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Junctional epidermolysis bullosa inversa

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Disease 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.

ORPHA:79405

Classification level: Disorder

Synonym(s):
  • JEB inversa
  • JEB-I

Prevalence: <1 / 1 000 000

Inheritance: Autosomal recessive

Age of onset: Infancy, Neonatal

ICD-10: Q81.8

ICD-11: EC31

OMIM: 226650

UMLS: C2673609

A summary on this disease is available in Français, Español, Deutsch, Italiano, Português, Nederlands Русский
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

General public
Article for general public
Svenska (2024) - Socialstyrelsen
Guidelines
Emergency guidelines
Français (2012.pdf) - Orphanet Urgences
Anesthesia guidelines
English (2020) - Orphananesthesia
Čeština (2020) - Orphananesthesia
Clinical practice guidelines
English (2020) - Br J Dermatol Logo ERN
English (2020) - Br J Dermatol Logo ERN
English (2019.pdf) - Debra International Logo ERN
English (2016) - Br J Dermatol Logo ERN
English (2014) - Orphanet J Rare Dis Logo ERN
English (2019) - Orphanet J Rare Dis Logo ERN
English (2012) - Int J Paediatr Dent Logo ERN
English (2014) - BMC Med Logo ERN
English (2019.pdf) - Debra International Logo ERN
English (2019) - Orphanet J Rare Dis Logo ERN
English (2020) - Orphanet J Rare Dis Logo ERN
English (2017.pdf) - Wounds International
Español (2017.pdf) - Wounds International
Disease review articles
Review article
English (2010) - Orphanet J Rare Dis
Clinical genetics review
English (2018) - GeneReviews
Disability
Disability factsheet
Español (2018.pdf) - Orphanet
The documents contained in this website are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.