysis, papillomatosis, and eosinophilic infiltration of the dermis and epidermis. Immunofluorescence showed precipitation of immunoglobulin-G (IgG) and C, in the intercellular spaces of the epidermis. Electron microscopy demonstrated destruction of desmosomes. Based on these findings, pemphigus vegetans was diagnosed. Serum indirect immunofluorescence was negative. Histocompatibility antigens (HLA) typing demonstrated A-1 1, A-28, B-14, B-35, CW4, DR1, DRW1, and BW6 alleles. Steroid treatment with 80 mg fluorcortolone (Ultralan, Schering AG/Berlin) was started, and later azathioprine (100 mg/day) was added, in addition to local treatment with wet dressings and antibiotics. Gradual healing was accompanied by the development of hyperkeratosis and fissure formation in the vulval skin. Two years later, during one of her remissions, she conceived. The pregnancy was uneventful with a vaginal delivery at term of a healthy newborn. In subsequent years she suffered from intermittent flare-ups of the disease that were restricted to the vulva. She was treated with steroids during the flare-ups, but later tried homeopathy. During her second pregnancy there was a severe exacerbation of the disease, and at 26 weeks of pregnancy she was admitted to hospital. On admission, bullae and crustae covered an extensive part of her skin and mucous membranes. The labia majora were covered with erosions, with no vegetations or pustules. Skin biopsy showed typical pemphigus vulgaris changes with acantolysis, suprabasal cleft formation, and direct immunofluorescence demonstrated IgG autoantibodies within the intercellular spaces of the epidermis. There was no eosinophyllic infiltration in the dermis or epidermis. Indirect immunofluorescence demonstrated a titre of 1 : 80 serum IgG autoantibodies against the intercellular space of the epidermis. Based on these findings, pemphigus vulgaris was
寻常型天疱疮和素食者。
影响因子：
2.4
作者：
Becker,BA;Gaspari,AA
通讯作者：
Gaspari,AA