[Digestive involvement in dystrophic bullous epidermolysis. Presentation of 2 cases and review of the literature].
Congenital epidermolysis bullosa is an inherited disorder characterized by an anomaly of joint structures between epidermis and dermis. This anomaly involves different malpighian epithelium and particularly in the digestive chorioepithelial junction. Digestive and oropharyngeal manifestations include bullae, erosions and ulcerations that heal by fibrosis, leading to retraction and stenosis. We report two cases of esophageal stenosis, the first one in a young patient with a congenital epidermolysis bullosa of Hallopeau-Siemens and the second one, in a young woman with a Pasini variant. In the first case, the esophageal stenosis was treated by careful dilation. In the second case, the endoscopy induced hemorragic bullae and septicemia, which were treated with antibiotics and total parenteral nutrition.
Cocciale
M P
MP
Service de Dermatologie, CHR, Metz-Thionville.
Gilbert
C
C
Gogolewski
S
S
Courrier
A
A
Perarnau
J M
JM
Truchetet
F
F
Cuny
J F
JF
D000293:Adolescent; D004066:Digestive System Diseases / Q000209:etiology* / Q000628:therapy; D016108:Epidermolysis Bullosa Dystrophica / Q000150:complications* / Q000235:genetics / Q000628:therapy; D005260:Female; D006801:Humans; D008297:Male