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Recessive dystrophic epidermolysis bullosa inversa

What is Recessive dystrophic epidermolysis bullosa inversa?

Recessive dystrophic epidermolysis bullosa inversa (RDEB-I) is rare subtype of dystrophic epidermolysis bullosa (DEB, see this term) characterized by blisters and erosions which are primarily confined to intertriginous skin sites, the base of the neck, the uppermost back, and the lumbosacral area.

Other condition names

  • Dystrophic epidermolysis bullosa inversa
  • Inverse RDEB
  • Inverse recessive dystrophic epidermolysis bullosa
  • RDEB-I

Inheritance type

Autosomal recessive

Prevalence

  • Worldwide: <1 in 1,000 000

Age of Onset

  • Infancy
  • Neonatal
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on "https://www.orphadata.com". Data version 1.3.16 / 4.1.7.