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Late-onset junctional epidermolysis bullosa

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Disease definition

A form of junctional epidermolysis bullosa characterized by onset in childhood or young adulthood of blistering that first occurs around nails, accompanied by nail dystrophy and shedding, and then affects the hands and feet and, to a lesser extent, the elbows, and knees. Lesions heal with atrophic scarring. Other manifestations include disappearance of dermatoglyphs and palmoplantar hyperhidrosis. Extracutaneous involvement is restricted to soft tissue abnormalities of the oral cavity and enamel defects with development of caries.

ORPHA:79406

Classification level: Disorder

Synonym(s):
  • JEB-lo
  • Epidermolysis bullosa progressiva
  • Late-onset JEB

Prevalence: <1 / 1 000 000

Inheritance: Autosomal recessive

Age of onset: Adolescent, Adult, Childhood

ICD-10: Q81.8

ICD-11: EC31

OMIM: 619787

UMLS: C4304724

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.