A case of herpetiform pemphigus with anti-desmoglein 3 IgG autoantibodies

Rieko Isogai, Akira Kawada, Yoshinori Aragane, Masayuki Amagai, Tadashi Tezuka

Research output: Contribution to journalArticle

11 Citations (Scopus)

Abstract

Herpetiform pemphigus (HP) is a rare variant of pemphigus characterized by a unique clinical phenotype of erythematous or urticarial plaques and vesicles that present in a herpetiform arrangement. Most HP cases have circulating anti-desmoglein 1 (Dsg1) IgG autoantibodies, but some HP cases have anti-desmoglein 3 (Dsg3) IgG. A 92-year-old Japanese woman presented with severely pruritic annular erythema and vesicles in a herpetiform arrangement on the trunk. No oral mucosal lesions were present. Histopathologically, these vesicles showed eosinophilic spongiosis as well as suprabasilar acantholysis. Direct immunofluorescence showed in vivo IgG deposition on keratinocyte cell surfaces, and indirect immunofluorescence showed circulating IgG autoantibodies against keratinocyte cell surfaces at a titer of 1:30. Enzyme-linked immunosorbent assay using recombinant Dsg1 and Dsg3 revealed the presence of anti-Dsg3 IgG but no anti-Dsg1 IgG autoantibodies. The lack of oral mucosal involvement and the unique clinical features favored the diagnosis of HP. It remains to be clarified why the anti-Dsg3 IgG autoantibodies in this patient induced this unique features of HP, rather than the mucosal dominant type of pemphigus vulgaris.

Original languageEnglish
Pages (from-to)407-410
Number of pages4
JournalJournal of Dermatology
Volume31
Issue number5
Publication statusPublished - 2004 May

Fingerprint

Desmoglein 3
Pemphigus
Autoantibodies
Immunoglobulin G
Desmoglein 1
Keratinocytes
Acantholysis
Direct Fluorescent Antibody Technique
Indirect Fluorescent Antibody Technique
Enzyme-Linked Immunosorbent Assay
Phenotype

Keywords

  • Anti-Desmoglein 3 antibodies
  • Eosinophilic spongiosis
  • Herpetiform pemphigus

ASJC Scopus subject areas

  • Dermatology

Cite this

Isogai, R., Kawada, A., Aragane, Y., Amagai, M., & Tezuka, T. (2004). A case of herpetiform pemphigus with anti-desmoglein 3 IgG autoantibodies. Journal of Dermatology, 31(5), 407-410.

A case of herpetiform pemphigus with anti-desmoglein 3 IgG autoantibodies. / Isogai, Rieko; Kawada, Akira; Aragane, Yoshinori; Amagai, Masayuki; Tezuka, Tadashi.

In: Journal of Dermatology, Vol. 31, No. 5, 05.2004, p. 407-410.

Research output: Contribution to journalArticle

Isogai, R, Kawada, A, Aragane, Y, Amagai, M & Tezuka, T 2004, 'A case of herpetiform pemphigus with anti-desmoglein 3 IgG autoantibodies', Journal of Dermatology, vol. 31, no. 5, pp. 407-410.
Isogai, Rieko ; Kawada, Akira ; Aragane, Yoshinori ; Amagai, Masayuki ; Tezuka, Tadashi. / A case of herpetiform pemphigus with anti-desmoglein 3 IgG autoantibodies. In: Journal of Dermatology. 2004 ; Vol. 31, No. 5. pp. 407-410.
@article{987570d2c7434cf2bd8efa7f73a4a90f,
title = "A case of herpetiform pemphigus with anti-desmoglein 3 IgG autoantibodies",
abstract = "Herpetiform pemphigus (HP) is a rare variant of pemphigus characterized by a unique clinical phenotype of erythematous or urticarial plaques and vesicles that present in a herpetiform arrangement. Most HP cases have circulating anti-desmoglein 1 (Dsg1) IgG autoantibodies, but some HP cases have anti-desmoglein 3 (Dsg3) IgG. A 92-year-old Japanese woman presented with severely pruritic annular erythema and vesicles in a herpetiform arrangement on the trunk. No oral mucosal lesions were present. Histopathologically, these vesicles showed eosinophilic spongiosis as well as suprabasilar acantholysis. Direct immunofluorescence showed in vivo IgG deposition on keratinocyte cell surfaces, and indirect immunofluorescence showed circulating IgG autoantibodies against keratinocyte cell surfaces at a titer of 1:30. Enzyme-linked immunosorbent assay using recombinant Dsg1 and Dsg3 revealed the presence of anti-Dsg3 IgG but no anti-Dsg1 IgG autoantibodies. The lack of oral mucosal involvement and the unique clinical features favored the diagnosis of HP. It remains to be clarified why the anti-Dsg3 IgG autoantibodies in this patient induced this unique features of HP, rather than the mucosal dominant type of pemphigus vulgaris.",
keywords = "Anti-Desmoglein 3 antibodies, Eosinophilic spongiosis, Herpetiform pemphigus",
author = "Rieko Isogai and Akira Kawada and Yoshinori Aragane and Masayuki Amagai and Tadashi Tezuka",
year = "2004",
month = "5",
language = "English",
volume = "31",
pages = "407--410",
journal = "Journal of Dermatology",
issn = "0385-2407",
publisher = "Wiley-Blackwell",
number = "5",

}

TY - JOUR

T1 - A case of herpetiform pemphigus with anti-desmoglein 3 IgG autoantibodies

AU - Isogai, Rieko

AU - Kawada, Akira

AU - Aragane, Yoshinori

AU - Amagai, Masayuki

AU - Tezuka, Tadashi

PY - 2004/5

Y1 - 2004/5

N2 - Herpetiform pemphigus (HP) is a rare variant of pemphigus characterized by a unique clinical phenotype of erythematous or urticarial plaques and vesicles that present in a herpetiform arrangement. Most HP cases have circulating anti-desmoglein 1 (Dsg1) IgG autoantibodies, but some HP cases have anti-desmoglein 3 (Dsg3) IgG. A 92-year-old Japanese woman presented with severely pruritic annular erythema and vesicles in a herpetiform arrangement on the trunk. No oral mucosal lesions were present. Histopathologically, these vesicles showed eosinophilic spongiosis as well as suprabasilar acantholysis. Direct immunofluorescence showed in vivo IgG deposition on keratinocyte cell surfaces, and indirect immunofluorescence showed circulating IgG autoantibodies against keratinocyte cell surfaces at a titer of 1:30. Enzyme-linked immunosorbent assay using recombinant Dsg1 and Dsg3 revealed the presence of anti-Dsg3 IgG but no anti-Dsg1 IgG autoantibodies. The lack of oral mucosal involvement and the unique clinical features favored the diagnosis of HP. It remains to be clarified why the anti-Dsg3 IgG autoantibodies in this patient induced this unique features of HP, rather than the mucosal dominant type of pemphigus vulgaris.

AB - Herpetiform pemphigus (HP) is a rare variant of pemphigus characterized by a unique clinical phenotype of erythematous or urticarial plaques and vesicles that present in a herpetiform arrangement. Most HP cases have circulating anti-desmoglein 1 (Dsg1) IgG autoantibodies, but some HP cases have anti-desmoglein 3 (Dsg3) IgG. A 92-year-old Japanese woman presented with severely pruritic annular erythema and vesicles in a herpetiform arrangement on the trunk. No oral mucosal lesions were present. Histopathologically, these vesicles showed eosinophilic spongiosis as well as suprabasilar acantholysis. Direct immunofluorescence showed in vivo IgG deposition on keratinocyte cell surfaces, and indirect immunofluorescence showed circulating IgG autoantibodies against keratinocyte cell surfaces at a titer of 1:30. Enzyme-linked immunosorbent assay using recombinant Dsg1 and Dsg3 revealed the presence of anti-Dsg3 IgG but no anti-Dsg1 IgG autoantibodies. The lack of oral mucosal involvement and the unique clinical features favored the diagnosis of HP. It remains to be clarified why the anti-Dsg3 IgG autoantibodies in this patient induced this unique features of HP, rather than the mucosal dominant type of pemphigus vulgaris.

KW - Anti-Desmoglein 3 antibodies

KW - Eosinophilic spongiosis

KW - Herpetiform pemphigus

UR - http://www.scopus.com/inward/record.url?scp=3042531465&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=3042531465&partnerID=8YFLogxK

M3 - Article

C2 - 15187309

AN - SCOPUS:3042531465

VL - 31

SP - 407

EP - 410

JO - Journal of Dermatology

JF - Journal of Dermatology

SN - 0385-2407

IS - 5

ER -