Non-Herlitz Junctional Epidermolysis Bullosa

Kim B. Yancey, Helmut Hintner

Research output: Contribution to journalReview article

35 Scopus citations

Abstract

Non-Herlitz junctional epidermolysis bullosa (nH JEB) is characterized by generalized blisters that predominate in sites exposed to friction, trauma, or heat. Whereas infants and children with nH JEB often appear to resemble patients with other forms of EB, adults with this disorder typically display atrophic scars, hypopigmentation, or hyperpigmentation at sites of healed blisters as well as incomplete alopecia, dystrophic nails, mucous membrane involvement, and dental abnormalities. Mild (or severe) disease early in life may be characterized by the opposite phenotype in adults with nH JEB. Although nH JEB is generally less severe than Herlitz disease, fatalities (especially in neonates) are not uncommon among patients with the former diagnosis.

Original languageEnglish (US)
Pages (from-to)67-77
Number of pages11
JournalDermatologic Clinics
Volume28
Issue number1
DOIs
StatePublished - Jan 1 2010

Keywords

  • Blistering disease
  • Epidermal adhesion
  • Generalized atrophic benign epidermolysis bullosa
  • Genetic disease
  • Herlitz disease

ASJC Scopus subject areas

  • Dermatology

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