A rare case of Cicatricial Pemphigoid (Mucus Membrane Pemphigoid)

Document Type : Case Report

Authors

1 ENT ,NGHA

2 ENT NGHA

3 NGHA DENT

4 NGHA

Abstract

Cicatricial pemphigoid (CP), refers to a rare chronic autoimmune blister disease that predominantly affect mucosal membranes. We report a case of 60-year-old male with the diagnosis of cicatricial pemphigoid (CP). The pathology involved oral mucosa, Pharynx and esophagus. Diagnosis was confirmed by histopathology, and the patient responded well to oral steroids. It is characterized by progressive bullous skin and mucous membrane lesions that tend toward scarring and involution.CP is commonly seen in elderly age group, but it has been reported in children in much lower frequency. It differs from bullous pemphigoid in its prolonged course and significant scarring.The oral mucosa is the most frequently affected area (85 to 90% of patients), followed by the ocular mucosa (66%).The upper airway and digestive tract are also affected in a considerable number of patients. Nasal involvement has been reported to occur in (15 to 23%) of patients, and laryngeal involvement has been seen in . Clinical diagnosis is made on the basis of clinical presentation established by histology,and confirmed by immunpathological studies.Approximately, 50% of patients with oral pemphigoid have been reported progression to involve extra-oral sites, such as eyes, larynx, pharynx, or oesophagus.

Keywords



Articles in Press, Accepted Manuscript
Available Online from 29 August 2023
  • Receive Date: 08 May 2023
  • Revise Date: 29 April 2024
  • Accept Date: 15 May 2023