Bullous pemphigoid associated with milia, increased serum IgE, autoantibodies against desmogleins, and refractory treatment in a young patient

Abstract: Bullous pemphigoid is a blistering autoimmune disease characterized by two hemidesmosomal proteins (anti-BP180 and 230). Pemphigus, by contrast, is characterized by two autoantibodies (anti-desmoglein 1 and 3). Coexistence of autoantibodies of bullous pemphigoid and pemphigus in a patient...

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Main Authors: Shu Ding, Qiancheng Deng, Yaping Xiang, Jing Chen, Jinhua Huang, Jianyun Lu
Format: Article
Language:English
Published: Sociedade Brasileira de Dermatologia 2017-01-01
Series:Anais Brasileiros de Dermatologia
Subjects:
Online Access:http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100034&tlng=en
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author Shu Ding
Qiancheng Deng
Yaping Xiang
Jing Chen
Jinhua Huang
Jianyun Lu
author_facet Shu Ding
Qiancheng Deng
Yaping Xiang
Jing Chen
Jinhua Huang
Jianyun Lu
author_sort Shu Ding
collection DOAJ
description Abstract: Bullous pemphigoid is a blistering autoimmune disease characterized by two hemidesmosomal proteins (anti-BP180 and 230). Pemphigus, by contrast, is characterized by two autoantibodies (anti-desmoglein 1 and 3). Coexistence of autoantibodies of bullous pemphigoid and pemphigus in a patient is rare. A 25-year-old male patient was admitted to our hospital, reporting a 3-month history of multiple papules, vesicles, and erosions over an extensive erythema on the entire body. Laboratory tests showed high levels of serum IgE, anti-BP180 antibodies, and anti-desmoglein 1 and 3. Histopathologic and immunopathologic features were characterized by bullous pemphigoid. No improvement was seen with systemic corticosteroid therapy, however, pulse corticosteriod therapy combined with methylprednisolone, immunosuppressants, immunomodulators, and plasmapheresis led to the recovery of his condition with numerous milia.
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spelling doaj.art-e588a93d23484953978a8c04e37228232022-12-21T21:28:39ZengSociedade Brasileira de DermatologiaAnais Brasileiros de Dermatologia0365-05962017-01-01925 suppl 1343610.1590/abd1806-4841.20176124Bullous pemphigoid associated with milia, increased serum IgE, autoantibodies against desmogleins, and refractory treatment in a young patientShu DingQiancheng DengYaping XiangJing ChenJinhua HuangJianyun LuAbstract: Bullous pemphigoid is a blistering autoimmune disease characterized by two hemidesmosomal proteins (anti-BP180 and 230). Pemphigus, by contrast, is characterized by two autoantibodies (anti-desmoglein 1 and 3). Coexistence of autoantibodies of bullous pemphigoid and pemphigus in a patient is rare. A 25-year-old male patient was admitted to our hospital, reporting a 3-month history of multiple papules, vesicles, and erosions over an extensive erythema on the entire body. Laboratory tests showed high levels of serum IgE, anti-BP180 antibodies, and anti-desmoglein 1 and 3. Histopathologic and immunopathologic features were characterized by bullous pemphigoid. No improvement was seen with systemic corticosteroid therapy, however, pulse corticosteriod therapy combined with methylprednisolone, immunosuppressants, immunomodulators, and plasmapheresis led to the recovery of his condition with numerous milia.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100034&tlng=enDesmoglein 1MiliariaPemphigoid, bullousPemphigus
spellingShingle Shu Ding
Qiancheng Deng
Yaping Xiang
Jing Chen
Jinhua Huang
Jianyun Lu
Bullous pemphigoid associated with milia, increased serum IgE, autoantibodies against desmogleins, and refractory treatment in a young patient
Anais Brasileiros de Dermatologia
Desmoglein 1
Miliaria
Pemphigoid, bullous
Pemphigus
title Bullous pemphigoid associated with milia, increased serum IgE, autoantibodies against desmogleins, and refractory treatment in a young patient
title_full Bullous pemphigoid associated with milia, increased serum IgE, autoantibodies against desmogleins, and refractory treatment in a young patient
title_fullStr Bullous pemphigoid associated with milia, increased serum IgE, autoantibodies against desmogleins, and refractory treatment in a young patient
title_full_unstemmed Bullous pemphigoid associated with milia, increased serum IgE, autoantibodies against desmogleins, and refractory treatment in a young patient
title_short Bullous pemphigoid associated with milia, increased serum IgE, autoantibodies against desmogleins, and refractory treatment in a young patient
title_sort bullous pemphigoid associated with milia increased serum ige autoantibodies against desmogleins and refractory treatment in a young patient
topic Desmoglein 1
Miliaria
Pemphigoid, bullous
Pemphigus
url http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017001100034&tlng=en
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