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Papers of the Week

2020 Jun

Br J Dermatol



Treatment of epidermolysis bullosa pruriginosa-associated pruritus with dupilumab.


Shehadeh W, Sarig O, Bar J, Sprecher E, Samuelov L
Br J Dermatol. 2020 Jun; 182(6):1495-1497.
PMID: 31899820.


Epidermolysis bullosa pruriginosa (EBP; MIM#604129) is a rare clinical subtype of autosomal dominant (or less commonly recessive) dystrophic epidermolysis bullosa (DEB). In addition to usual manifestations of DEB including trauma-induced skin fragility, milia and nail dystrophy, EBP features severe pruritus, prurigo nodularis (PN) and lichen simplex chronicus-like lesions which may resemble other dermatoses and thereby complicate clinical diagnosis. Like other types of DEB, EBP results from heterozygous or bi-allelic mutations in the gene COL7A1 encoding collagen VII which is the main component of the anchoring fibrils.