A rare case of Cicatricial Pemphigoid (Mucus Membrane Pemphigoid)

Document Type : Original Article

Authors

1 Department of Otorhinolaryngology-Head and Neck Surgery, King Abdulaziz Medical City, Ministry of National Guard-Health Affairs, Riyadh, Saudi Arabia

2 Department of Otorhinolaryngology- Head and Neck Surgery, College of Medicine, Imam Mohammad ibn Saud Islamic University, Riyadh, Saudi Arabia

3 Ministry of Health, Riyadh, Saudi Arabia

4 King Fahad Medical City, Riyadh, Saudi Arabia

5 Ad Diriyah Hospital, Riyadh, Saudi Arabia

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