Junctional epidermolysis bullosa inversa

Orpha code: 79405OMIM code: 226650

Definition

A rare intermediate form of junctional epidermolysis bullosa characterized by congenital blistering and erosions confined to intertriginous skin sites, the esophagus, groin, and perineum. Blistering is usually severe and lesions may heal with atrophic scarring and milia formation. Extracutaneous manifestations include nail dystrophy, enamel hypoplasia and dental caries, oral, esophageal and vaginal blisters and erosions.

Disease data
Classification

Disease

Synonyms
JEB inversa
JEB-I
JEB inversa
JEB-I
ORPHA code
79405
OMIM code
226650
ICD10 code
Q81.8
ICD11 code
-

No additional description.

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