scholarly journals Pemphigus vulgaris presenting as esophageal ulceration. Report of an underdiagnosed manifestation

2021 ◽  
Vol 84 (2) ◽  
pp. 365-366
Author(s):  
C Leal ◽  
M Silva ◽  
S Barbeiro ◽  
H Vasconcelos

Pemphigus vulgaris (PV) is a rare autoimmune blistering disorder of the skin and mucous membranes. The true prevalence of esophageal involvement is unknown; esophageal symptoms almost always occur in the context of oral mucosa involvement. We report the case of a 66-year-old man with cutaneous blisters and esophageal symptoms that did not respond to acid suppression therapy. Esophagogastroduodenoscopy showed esophageal ulcers and mucosal desquamation. Biopsies were consistent with the diagnosis of PV. The patient was started on immunosuppressive therapy, achieving remission. This represents a rare case of esophageal involvement of PV without mucosal involvement and draws attention to a rare cause of dysphagia, which can be fatal if left untreated


2018 ◽  
Vol 12 (2) ◽  
pp. 260-265 ◽  
Author(s):  
Angelo Gualberto de Macedo ◽  
Erika Ruback Bertges ◽  
Luiz Carlos Bertges ◽  
Renata Alvim Mendes ◽  
Thais Abranches Bueno Sabino Bertges ◽  
...  

Pemphigus vulgaris (PV) is a chronic autoimmune mucocutaneous disease. In most cases, the initial manifestation occurs in the mouth as multiple ulcerations preceded by blisters that rupture and later spread to other mucous membranes and the skin. Esophageal impairment is rare. We report a case of PV with esophageal involvement in a 53-year-old woman who sought medical care, complaining of diffuse painful lesions in the oral cavity for approximately 1 month, with no improvement with nystatin. Upper digestive endoscopy (UDE) was performed with findings of vesiculobullous lesions in the proximal and middle esophagus, which were biopsied and for which histopathology confirmed PV in the esophageal mucosa. We draw attention to UDE as important for the diagnosis, and it should be indicated in patients with vesiculobullous lesions of the mouth, especially if there are esophageal complaints.



2021 ◽  
Vol 5 (2) ◽  
pp. 170-173
Author(s):  
Stefanie Altmann ◽  
Adam Chahine ◽  
Jarett Casale ◽  
Jessica Forbes ◽  
Sarah Ferrer-Bruker

Pemphigus Vulgaris (PV) is the most common subtype of pemphigus, a rare group of autoimmune bullous diseases affecting the skin and mucous membranes. PV can be further subdivided into mucocutaneous and mucosal dominant types, depending on the extent of cutaneous involvement. Almost all cases of PV have mucosal involvement; however, a rare variant of cutaneous-only PV has been reported in the literature. To our knowledge, only two previous accounts of unilesional scalp PV have been reported. We present an unusual case of cutaneous-only PV involving the scalp.



2016 ◽  
Vol 2016 ◽  
pp. 1-5
Author(s):  
Zeinab Fagheei Aghmiyuni ◽  
Ahmad Khorshidi ◽  
Rezvan Moniri ◽  
Tahereh Soori ◽  
Seyed Gholam Abbas Musavi

Pemphigus vulgaris are autoimmune blistering diseases that may result in significant morbidity and death. Immunosuppressive therapy of pemphigus vulgaris would predispose the patients to infections. The aim of this study was to assess the prevalence ofS. aureusinfection andPVLgene in patients with pemphigus admitted to dermatology clinic.Materials and Methods. This descriptive study was conducted on 196 pemphigus vulgaris patients (119 males, 77 females) admitted to dermatology clinic between 2014 and 2015. In this study, the diagnosis of pemphigus vulgaris was made by histology, immunofluorescence pattern of perilesional skin, and indirect immunofluorescence testing of serum. Data were collected through a questionnaire.Results. 59.1% of pemphigus vulgaris patients hadS. aureusinfection. 49 out of 116 were methicillin-resistant.PVLgene was detected in 25 out of 116S. aureuspositive patients.Conclusion. This is the first report ofS. aureusinfection in pemphigus patients in Iran. More than forty percent of isolates were methicillin-resistantS. aureus.PVLgene carried by methicillin-resistantS. aureuswas high in this study.



2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Jennifer Rose F. Del Castillo ◽  
Muhammad Nadeem Yousaf ◽  
Fizah S. Chaudhary ◽  
Nahar Saleh ◽  
Lawrence Mills

Pemphigus vulgaris (PV) is an autoimmune blistering disorder of the skin and mucosal surfaces characterized by acantholysis (loss of adhesion between epidermal cells). Esophageal involvement of PV is an underdiagnosed entity as routine diagnostic endoscopy is not recommended in asymptomatic patients. Dysphagia and odynophagia are common presenting symptoms; however, upper gastrointestinal bleeding (UGIB) associated with esophageal involvement of PV without a history of mucosal blistering is extremely uncommon. We present a case of esophageal involvement of PV associated with active UGIB that was diagnosed on endoscopic evaluation. This case illustrated the importance of early endoscopy to identify the esophageal involvement of PV especially in patients with preexisting disease who present with gastrointestinal symptoms such as dysphagia, odynophagia, and hematemesis. Early recognition of esophageal involvement of PV and initiation of corticosteroid and/or immunosuppressant therapy may improve the outcome of the disease.



1994 ◽  
Vol 111 (3P1) ◽  
pp. 236-242 ◽  
Author(s):  
Michael G. Stewart ◽  
Newton O. Duncan ◽  
Daniel J. Franklin ◽  
Ellen M. Friedman ◽  
Marcelle Sulek

Erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis are related disorders of skin and mucous membranes, which are typically associated with antecedent medication use or infection. We review 108 cases of erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis from Texas Children's Hospital, Houston, Texas, from 1981 to 1991, and illustrate the characteristic skin and mucosal lesions. In addition, we describe in detail two unusual cases requiring intensive airway management. Head and neck manifestations were present in 4 of 79 patients (5%) with erythema multiforme and 26 of 28 patients (93%) with Stevens-Johnson syndrome. In Stevens-Johnson syndrome, mucosal involvement of the lip (93%), conjunctiva (82%), oral cavity (79%), and nose (36%) were most common. Antecedent medication use was identified in 59% of erythema multiforme patients and 68% of Stevens-Johnson syndrome patients. We note a striking increase in the number of cases in our series caused by cephalosporins. Fifty percent of Stevens-Johnson syndrome patients required supplemental hydration or alimentation because of the severity of the oral cavity involvement. The head and neck mucosal manifestations largely respond to local care, and the routine use of prophylactic antibiotics or systemic steroids is not recommended.



2012 ◽  
Vol 16 (2) ◽  
pp. 128-130 ◽  
Author(s):  
Ashley O'toole ◽  
Maureen O'malley

Background: Pemphigus vulgaris is an autoimmune blistering disease of the skin and mucous membranes. Sweet syndrome is an uncommon reactive neutrophilic dermatosis. Objective: This case report describes the rare presentation of a 58-year-old female patient with newly diagnosed pemphigus vulgaris who later presented with erythematous plaques and leukocytosis highly suggestive of Sweet syndrome. Methods: Review of the literature using the PubMed and Medline databases. Results: The clinical presentation of new-onset Sweet syndrome in a patient with pemphigus has been reported in the literature only on one other occasion. Conclusion: The observation of an association between Sweet syndrome and autoimmune diseases, such as pemphigus vulgaris, may ultimately lead to better understanding of the pathophysiology of this disease.



Dysphagia ◽  
2019 ◽  
Vol 35 (3) ◽  
pp. 503-508
Author(s):  
Katharine A. Ozeki ◽  
Thomas A. Zikos ◽  
John O. Clarke ◽  
Irene Sonu


2018 ◽  
Vol 113 (Supplement) ◽  
pp. S177
Author(s):  
Katharine Ozeki ◽  
Thomas A. Zikos ◽  
John Clarke ◽  
Irene Sonu


2020 ◽  
Vol 83 (6) ◽  
pp. 520-521
Author(s):  
Miki Hamanaka ◽  
Hiromi Kanto ◽  
Hitomi Mikai ◽  
Hiroko Tanaka ◽  
Takashi Ito ◽  
...  


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