Junctional epidermolysis bullosa

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Junctional epidermolysis bullosa

ORPHA:305Clinical group

Also called Epidermolysis bullosa atrophicans · JEB

What it is

A group of inherited epidermolysis bullosa characterized by involvement of the skin and mucous membranes, and is defined by the formation of blistering lesions between the epidermis and the dermis at the lamina lucida level of the cutaneous basement membrane zone and by healing of lesions with atrophy and/or exuberant granulation tissue formation.

Key facts

Prevalence
<1 / 1 000 000 (Sweden)
Age of onset
Adolescent, Antenatal, Infancy, Neonatal
Inheritance
Autosomal recessive
Classified as
Clinical group

Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.

Genes reported in subtypes

COL17A1ITGA3ITGA6ITGB4LAMA3LAMB3LAMC2

Orphanet records these genes on 7 more specific entries under this disorder, not on this entry itself:

A well-studied disease is usually split into subtypes, and the gene is curated on whichever one the evidence belongs to. Open a subtype to see which gene goes with it.

Cross-references

GARD 2152MESH D016109MONDO 0017612MONDO 17612UMLS C0079301

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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