Pemphigus Vegetans Involving the Esophagus

Abstract

A 44‐year‐old man with pemphigus vegetans had severe odynophagia. He received an endoscopic examination for esophageal involvement. Many white plaque‐like lesions with an erythematous base were seen on the esophageal mucosa. Biopsy from the mucosal epithelial layer showed rounded epidermal cells with large nuclei and numerous inflammatory cells including eosinophils.

Development of oesophageal involvement in a subject with pemphigus vulgaris: a case report and review of the literature

“…Since 1970 only about 58 cases of oesophageal pemphigus have been reported, 2–31 although in 1892 Rosenberg 32 had already coined the phrase ‘oesophagitis dissecans superficialis’ in reference to healthy subjects with oesophageal sloughing and in 1935 Patterson 33 first documented oesophageal involvement in pemphigus. Commonly, the disease involves blisters and superficial erosions and/or red erythematous longitudinal lines along the entire organ 16 .…”

Genetic dissection of eosinophilic esophagitis provides insight into disease pathogenesis and treatment strategies

“…Pemphigus foliaceus and pemphigus vulgaris are autoimmune diseases in which autoantibodies targeting DSG1 decrease cellular adhesion, resulting in epidermal blistering 42 . Notably, epithelial microabcesses exhibiting pronounced eosinophilic inflammation that can be associated with pemphigoid disorders have also been demonstrated within the esophagus, such as in pemphigus vegetans 43 . Furthermore, multiple heterozygous mutations in the extracellular domain coding region of DSG1 have been linked with striate palmoplantar keratoderma (SPPK), a disease characterized by epidermal thickening on the palms and soles 44 .…”

What’s new in the pathology, pathophysiology and management of benign esophageal disorders?

“…Other unusual causes of dysphagia are reported, including various benign tumors, 388 –393 pemphigus, 394 pseudodiverticulosis, 395 chronic granulomatous disease, 396 subepithelial fibrosis, 397 , 398 after external compression by the spine and aorta in untreated scoliosis, 399 or by the aorta alone after an arterial switch operation 400 . Bak et al 401 report a rare case of extensive spontaneous intramural dissection of the esophagus successfully treated endoscopically.…”