Esophageal Bullous Pemphigoid
  • Catarina Castelo Branco
    Department of Internal Medicine, Centro Hospitalar Universitário do Porto, Porto, Portugal
  • Tomás Fonseca
    Department of Internal Medicine, Centro Hospitalar Universitário do Porto, Porto, Portugal
  • Ricardo Marcos-Pinto
    Department of Gastroenterology, Centro Hospitalar Universitário do Porto, Porto, Portugal

Keywords

Bullous pemphigoid, esophagus, dementia

Abstract

Bullous pemphigoid is a rare autoimmune dermatologic disease that usually occurs in the elderly. Mucous membrane lesions occur in about 10–35% of patients and are almost always limited to the oral mucous membrane. Esophageal involvement is very rare (4% of cases) and usually presents with chest pain, dysphagia, and odynophagia, though patients are frequently asymptomatic. We report the case of newly diagnosed bullous pemphigoid in a 76-year-old man with a past medical history of dementia. He presented with cutaneous manifestations but also severe gastrointestinal bleeding due to extensive esophageal involvement. Although bullous pemphigoid is mainly a skin disease, mucous membrane lesions should not be overlooked as they are associated with an even poorer outcome. A high index of suspicion for esophageal involvement is needed as its presentation can be fatal, as with our patient.

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References

  • Miyamoto D, Santi CG, Aoki V, Maruta CW. Bullous pemphigoid. An Bras Dermatol 2019;94(2):133–146.
  • Schmidt E, Zillikens D. Pemphigoid diseases. Lancet 2013;381(9863):320–332.
  • Lai YC, Yew YW, Lambert WC. Bullous pemphigoid and its association with neurological diseases: a systematic review and meta-analysis. J Eur Acad Dermatol Venereol 2016;30(12):2007–2015.
  • Gaspar R, Moutinho-Ribeiro P, Macedo G. Bullous pemphigoid: extensive esophageal involvement. Gastrointest Endosc 2017;86(2):400–402.
  • Sárdy M, Kostaki D, Varga R, Peris K, Ruzicka T. Comparative study of direct and indirect immunofluorescence and of bullous pemphigoid 180 and 230 enzyme-linked immunosorbent assays for diagnosis of bullous pemphigoid. J Am Acad Dermatol 2013;69(5):748–753.
  • Venning VA, Taghipour K, Mohd Mustapa MF, Highet AS, Kirtschig G. British Association of Dermatologists’ guidelines for the management of bullous pemphigoid 2012. Br J Dermatol 2012;167(6):1200–1214.
  • Amagai M, Ikeda S, Hashimoto T, Mizuashi M, Fujisawa A, Ihn H, et al. A randomized double-blind trial of intravenous immunoglobulin for bullous pemphigoid. J Dermatol Sci 2017; 85(2):77–84.
  • Holtsche MM, Goletz S, van Beek N, Zillikens D, Benoit S, Harman K, et al. Prospective study in bullous pemphigoid: association of high serum anti-BP180 IgG levels with increased mortality and reduced Karnofsky score. Br J Dermatol 2018;179(4):918–924.
  • Bystryn JC, Rudolph JL. Why is the mortality of bullous pemphigoid greater in Europe than in the US? J Invest Dermatol, 2005;124(3):xx–xxi.

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    Published: 2022-02-01
    Issue: 2022: Vol 9 No 2 (view)

    How to cite:
    1.
    Castelo Branco C, Fonseca T, Marcos-Pinto R. Esophageal Bullous Pemphigoid. EJCRIM 2022;9 doi:10.12890/2022_003160.

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