[Acute pseudo-membranous laryngitis in epidermolysis bullosa acquisita].
Epidermolysis bullosa acquisita is a rare entity belonging to the auto-immune cutaneous blistering disorders of the dermo-epidermal junction. Clinical manifestations are generally cutaneous including the development of sub-epidermal blisters. Mucosal manifestations should be systematically looking for, but laryngeal involvement remains uncommon. We report an 81-year-old woman who presented with dysphagia, dyspnea and dysphonia as the presenting features of laryngeal involvement of an epidermolysis bullosa acquisita. This is the tenth reported case in the literature. We describe our diagnostic approach and the therapeutic management, comparing them with the literature.
Lecomte
H
H
Service d'oto-rhinolaryngologie et de chirurgie cervicofaciale, hôpital Général, CHU de Dijon, 3, rue du Faubourg-Raines, 21000 Dijon, France. lnlecomte@gmail.com
Folia
M
M
Muller
G
G
Gilbert
D
D
Tron
F
F
Besancenot
J-F
JF
Romanet
P
P
D000208:Acute Disease; D000369:Aged, 80 and over; D000890:Anti-Infective Agents / Q000627:therapeutic use; D003622:Dapsone / Q000627:therapeutic use; D003680:Deglutition Disorders / Q000209:etiology; D004359:Drug Therapy, Combination; D055154:Dysphonia / Q000209:etiology; D004417:Dyspnea / Q000209:etiology; D016107:Epidermolysis Bullosa Acquisita / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology*; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007827:Laryngitis / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology*; D011241:Prednisone / Q000627:therapeutic use; D016896:Treatment Outcome
D000890:Anti-Infective Agents; D005938:Glucocorticoids; D003622:Dapsone; D011241:Prednisone