Pemphigus vulgaris with exclusive affectation in the esophagus: a case report

Abstract

Background: Pemphigus vulgaris comprises a group of heterogeneous blistering autoimmune diseases of the skin and mucosa. Esophageal involvement within pemphigus vulgaris is rare with an uncertain prevalence that requires a detailed diagnostic and a therapeutic approach.

Clinical case: 37-year-old female, with a history of treatment with Cox-2 inhibitors due to herniated disc. She is sent to the Gastroenterology Service for weight loss of approximately 5 kilos in a month, with the presence of dysphagia, odynophagia and retrosternal pain with poor tolerance to the oral route. Endoscopy was performed, which reported esophagitis dissecans superficialis (EDS), erythematous gastropathy of the antrum and normal duodenum. Findings were correlated with the diagnosis of pemphigus vulgaris with exclusive involvement of the esophagus. The evaluation did not identify lesions on the skin, oral cavity or other mucous membranes. A new endoscopy was performed as a control and it was found immunofluorescence of the esophageal biopsy reactive to IgG 2. Initial management was given with glucocorticoids, anti-inflammatories and immunosuppressants.

Conclusions: The importance of the study of pemphigus lies not only in the high associated morbidity and mortality, but also in its intrinsic rarity and the complexity of its detection, given that patients usually take several months to have an accurate diagnosis and even more time to achieve therapeutic goals. It is a priority the dissemination of the study of pemphigus among health professionals involved in its detection.