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

More general terms

• epidermolysis bullosa
• genetic disease of the skin (genodermatosis)

More specific terms

• 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