A Case of Kindler Syndrome presenting with Dysphagia

Abstract

Kindler syndrome is a rare hereditary disorder that predominantly involves the skin and mucous membrane. Acral skin blistering, progressive photosensivity, skin atrophy and poikiloderma that begin from infancy and childhood are considered to be characteristic manifestations. Urethral, anal, esophageal, mouth and laryngeal mucosa may be involved in this syndrome, thus periodontitis and gingival involvement, anal, esophageal or urethral strictures may be present in this disease.
Although gastrointestinal tract involvement in patients with Kindler syndrome is possible, it is rare in the literature. We report the case of a 34-year-old male with Kindler syndrome who had referred with dysphagia. Upper gastrointestinal endoscopy revealed an esophageal web in the upper third of the esophagus. Endoscopic dilatation of the esophageal web was performed by using a mechanical dilating bougie.Two months after esophageal dilatation we found significant improvement in the patient's dysphagia according to the validated Mayo Dysphagia Questionnaire (MDQ).