Pemphigus and Plummer-Vinson Syndrome: An Uncommon Association

Abstract

Aims: The reported association of Plummer-Vinson syndrome and some autoimmune diseases, including pemphigus -association described for the first time-, raises the hypothesis of the autoimmune origin of this syndrome. On the other hand, this unexpected association represents a therapeutic challenge given the risk of the progression of pemphigus lesions after an esophageal dilatation session, hence the originality and interest of our case. Case Report: A 60-year-old patient was admitted to our department for pemphigus foliaceus and treated with oral corticosteroids. The workup revealed an iron deficiency anemia. A dysphagia to solids evolving for 4 years led to an esophageal endoscopy which revealed the presence of esophageal ring. The diagnosis of Plummer-Vinson syndrome was retained but the dilatation could only be done after stabilizing the pemphigus to avoid a detachment of the esophageal mucosa by Koebner phenomenon. Discussion: Plummer-Vinson syndrome is a rare condition defined by the presence of dysphagia, iron deficiency anemia and esophageal ring. Its etiopathogeny is poorly understood. It can be associated with some autoimmune pathologies suggesting an autoimmune origin. To date, no association with pemphigus has been described in the literature. Conclusion: The autoimmune origin of Plummer Vinson syndrome is to be discussed in regard to the described associations with autoimmune diseases which would not be random.