Cicatricial pemphigoid with severe gingival and laryngeal involvement in an 18-year-old female.
Cicatricial pemphigoid (CP), also known as mucous membrane pemphigoid, is an autoimmune vesiculobullous disease occurring mostly in elderly people and seldom occurring in individuals under the age of 20 years. It predominantly affects the mucosal surfaces, primarily the oral and conjunctival mucosa. Uncommonly, the upper aerodigestive tract is involved, which can lead to life-threatening complications. We present the case of an 18-year-old girl with desquamative gingivitis and severe laryngeal webbing and stenosis, caused by cicatricial pemphigoid. Airway management necessitated a tracheostomy and additional surgical procedure. The clinical, diagnostic, and therapeutic features of this disease are discussed, focusing on the oral and unique laryngeal manifestations.
Ojha
Junu
J
Department of Oral and Maxillofacial Surgery and Dental Diagnostic Sciences, University of Florida, College of Dentistry, Gainesville, FL 32610, USA. jojha@dental.ufl.edu
Bhattacharyya
Indraneel
I
Stewart
Carol
C
Katz
Joseph
J
D000293:Adolescent; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D004417:Dyspnea / Q000209:etiology; D004844:Epistaxis / Q000209:etiology; D005260:Female; D005882:Gingival Diseases / Q000209:etiology*; D006801:Humans; D007818:Laryngeal Diseases / Q000209:etiology*; D010390:Pemphigoid, Benign Mucous Membrane / Q000150:complications* / Q000188:drug therapy; D011241:Prednisone / Q000627:therapeutic use; D012135:Respiratory Sounds / Q000209:etiology; D014139:Tracheostomy; D014832:Voice Disorders / Q000209:etiology
D000893:Anti-Inflammatory Agents; D011241:Prednisone