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Autosomal recessive generalized epidermolysis bullosa simplex

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Disease definition

A rare, inherited, epidermolysis bullosa simplex characterized by neonatal onset of generalized or, less frequently, localized acral blistering. Milia are rare but atrophic scarring and dystrophic nails usually occur, along with focal keratoderma (palms and soles). Severe generalized blistering may cause perinatal death or persist during the entire life. Extracutaneous involvement is common, including anemia, growth retardation, oral cavity abnormalities (blisters and erosions, and caries) and constipation.

ORPHA:89838

Classification level: Disorder

Synonym(s):
  • Autosomal recessive generalized EBS

Prevalence: <1 / 1 000 000

Inheritance: Autosomal recessive

Age of onset: Infancy, Neonatal

ICD-10: Q81.0

ICD-11: EC30

OMIM: 619599 601001

UMLS: C5680184

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

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

General public
Article for general public
English (2022.pdf) - Bambino Gesù Children’s Hospital Logo ERN
Svenska (2024) - Socialstyrelsen
Italiano (2022.pdf) - Bambino Gesù Children’s Hospital Logo ERN
Guidelines
Emergency guidelines
Français (2012.pdf) - Orphanet Urgences
Clinical practice guidelines
English (2019.pdf) - Debra International Logo ERN
English (2012) - Int J Paediatr Dent Logo ERN
English (2014) - Orphanet J Rare Dis Logo ERN
English (2014) - BMC Med Logo ERN
English (2016) - Br J Dermatol Logo ERN
English (2019) - Orphanet J Rare Dis Logo ERN
English (2019) - Orphanet J Rare Dis Logo ERN
English (2020) - Br J Dermatol Logo ERN
English (2020) - Br J Dermatol Logo ERN
English (2019.pdf) - Debra International 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 (2025) - Orphanet J Rare Dis
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.