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Erfolgreiche Therapie des IgA-Pemphigus vom subkorneal pustulösen Typ

Successful treatment of subcorneal pustular type of IgA pemphigus

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Zusammenfassung

Der IgA-Pemphigus ist eine seltene Erkrankung aus der Gruppe der bullösen Autoimmundermatosen, der durch Autoantikörper der Klasse IgA, die gegen Zielstrukturen (Cadherine) der Epidermis gerichtet sind, charakterisiert wird. Das Manifestationsalter des IgA-Pemphigus betrifft das 50. Lebensjahr. Histopathologische Hauptmerkmale des IgA-Pemphigus sind die Akantholyse mit subkornealer oder intraepidermaler Pustulation und teilweise epidermaler Infiltration neutrophiler Granulozyten. Charakteristisch für das klinische Erscheinungsbild sind erythematöse Papeln, Pusteln, Krusten, Schuppen und Erosionen, die insbesondere am Rumpf, an den Axillen, Leisten und proximalen Extremitäten auftreten. Das Mittel der Wahl zur Therapie des IgA-Pemphigus ist Diaminodiphenylsulfon. In der vorliegenden Kasuistik berichten wir über einen Patienten, der an einem IgA-Pemphigus erkrankt war und erfolgreich mit Diaminodiphenylsulfon behandelt wurde.

Abstract

IgA pemphigus is a rare autoimmune intraepidermal blistering disease characterized by in vivo bound, and circulating IgA autoantibodies that target cell surface components (cadherins) of epidermal keratinocytes. The average age of onset is approximately 50 years. Histological hallmarks of IgA pemphigus are epidermal acantholysis, subcorneal or intraepidermal pustulosis, and neutrophilic infiltration. The main clinical characteristics are erythematous skin lesions with vesiculopustules, erosions, crusts and desquamation, favoring the trunk, groins, axillaries, and proximal extremities. The drug of choice for treating IgA pemphigus is diaminodiphenylsulfone. In this case study, we describe a patient who suffered from IgA pemphigus and responded well to diaminodiphenylsulfone.

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Authors and Affiliations

  1. Klinik und Poliklinik für Dermatologie und Venerologie der Medizinischen Hochschule Hannover

    U. Raap, B. Völker, H. Petering, A. Kapp & T. Werfel

  2. Klinik für Dermatologie und Venerologie, Universitätsklinikum Schleswig-Holstein, Campus Lübeck

    D. Zillikens

  3. Department of Dermatology, Kurume University School of Medicine, Fukuoka, Japan

    T. Hashimoto

  4. Klinik und Poliklinik für Dermatologie und Venerologie, Medizinische Hochschule Hannover, Hautklinik Linden, Ricklingerstraße 5, 30451 , Hannover

    U. Raap

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  1. U. Raap
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  2. B. Völker
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  3. H. Petering
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  4. T. Hashimoto
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  5. D. Zillikens
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  6. A. Kapp
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  7. T. Werfel
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Correspondence to U. Raap.

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Raap, U., Völker, B., Petering, H. et al. Erfolgreiche Therapie des IgA-Pemphigus vom subkorneal pustulösen Typ. Hautarzt 56, 66–70 (2005). https://doi.org/10.1007/s00105-004-0822-4

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  • DOI: https://doi.org/10.1007/s00105-004-0822-4

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