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epidermolysis bullosa simplex (EBS)

Pathology: - blistering occurs in epidermis - generally milder than EB junctional or EB dystrophica which affect deeper layers Genetics: - various forms of EBS: - Koebner type EBS (EBS2) - Weber-Cockayne type EBS - Dowling-Meara type EBS associated with mutations in keratin 5 or keratin 14 genes

Specific

epidermolysis bullosa simplex superficialis (EBSS); epidermolysis bullosa dystrophica with subcorneal cleavage (EBDSC) epidermolysis bullosa simplex with migratory circinate erythema (EBSMCE) epidermolysis bullosa simplex with mottled pigmentation epidermolysis bullosa simplex with pyloric atresia (EBS-PA) epidermolysis bullosa simplex, autosomal recessive epidermolysis bullosa simplex, Dowling-Meara type; epidermolysis bullosa herpetiformis, Dowling-Meara type (EBS-DM) epidermolysis bullosa simplex, Koebner type epidermolysis bullosa simplex, Ogna type epidermolysis bullosa simplex, Weber-Cockayne type; Cockaye-Touraine type epidermolysis bullosa; epidermolysis bullosa of hands & feet

General

epidermolysis bullosa genetic disease of the skin (genodermatosis)

References

  1. OMIM :accession 601001, 131760, 131900, 131950
  2. Harrison's Principles of Internal Medicine, 14th ed., Fauci et al eds., 1998, p2193-4