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Anti-p200/Laminin-γ1-Pemphigoid in Kombination mit einem BP180 NC16A/4575-positiven Schleimhautpemphigoid

Späte Diagnosestellung bei krankheitsbedingter Amaurosis und mehrfachen Voroperationen

Anti-p200/anti-laminin γ1 pemphigoid and BP180 NC16A/4575– positive mucous membrane pemphigoid

Late diagnosis in a patient with disease-related loss of vision and multiple previous surgical interventions

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Zusammenfassung

Eine 87-jährige Patientin stellte sich mit teils erosiven, teils bullösen Haut- und Schleimhautveränderungen, einem Symblepharon der Augenlider beidseits sowie einer Dysphagie vor. In den letzten Jahren wurden mehrfache Exzisionen der von Chirurgen als „Hauttumore“ bezeichneten Hautveränderungen durchgeführt. In Zusammenschau der Histologie sowie der direkten und indirekten Immunfluoreszenz konnte die Diagnose eines Anti-p200/Laminin-γ1-Pemphigoids und BP180 NC16A/4575-positiven Schleimhautpemphigoids mit einem ungewöhnlichen Epitope-spreading-Phänomen gestellt werden. Aufgrund des langen Zeitraums ohne Therapie war die Amaurosis leider nicht mehr reversibel.

Abstract

A 87-year-old woman presented with a three-year history of partially erosive, partially bullous skin and mucosal lesions, symblepharon of both eyelids as well as dysphagia. To date, multiple excisions of the skin lesions, which had been described as “skin tumors” by surgeons, had been performed. The synopsis of histology, direct and indirect immunofluorescence established the diagnosis of anti-p200/anti-laminin γ1 pemphigoid and BP180 NC16A/4575– positive mucous membrane pemphigoid with an unusual “epitope-spreading” phenomenon. Due to the late initiation of therapy, the disease-related loss of vision unfortunately was irreversible.

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Interessenkonflikt

K.M. Kaune, M. Kasperkiewicz, D. Tams, M. Bergmann und M. Zutt geben an, dass kein Interessenkonflikt besteht.

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Authors and Affiliations

  1. Klinik für Dermatologie und Allergologie, Klinikum Bremen-Mitte, St.-Jürgen-Str. 1, 28177, Bremen, Deutschland

    K.M. Kaune, D. Tams & M. Zutt

  2. Klinik für Dermatologie, Allergologie und Venerologie, Universität Lübeck, Lübeck, Deutschland

    M. Kasperkiewicz

  3. Institut für Pathologie und Neuropathologie, Klinikum Bremen-Mitte, Bremen, Deutschland

    M. Bergmann

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  1. K.M. Kaune
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  2. M. Kasperkiewicz
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  4. M. Bergmann
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  5. M. Zutt
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Correspondence to K.M. Kaune.

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Kaune, K., Kasperkiewicz, M., Tams, D. et al. Anti-p200/Laminin-γ1-Pemphigoid in Kombination mit einem BP180 NC16A/4575-positiven Schleimhautpemphigoid. Hautarzt 66, 60–64 (2015). https://doi.org/10.1007/s00105-014-3529-1

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  • DOI: https://doi.org/10.1007/s00105-014-3529-1

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