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Late-onset localized junctional epidermolysis bullosa-intellectual disability syndrome

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Disease definition

A rare junctional epidermolysis bullosa subtype characterized by late-onset blistering surrounded by erythema and localized on the anterior aspect of the lower legs, associated with dystrophic toenails, tooth enamel defects and mild to severe intellectual disability. Lens subluxation and mild facial dysmorphism (with short midface, prognatism and thin upper lip vermilion) are additional reported features. There have been no further descriptions in the literature since 1992.

ORPHA:231556

Classification level: Disorder

Synonym(s):
  • Late-onset localized JEB-intellectual disability syndrome

Source: PubMed ID 1642260

Prevalence: <1 / 1 000 000

Inheritance: Unknown

Age of onset: Childhood

ICD-10: Q81.8

OMIM: 226440

UMLS: C4751165

GARD: 299

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

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

General public
Article for general public
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
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