Study of oral, ear, nose and throat involvement in pemphigus vulgaris by endoscopic examination.
BACKGROUND
Pemphigus vulgaris (PV) is an autoimmune blistering skin disorder characterized by the presence of suprabasal acantholysis and autoantibodies against desmoglein 3. There are two different clinical forms: mucocutaneous (MCPV) and mucosal (MPV). However, little is known about PV lesions in oral, ear, nose and throat (OENT) areas produced by the very dynamic of the anatomical structures involved in the functions of the aerodigestive tract.


OBJECTIVE
To investigate the pattern of OENT manifestations in PV, and their relationship with physiological traumatic mechanisms in stratified squamous epithelial structures.


METHODS
A prospective analysis of 40 patients diagnosed with MCPV (22 patients) or MPV (18 patients) was carried out at the University Clinic of Navarra. OENT manifestations were evaluated in all patients endoscopically. OENT involvement was divided into anatomical areas.


RESULTS
The most frequent symptom was pain, mainly on oral mucosa (87·5%). Buccal mucosa (90%), posterior wall of pharynx (67·5%), upper edge of epiglottis (85%) and nasal vestibule (70%) were the areas most frequently affected in the OENT mucosa. These localizations were related to physiological traumatic mechanisms in polystratified squamous epithelial structures.


CONCLUSIONS
OENT endoscopy should be included in the examination of all patients with PV. Knowledge of the most frequent localizations of active lesions on OENT mucosa in PV will help us to interpret more efficiently the findings from OENT endoscopy. Also, information related to traumatic physiological mechanisms on OENT areas must be offered to patients in order to avoid the appearance of new active PV lesions.
Department of Otorhinolaryngology, Unit of Autoimmune Blistering Skin Diseases, University Clinic of Navarra, School of Medicine, PO Box 4209, Pamplona 31080, Navarra, Spain.
S
S
Fernández
A
A
España
M
M
Navedo
L
L
Barona
D001379:Azathioprine / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D004427:Ear Diseases / Q000175:diagnosis* / Q000188:drug therapy; D004724:Endoscopy / Q000379:methods*; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008875:Middle Aged; D009059:Mouth Diseases / Q000175:diagnosis* / Q000188:drug therapy; D009173:Mycophenolic Acid / Q000031:analogs & derivatives / Q000627:therapeutic use; D009668:Nose Diseases / Q000175:diagnosis* / Q000188:drug therapy; D010392:Pemphigus / Q000175:diagnosis* / Q000188:drug therapy; D010608:Pharyngeal Diseases / Q000175:diagnosis* / Q000188:drug therapy; D011446:Prospective Studies; D012720:Severity of Illness Index
D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D009173:Mycophenolic Acid; D001379:Azathioprine