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Case Report
Esophageal Involvement of Pemphigus Vulgaris Associated with Upper Gastrointestinal Bleeding
Sooyun Chang, Soo Jung Park, Sun Wook Kim, Moo-Nyun Jin, Jung-Hee Lee, Hyun Ju Kim, Sung Pil Hong, Tae Il Kim
Clin Endosc 2014;47(5):452-454.   Published online September 30, 2014
DOI: https://doi.org/10.5946/ce.2014.47.5.452
AbstractAbstract PDFPubReaderePub

Esophageal involvement of pemphigus vulgaris is rare, and when present, the most common presenting symptoms reported in the medical literature are odynophagia and dysphagia. Here, we present two cases of pemphigus vulgaris presenting with upper gastrointestinal hemorrhage because of esophageal involvement of the disease. In case 1, a 41-year-old female patient with a prior diagnosis of pemphigus vulgaris presented with hematemesis. Esophagogastroduodenoscopy showed diffuse mucosal exfoliation and oozing bleeding of the oropharynx and esophagus. The patient recovered after the administration of high-dose corticosteroids and immunosuppressants. In case 2, a 30-year-old female patient with known pemphigus vulgaris also presented with hematemesis, showing similar endoscopic findings to the first case. She also responded to the same treatment. Esophageal involvement of pemphigus vulgaris responds to high-dose corticosteroids and immunosuppressants. Thus, in patients with pemphigus vulgaris with signs or symptoms of upper gastrointestinal bleeding, an early endoscopy for the evaluation of esophageal involvement is beneficial.

Citations

Citations to this article as recorded by  
  • Hematemesis in pemphigus vulgaris: A challenging case of isolated esophageal flare in patient in remission
    Maninder Kaur, Nagbhushan Hedge, Ashish Agarwal, Vikarn Vishwajeet, Suman Patra, Saurabh Singh
    JDDG: Journal der Deutschen Dermatologischen Gesellschaft.2023; 21(12): 1551.     CrossRef
  • Hämatemesis bei Pemphigus vulgaris: Ein herausfordernder Fall eines isolierten Ösophagusschubs bei einer Patientin in Remission
    Maninder Kaur, Nagbhushan Hedge, Ashish Agarwal, Vikarn Vishwajeet, Suman Patra, Saurabh Singh
    JDDG: Journal der Deutschen Dermatologischen Gesellschaft.2023; 21(12): 1551.     CrossRef
  • A Case of Exfoliative Esophagitis with Pemphigus Vulgaris
    Wei-Feng Huang, Wei Liu
    Indian Journal of Surgery.2022; 84(3): 563.     CrossRef
  • Pemphigus for the Inpatient Dermatologist
    Connor Cole, Kyle Amber
    Current Dermatology Reports.2022; 11(4): 221.     CrossRef
  • Novel and rare forms of oesophagitis
    Victoria Malone, Kieran Sheahan
    Histopathology.2021; 78(1): 4.     CrossRef
  • Esophageal Pemphigus Vulgaris: A Rare Etiology of Upper Gastrointestinal Hemorrhage
    Jennifer Rose F. Del Castillo, Muhammad Nadeem Yousaf, Fizah S. Chaudhary, Nahar Saleh, Lawrence Mills, Yoshiro Kawahara
    Case Reports in Gastrointestinal Medicine.2021; 2021: 1.     CrossRef
  • Pemphigus Vulgaris and Bullous Pemphigoid of the Upper Aerodigestive Tract: A Review Article and Novel Approaches to Management
    Mohammed Hassan Hussain, Faiz Tanweer, Georgios Sakagiannis, Manish Mair, Sara Mahmood, Sithamparappillai Ashokkumar
    ORL.2021; 83(6): 395.     CrossRef
  • Gastrointestinal involvement of primary skin diseases
    C.‐Y. Lu, M.‐S. Hsieh, K.‐C. Wei, M. Ezmerli, C.‐H. Kuo, W. Chen
    Journal of the European Academy of Dermatology and Venereology.2020; 34(12): 2766.     CrossRef
  • Oesophageal pemphigoid: a rare cause of dysphagia
    Michael McFarlane, Ayesha Azam, David Snead, Ben Disney
    Clinical Journal of Gastroenterology.2019; 12(1): 25.     CrossRef
  • Esophagitis unrelated to reflux disease: current status and emerging diagnostic challenges
    Melanie E. Johncilla, Amitabh Srivastava
    Virchows Archiv.2018; 472(1): 29.     CrossRef
  • Pemphigus Vulgaris in the Mouth and Esophageal Mucosa
    Angelo Gualberto de Macedo, Erika Ruback Bertges, Luiz Carlos Bertges, Renata Alvim Mendes, Thais Abranches Bueno Sabino Bertges, Klaus Ruback Bertges, Fernando Monteiro Aarestrup
    Case Reports in Gastroenterology.2018; 12(2): 260.     CrossRef
  • Ulcerative colitis associated with pemphigus: a population-based large-scale study
    Khalaf Kridin, Shira Zelber-Sagi, Doron Comaneshter, Arnon D. Cohen
    Scandinavian Journal of Gastroenterology.2017; 52(12): 1360.     CrossRef
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A Case of Esophageal Involvement in Pemphigus Vulgaris
Young Chul Kim, M.D., Woo Chul Chung, M.D., Seok Jin Kang, M.D.*, Chang Kyun Hong, M.D., Kang Moon Lee, M.D., Jin Mo Yang, M.D., Jae Kwang Kim, M.D., Sok Won Han, M.D., Kyu Yong Choi, M.D. and In-Sik Chung, M.D.
Korean J Gastrointest Endosc 2007;35(3):159-164.   Published online September 30, 2007
AbstractAbstract PDF
Pemphigus vulgaris is a rare chronic disorder that is characterized by the development of bullae on the skin and mucous membrane due to an acantholysis mediated by circulating autoantibodies. It begins as painful, non- healing ulcerations in the mouth. After a period of weeks to months, the condition progresses to the skin. Occasionally, skin lesions may develop as the initial manifestation of this disease. However, esophageal involvement of pemphigus vulgaris is rare. An 84-year-old man was referred due to chest pain, dysphagia, oral ulcerations and facial bullae. The histopathological findings showed a suprabasal blister and acantholysis in the epithelium of the facial skin. An endoscopic examination revealed exfoliated erosions, ulcerations and hemorrhagic bullae on the oral and esophageal mucosa. The patient was started on high dose of corticosteroids and azathioprine, but there was no clinical improvement. The use of cyclophosphamide led to the remission of the lesions. (Korean J Gastrointest Endosc 2007;35:159-164)
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