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Pemphigus vulgaire de l’œsophage

Pemphigus vulgaris of the oesophagus

  • Published:
Acta Endoscopica

Résumé

Le pemphigus vulgaire est une dermatose bulleuse envahissant la peau et certaines muqueuses (oropharynx, muqueuse nasale, conjonctivites, muqueuse anale et vaginale).

Cette affection est caractérisée par un dépôt d’immunoglobulines au niveau de la paroi des cellules épithéliales provoquant une acantholyse. Nous rapportons un cas d’atteinte œsophagienne inaugurale particulièrement rare, l’envahissement de l’œsophage n’ayant été décrit que dans 19 cas dans la littérature internationale.

Les plaintes habituelles sont la dysphagie et l’odynophagie. Les découvertes endoscopiques sont variables et peuvent être confondues avec une candidose ou des lésions herpétiques. Le diagnostic repose sur la mise en évidence de l’acantholyse à l’examen anatomo-pathologique. Ce diagnostic peut être confirmé par examen immunopathologique direct ou indirect.

Le traitement par corticostéroïdes doit être instauré rapidement afin d’éviter les complications éventuelles (sténose œsophagienne, dénutrition…). Parfois le recours aux immunopresseurs est nécessaire.

Summary

Pemphigus vulgaris is a bullous dermatosis which can invade the skin and certain mucosa (oropharynx, nasal mucosa, conjonctiva, anal and vaginal mucosa).

This disease is characterized by a deposit of immunoglobulins in the membrane of epithelial cells thus causing acantholysis. We report a case of an inaugural affection of the œsophagus which is particularly rare since barely 19 cases of involvement of the œsophagus have been described in the international literature.

The usual complaints are dysphagia and odynophagia. The endoscopic findings vary and can be mistaken for a candidosis or herpetic lesions. The diagnosis depends on the presence of acantholysis on the anatomopathological examination. The diagnosis can be further confirmed by direct or indirect immunopathological examination.

The treatment using corticosteroids should be initiated as soon as possible to avoid possible complications (œsophageal stenosis, denutrition…). Sometimes the use of immunosuppressory may be necessary.

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Authors and Affiliations

  1. CHU Liège, Service d’Anatomie Pathologique, Belgium

    V. Fridman

  2. CHU Liège, Service de Dermatologie, Belgium

    G. Pierard

  3. CHR Citadelle, boulevard du 12e de Ligne, 1, 4000, Liège, Belgique

    R. Delrez, M. Lebas & J. Deflandre

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  1. R. Delrez
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  2. M. Lebas
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  4. G. Pierard
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Delrez, R., Lebas, M., Fridman, V. et al. Pemphigus vulgaire de l’œsophage. Acta Endosc 23, 379–384 (1993). https://doi.org/10.1007/BF02969561

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