Pemphigus vegetans mit autoantikorpern gegen rekombinantes desmoglein 3

J. Arnold, Chr Rose, Masayuki Amagai, E. B. Brocker, D. Zillikens

研究成果: Article

2 引用 (Scopus)

抄録

Pemphigus vegetans is a rare variant of pemphigus vulgaris and belongs to the group of autoimmune blistering diseases. A 73-year old female patient suffering from pemphigus vulgaris presented with erosions on oral and laryngeal mucous membranes. With systemic treatment the mucous membrane lesions improved, but a vegetating erosive plaque developed on the left inguinal region. A biopsy from this site showed acanthosis and suprabasal acantholytic blister formation. Direct immunofluorescence microscopy revealed an epidermal intercellular deposition of IgG antibodies. Indirect immunofluorescence analysis using monkey oesophagus as a substrate demonstrated the presence of circulating IgG autoantibodies binding to the epithelium with an intercellular staining pattern and a titer of 1:320. By ELISA, using recombinant forms of desmoglein 1 and 3, the serum of our patient showed high levels of autoantibodies to desmoglein 3, but not to desmoglein 1. Systemic treatment with a intravenous pulse therapy of dexamethasone and cyclophosphamide as well as intralesional injections of the left inguinal region led to complete remission of the skin lesions. This report confirms the close relationship between pemphigus vulgaris and pemphigus vegetans. With regard to this case, we discuss clinical spectrum, diagnosis and therapeutic options of pemphigus vegetans. To the best of our knowledge, this is the first case of pemphigus vegetans in which the autoimmune response has been characterized using recombinant forms of desmoglein 1 and 3.

元の言語German
ページ(範囲)241-244
ページ数4
ジャーナルAktuelle Dermatologie
26
発行部数7
出版物ステータスPublished - 2000

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Desmoglein 3
Pemphigus
Autoantibodies
Desmoglein 1
Groin
Mucous Membrane
Immunoglobulin G
Intralesional Injections
Direct Fluorescent Antibody Technique
Therapeutics
Blister
Indirect Fluorescent Antibody Technique
vegetan
Autoimmunity
Fluorescence Microscopy
Cyclophosphamide
Dexamethasone
Esophagus
Autoimmune Diseases
Haplorhini

ASJC Scopus subject areas

  • Dermatology

これを引用

Arnold, J., Rose, C., Amagai, M., Brocker, E. B., & Zillikens, D. (2000). Pemphigus vegetans mit autoantikorpern gegen rekombinantes desmoglein 3. Aktuelle Dermatologie, 26(7), 241-244.

Pemphigus vegetans mit autoantikorpern gegen rekombinantes desmoglein 3. / Arnold, J.; Rose, Chr; Amagai, Masayuki; Brocker, E. B.; Zillikens, D.

:: Aktuelle Dermatologie, 巻 26, 番号 7, 2000, p. 241-244.

研究成果: Article

Arnold, J, Rose, C, Amagai, M, Brocker, EB & Zillikens, D 2000, 'Pemphigus vegetans mit autoantikorpern gegen rekombinantes desmoglein 3', Aktuelle Dermatologie, 巻. 26, 番号 7, pp. 241-244.
Arnold, J. ; Rose, Chr ; Amagai, Masayuki ; Brocker, E. B. ; Zillikens, D. / Pemphigus vegetans mit autoantikorpern gegen rekombinantes desmoglein 3. :: Aktuelle Dermatologie. 2000 ; 巻 26, 番号 7. pp. 241-244.
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