A Case of IgG and IgA Anti-Laminin-332 Antibody-Positive Mucous Membrane Pemphigoid with IgG and IgA Anti-Envoplakin and Anti-Periplakin Antibodies

A 76-year-old Japanese man presented with a 6-year history of a sore throat. He was treated at several clinics without any improvement before being referred to us. Physical examination revealed widespread erosions and ulcers from the palate to the larynx. Approximately 25 × 15 mm in size, erosive le...

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Main Authors: Yoshiaki Matsushima, Masako Kitano, Daisuke Hayashi, Hiroyuki Goto, Mako Mine, Takeshi Yokoe, Makoto Kondo, Koji Habe, Yuji Toiyama, Takashi Hashimoto, Daisuke Tsuruta, Kazuhiko Takeuchi, Keiichi Yamanaka
Format: Article
Language:English
Published: MDPI AG 2022-08-01
Series:Dermatopathology
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Online Access:https://www.mdpi.com/2296-3529/9/3/34
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author Yoshiaki Matsushima
Masako Kitano
Daisuke Hayashi
Hiroyuki Goto
Mako Mine
Takeshi Yokoe
Makoto Kondo
Koji Habe
Yuji Toiyama
Takashi Hashimoto
Daisuke Tsuruta
Kazuhiko Takeuchi
Keiichi Yamanaka
author_facet Yoshiaki Matsushima
Masako Kitano
Daisuke Hayashi
Hiroyuki Goto
Mako Mine
Takeshi Yokoe
Makoto Kondo
Koji Habe
Yuji Toiyama
Takashi Hashimoto
Daisuke Tsuruta
Kazuhiko Takeuchi
Keiichi Yamanaka
author_sort Yoshiaki Matsushima
collection DOAJ
description A 76-year-old Japanese man presented with a 6-year history of a sore throat. He was treated at several clinics without any improvement before being referred to us. Physical examination revealed widespread erosions and ulcers from the palate to the larynx. Approximately 25 × 15 mm in size, erosive lesions were present on the retroauricular regions, forearms, and glans penis. Pseudomembranous conjunctivitis was also observed. The skin biopsy revealed a partial cleft formation below the epidermis, suggesting subepidermal bullous disease. Immuno-serological tests were negative for anti-desmoglein 1 (Dsg1), anti-Dsg3, anti-BP180, and anti-BP230 antibodies by ELISAs. A whole-body examination revealed gastric cancer. The possibility of mucous membrane pemphigoid (MMP) or paraneoplastic pemphigus (PNP) was considered. Indirect immunofluorescence using rat bladders showed positive IgG reactivity with cell surfaces on the transitional epithelia. Immunoblotting using recombinant proteins of laminin-332 showed both IgG and IgA reactivities with laminin-α3, and immunoblotting using normal human epidermal extract showed double-positive reactivities with envoplakin and periplakin for both IgG and IgA antibodies. Based on the clinical and histopathological features and results of various immuno-serological tests, our case was diagnosed as anti-laminin-332-type MMP with serological findings of PNP. Twenty days after laparoscopic gastrectomy, treatment with oral methylprednisolone 32 mg/day was initiated, and mucosal and skin lesions improved.
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spelling doaj.art-5c53a2c4c0b6488189845d69b433eb302023-11-23T15:47:28ZengMDPI AGDermatopathology2296-35292022-08-019328729110.3390/dermatopathology9030034A Case of IgG and IgA Anti-Laminin-332 Antibody-Positive Mucous Membrane Pemphigoid with IgG and IgA Anti-Envoplakin and Anti-Periplakin AntibodiesYoshiaki Matsushima0Masako Kitano1Daisuke Hayashi2Hiroyuki Goto3Mako Mine4Takeshi Yokoe5Makoto Kondo6Koji Habe7Yuji Toiyama8Takashi Hashimoto9Daisuke Tsuruta10Kazuhiko Takeuchi11Keiichi Yamanaka12Department of Dermatology, Graduate School of Medicine, Mie University, Tsu 514-8507, JapanDepartment of Otorhinolaryngology—Head & Neck Surgery, Graduate School of Medicine, Mie University, Tsu 514-8507, JapanDepartment of Dermatology, Graduate School of Medicine, Osaka Metropolitan University, Osaka 545-8585, JapanDepartment of Dermatology, Graduate School of Medicine, Mie University, Tsu 514-8507, JapanDepartment of Dermatology, Graduate School of Medicine, Osaka Metropolitan University, Osaka 545-8585, JapanDepartment of Gastrointestinal and Pediatric Surgery, Graduate School of Medicine, Mie University, Tsu 514-8507, JapanDepartment of Dermatology, Graduate School of Medicine, Mie University, Tsu 514-8507, JapanDepartment of Dermatology, Graduate School of Medicine, Mie University, Tsu 514-8507, JapanDepartment of Gastrointestinal and Pediatric Surgery, Graduate School of Medicine, Mie University, Tsu 514-8507, JapanDepartment of Dermatology, Graduate School of Medicine, Osaka Metropolitan University, Osaka 545-8585, JapanDepartment of Dermatology, Graduate School of Medicine, Osaka Metropolitan University, Osaka 545-8585, JapanDepartment of Otorhinolaryngology—Head & Neck Surgery, Graduate School of Medicine, Mie University, Tsu 514-8507, JapanDepartment of Dermatology, Graduate School of Medicine, Mie University, Tsu 514-8507, JapanA 76-year-old Japanese man presented with a 6-year history of a sore throat. He was treated at several clinics without any improvement before being referred to us. Physical examination revealed widespread erosions and ulcers from the palate to the larynx. Approximately 25 × 15 mm in size, erosive lesions were present on the retroauricular regions, forearms, and glans penis. Pseudomembranous conjunctivitis was also observed. The skin biopsy revealed a partial cleft formation below the epidermis, suggesting subepidermal bullous disease. Immuno-serological tests were negative for anti-desmoglein 1 (Dsg1), anti-Dsg3, anti-BP180, and anti-BP230 antibodies by ELISAs. A whole-body examination revealed gastric cancer. The possibility of mucous membrane pemphigoid (MMP) or paraneoplastic pemphigus (PNP) was considered. Indirect immunofluorescence using rat bladders showed positive IgG reactivity with cell surfaces on the transitional epithelia. Immunoblotting using recombinant proteins of laminin-332 showed both IgG and IgA reactivities with laminin-α3, and immunoblotting using normal human epidermal extract showed double-positive reactivities with envoplakin and periplakin for both IgG and IgA antibodies. Based on the clinical and histopathological features and results of various immuno-serological tests, our case was diagnosed as anti-laminin-332-type MMP with serological findings of PNP. Twenty days after laparoscopic gastrectomy, treatment with oral methylprednisolone 32 mg/day was initiated, and mucosal and skin lesions improved.https://www.mdpi.com/2296-3529/9/3/34mucous membrane pemphigoidanti-laminin-332laminin-α3envoplakinperiplakin
spellingShingle Yoshiaki Matsushima
Masako Kitano
Daisuke Hayashi
Hiroyuki Goto
Mako Mine
Takeshi Yokoe
Makoto Kondo
Koji Habe
Yuji Toiyama
Takashi Hashimoto
Daisuke Tsuruta
Kazuhiko Takeuchi
Keiichi Yamanaka
A Case of IgG and IgA Anti-Laminin-332 Antibody-Positive Mucous Membrane Pemphigoid with IgG and IgA Anti-Envoplakin and Anti-Periplakin Antibodies
Dermatopathology
mucous membrane pemphigoid
anti-laminin-332
laminin-α3
envoplakin
periplakin
title A Case of IgG and IgA Anti-Laminin-332 Antibody-Positive Mucous Membrane Pemphigoid with IgG and IgA Anti-Envoplakin and Anti-Periplakin Antibodies
title_full A Case of IgG and IgA Anti-Laminin-332 Antibody-Positive Mucous Membrane Pemphigoid with IgG and IgA Anti-Envoplakin and Anti-Periplakin Antibodies
title_fullStr A Case of IgG and IgA Anti-Laminin-332 Antibody-Positive Mucous Membrane Pemphigoid with IgG and IgA Anti-Envoplakin and Anti-Periplakin Antibodies
title_full_unstemmed A Case of IgG and IgA Anti-Laminin-332 Antibody-Positive Mucous Membrane Pemphigoid with IgG and IgA Anti-Envoplakin and Anti-Periplakin Antibodies
title_short A Case of IgG and IgA Anti-Laminin-332 Antibody-Positive Mucous Membrane Pemphigoid with IgG and IgA Anti-Envoplakin and Anti-Periplakin Antibodies
title_sort case of igg and iga anti laminin 332 antibody positive mucous membrane pemphigoid with igg and iga anti envoplakin and anti periplakin antibodies
topic mucous membrane pemphigoid
anti-laminin-332
laminin-α3
envoplakin
periplakin
url https://www.mdpi.com/2296-3529/9/3/34
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