Dystrophic epidermolysis bullosa pruriginosa: a new case series of a rare phenotype unveils skewed Th2 immunity.

doi:10.1111/jdv.17671

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Article

Dystrophic epidermolysis bullosa pruriginosa: a new case series of a rare phenotype unveils skewed Th2 immunity.

Journal of the European Academy of Dermatology and Venereology : JEADV - 1 Jan 2022

Darbord D, Hickman G, Pironon N, Barbieux C, Bonnet-des-Claustres M, Titeux M, Miskinyte S, Cordoliani F, Vignon-Pennamen M D, Amode R, Hovnanian A, Bourrat E

Abstract excerpt

BACKGROUND: Dystrophic epidermolysis bullosa pruriginosa (DEB-Pr) is a rare subtype of hereditary epidermolysis bullosa, with a poorly understood pathogenesis and no satisfactory treatment. OBJECTIVES: To assess the clinical and biological features, genetic basis and therapeutic management, to better characterize this rare genodermatosis. METHODS: We have conducted a retrospective study, reviewing the clinical...

Topics

Adult
Aged
Collagen Type VII
Epidermolysis Bullosa Dystrophica
Filaggrin Proteins
Humans
Middle Aged
Mutation
Phenotype
Retrospective Studies

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Identifiers

PMID: 34543471
DOI: 10.1111/jdv.17671

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