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Esophageal involvement in cicatricial pemphigoid
A Stallmach1, S Weg-Remers, C Moser
1Department of Internal Medicine II, University of Saarland, Homburg, Germany. inasta@med-rz.uni.sb.de
Endoscopy
|November 24, 1998
Summary
A 17-year-old male with dysphagia and cachexia was misdiagnosed with psychiatric issues. Cicatricial pemphigoid, an autoimmune blistering disease, was eventually diagnosed after esophageal biopsies, enabling proper treatment.
Area of Science:
- Gastroenterology
- Immunodermatology
- Internal Medicine
Background:
- Esophageal stenosis can present with severe symptoms like dysphagia and cachexia.
- Misdiagnosis can delay appropriate treatment, leading to prolonged patient suffering.
- Autoimmune conditions may manifest with gastrointestinal symptoms.
Observation:
- A 17-year-old male presented with dysphagia, cachexia, and physical underdevelopment.
- Despite a known esophageal stenosis, psychiatric treatment was administered for several months without clinical improvement.
- Repeated biopsies of the esophageal stenosis were crucial for diagnosis.
Findings:
- The patient was diagnosed with cicatricial pemphigoid, an autoimmune blistering disease affecting mucous membranes.
- Cicatricial pemphigoid was the underlying cause of the esophageal stenosis and associated symptoms.
- Initiation of adequate therapy for cicatricial pemphigoid was possible after diagnosis.
Implications:
- Highlights the importance of considering rare autoimmune diseases in patients with unexplained gastrointestinal symptoms.
- Emphasizes the need for thorough diagnostic workups, including biopsies, for persistent esophageal stenosis.
- Successful diagnosis and treatment of cicatricial pemphigoid can significantly improve patient outcomes and quality of life.