大疱性类天疱疮180抗体阳性的寻常型天疱疮3例
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摘要
报告3例大疱性类天疱疮(BP)180抗体阳性的寻常型天疱疮患者。所有患者均以口腔、外阴黏膜糜烂,躯干多发水疱逐渐加重就诊。皮肤科检查:患者分别在头皮、面部、颈部、口腔、外阴、躯干及下肢等部位出现绿豆至花生米大的孤立或融合性水疱,部分糜烂面潮湿。实验室检查:血清检测抗桥粒芯糖蛋白(Dsg)抗体和Bp180抗体均阳性,直接免疫荧光(DIF)示抗棘细胞桥粒抗体(PCDA)阳性或抗表皮基膜抗体(EBA)阳性。皮损组织病理检查:表皮内水疱,棘细胞松解,基底呈"绒毛"样改变,基底层见中量淋巴细胞浸润。结合临床及实验室检查诊断为BP180抗体阳性的寻常型天疱疮。
Three cases of phemphigus vulgaris with positive anti-BP180 antibody are reported. These patients presented with oral and genital mucosal erosion, and aggravating blisters on the trunk. Physical examination showed isolated and merged blisters sized from green bean to peanut on the scalp, face and neck, mouse, external genitalia, chest, back and lower limbs.Some blisters were with erosion. Laboratory tests showed positive for both serum anti-desmoglein(Dsg) and anti-BP180 antibody. Direct immunofluorescence(DIF) showed positive for PCDA or EBA antibody. Pathological examination revealed intraepidermal blisters, acantholysis, formation of villi by basal cells and lymphocytic infiltrate of the basal layer. Combined the clinical features with laboratory results, these patients were diagnosed of phemphigus vulgaris with anti-BPp180 antibody positive.
Three cases of phemphigus vulgaris with positive anti-BP180 antibody are reported. These patients presented with oral and genital mucosal erosion, and aggravating blisters on the trunk. Physical examination showed isolated and merged blisters sized from green bean to peanut on the scalp, face and neck, mouse, external genitalia, chest, back and lower limbs.Some blisters were with erosion. Laboratory tests showed positive for both serum anti-desmoglein(Dsg) and anti-BP180 antibody. Direct immunofluorescence(DIF) showed positive for PCDA or EBA antibody. Pathological examination revealed intraepidermal blisters, acantholysis, formation of villi by basal cells and lymphocytic infiltrate of the basal layer. Combined the clinical features with laboratory results, these patients were diagnosed of phemphigus vulgaris with anti-BPp180 antibody positive.
引文
[1]朱学骏,赵景辉,陈喜.重症天疱疮21例治疗分析[J].临床皮肤科杂志,2003,32(3):132-134.
[2]Ettlin DA.Pemphigus[J].Dent Clin North Am,2005,49(1):107-125.
[3]Mahoney MG,Wang Z,Rothenberger K,et al.Explanations for the clinical and microscopic localization of lesions in pemphigus foliaceus and vulgaris[J].J Clin Invest,1999,103(4):461-468.
[4]Li Q,Ujiie H,Shibaki A,et al.Human Ig G1 monoclonal antibody against human collagen 17 noncollagenous 16A domain induces blisters via complement activation in experimental bullous pemphigoid model[J].J Immunol,2010,185(12):7746-7755.
[5]Schmidt E,Goebeler M,Hertl M,et al.S2k guideline for the diagnosis of pemphigus vulgaris/foliaceus and bullous pemphigoid[J].J Dtsch Dermatol Ges,2015,13(7):713-727.
[6]Fairley JA,Baum CL,Brandt DS,et al.Pathogenicity of Ig E in autoimmunity:successful treatment of bullous pemphigoid with omalizumab[J].J Allergy Clin Immunol,2009,123(3):704-705.
[7]Yalcin AD,Genc GE,Celik B,et al.Anti-Ig E monoclonal antibody(omalizumab)is effective in treating bullous pemphigoid and its effects on soluble CD200[J].Clin Lab,2014,60(3):523-524.
[8]Cassano N,Mastrandrea V,Tampoia M,et al.Pemphigus vulgaris with circulating anti-desmoglein 3 and anti-BP180 antibodies:a case report and brief review of cases with coexistence of pemphigus vulgaris and bullous pemphigoid[J].J Biol Regul Homeost Agents,2009,23(3):197-201.
[9]Kobayashi K,Tanaka M,Nakajima S,et al.Simultaneous occurrence of anti-BP180 mucous membrane pemphigoid and mucosal-dominant pemphigus vulgaris[J].Clin Exp Dermatol,2009,34(8):e785-e788.
[10]Shams S,Amirzargar AA,Yousefi M,et al.HLA classⅡ(DRB,DQA1 and DQB1)allele and haplotype frequencies in the patients with pemphigus vulgaris[J].J Clin Immunol,2009,29(2):175-179.
[11]Svecova D,Parnicka Z,Pastyrikova L,et al.HLA DRB1*and DQB1*alleles are associated with disease severity in patients with pemphigus vulgaris[J].Int J Dermatol,2015,54(2):168-173.
[12]吴桂菊,张勇,朱海琴,等.HLA-DRB1和HLA-DQB1基因多态性与天疱疮的相关性[J].上海交通大学学报(医学版),2011,31(7):948-951.
[13]Eming R,Hennerici T,B覿cklund J,et al.Pathogenic Ig G antibodies against desmoglein 3 in pemphigus vulgaris are regulated by HLA-DRB1*04:02-restricted T cells[J].J Immunol,2014,193(9):4391-4399.
[14]Lee E,Lendas KA,Chow S,et al.Disease relevant HLA classⅡalleles isolated by genotypic,haplotypic,and sequence analysis in North American Caucasians with pemphigus vulgaris[J].Hum Immunol,2006,67(1-2):125-139.
[15]Oyama N,Setterfield JF,Powell AM,et al.Bullous pemphigoid antigenⅡ(BP180)and its soluble extracellular domains are major autoantigens in mucous membrane pemphigoid:the pathogenic relevance to HLA classⅡalleles and disease severity[J].Br J Dermatol,2006,154(1):90-98.
[16]徐牧迟,潘萌,郑捷.寻常型天疱疮并发黏膜型类天疱疮[J].临床皮肤科杂志,2012,41(5):322-324.
[17]Mc Grath JA,Gatalica B,Christiano AM,et al.Mutations in the180-k D bullous pemphigoid antigen(BPAG2),a hemidesmosomal transmembrane collagen(COL17A1),in generalized atrophic benign epidermolysis bullosa[J].Nat Genet,1995,11(1):83-86.
[18]朱晓浚,刘少珍,刘次伟.糖皮质激素联合环磷酰胺治疗寻常型天疱疮疗效观察[J].中国麻风皮肤病杂志,2004,20(5):430-432.
[2]Ettlin DA.Pemphigus[J].Dent Clin North Am,2005,49(1):107-125.
[3]Mahoney MG,Wang Z,Rothenberger K,et al.Explanations for the clinical and microscopic localization of lesions in pemphigus foliaceus and vulgaris[J].J Clin Invest,1999,103(4):461-468.
[4]Li Q,Ujiie H,Shibaki A,et al.Human Ig G1 monoclonal antibody against human collagen 17 noncollagenous 16A domain induces blisters via complement activation in experimental bullous pemphigoid model[J].J Immunol,2010,185(12):7746-7755.
[5]Schmidt E,Goebeler M,Hertl M,et al.S2k guideline for the diagnosis of pemphigus vulgaris/foliaceus and bullous pemphigoid[J].J Dtsch Dermatol Ges,2015,13(7):713-727.
[6]Fairley JA,Baum CL,Brandt DS,et al.Pathogenicity of Ig E in autoimmunity:successful treatment of bullous pemphigoid with omalizumab[J].J Allergy Clin Immunol,2009,123(3):704-705.
[7]Yalcin AD,Genc GE,Celik B,et al.Anti-Ig E monoclonal antibody(omalizumab)is effective in treating bullous pemphigoid and its effects on soluble CD200[J].Clin Lab,2014,60(3):523-524.
[8]Cassano N,Mastrandrea V,Tampoia M,et al.Pemphigus vulgaris with circulating anti-desmoglein 3 and anti-BP180 antibodies:a case report and brief review of cases with coexistence of pemphigus vulgaris and bullous pemphigoid[J].J Biol Regul Homeost Agents,2009,23(3):197-201.
[9]Kobayashi K,Tanaka M,Nakajima S,et al.Simultaneous occurrence of anti-BP180 mucous membrane pemphigoid and mucosal-dominant pemphigus vulgaris[J].Clin Exp Dermatol,2009,34(8):e785-e788.
[10]Shams S,Amirzargar AA,Yousefi M,et al.HLA classⅡ(DRB,DQA1 and DQB1)allele and haplotype frequencies in the patients with pemphigus vulgaris[J].J Clin Immunol,2009,29(2):175-179.
[11]Svecova D,Parnicka Z,Pastyrikova L,et al.HLA DRB1*and DQB1*alleles are associated with disease severity in patients with pemphigus vulgaris[J].Int J Dermatol,2015,54(2):168-173.
[12]吴桂菊,张勇,朱海琴,等.HLA-DRB1和HLA-DQB1基因多态性与天疱疮的相关性[J].上海交通大学学报(医学版),2011,31(7):948-951.
[13]Eming R,Hennerici T,B覿cklund J,et al.Pathogenic Ig G antibodies against desmoglein 3 in pemphigus vulgaris are regulated by HLA-DRB1*04:02-restricted T cells[J].J Immunol,2014,193(9):4391-4399.
[14]Lee E,Lendas KA,Chow S,et al.Disease relevant HLA classⅡalleles isolated by genotypic,haplotypic,and sequence analysis in North American Caucasians with pemphigus vulgaris[J].Hum Immunol,2006,67(1-2):125-139.
[15]Oyama N,Setterfield JF,Powell AM,et al.Bullous pemphigoid antigenⅡ(BP180)and its soluble extracellular domains are major autoantigens in mucous membrane pemphigoid:the pathogenic relevance to HLA classⅡalleles and disease severity[J].Br J Dermatol,2006,154(1):90-98.
[16]徐牧迟,潘萌,郑捷.寻常型天疱疮并发黏膜型类天疱疮[J].临床皮肤科杂志,2012,41(5):322-324.
[17]Mc Grath JA,Gatalica B,Christiano AM,et al.Mutations in the180-k D bullous pemphigoid antigen(BPAG2),a hemidesmosomal transmembrane collagen(COL17A1),in generalized atrophic benign epidermolysis bullosa[J].Nat Genet,1995,11(1):83-86.
[18]朱晓浚,刘少珍,刘次伟.糖皮质激素联合环磷酰胺治疗寻常型天疱疮疗效观察[J].中国麻风皮肤病杂志,2004,20(5):430-432.