Dystrophic epidermolysis bullosa (DEB) is a form of inherited epidermolysis bullosa (EB) characterized by cutaneous and mucosal fragility resulting in blisters and superficial ulcerations that develop below the lamina densa of the cutaneous basement membrane and that heal with significant scarring and milia formation. It comprises ten sub-types with the three most common being generalized dominant DEB (DDEB), severe generalized recessive DEB (RDEB- sev gen) and RDEB generalized-other. [ Orphanet:303 ]

This is just here as a test because I lose it

Term information

database cross reference
  • GARD:0002150 (Orphanet-shared)
  • ICD10:Q81.2 (Orphanet:303)
  • Orphanet:303 (MONDO:equivalentTo)
Subsets

ordo_group_of_disorders

abbreviation
DEB [ Orphanet:303 ]

closeMatch

http://linkedlifedata.com/resource/umls/id/C0079294

definition

Dystrophic epidermolysis bullosa (DEB) is a form of inherited epidermolysis bullosa (EB) characterized by cutaneous and mucosal fragility resulting in blisters and superficial ulcerations that develop below the lamina densa of the cutaneous basement membrane and that heal with significant scarring and milia formation. It comprises ten sub-types with the three most common being generalized dominant DEB (DDEB), severe generalized recessive DEB (RDEB- sev gen) and RDEB generalized-other.

exactMatch

http://www.orpha.net/ORDO/Orphanet_303

has exact synonym

DEB

epidermolysis bullosa dystrophica

Dermolytic epidermolysis bullosa

has related synonym

epidermolysis bullosa, dermolytic

id

MONDO:0017608