Kidney-Urinary Tract Involvement in Intermediate Junctional Epidermolysis Bullosa.

ABSTRACT:

Importance

Kidney-urinary tract (KUT) manifestations cause substantial morbidity in patients with junctional epidermolysis bullosa (JEB), but the spectrum of disease severity and the clinical course have been poorly characterized.

Objective

To examine in a large cohort of patients with intermediate JEB the KUT manifestations, diagnostic and therapeutic procedures, genotype-phenotype correlations, and outcomes as a basis for recommendations, prognosis, and management.

Design, setting, and participants

In this retrospective, longitudinal case series study, 99 patients with a diagnosis of JEB based on clinical and genetic findings who were treated in a single dermatology department in Freiburg, Germany, were assessed during an 18-year period (January 1, 2003, to December

SUBMITTER: Trefzer L 

PROVIDER: S-EPMC9350844 | biostudies-literature | 2022 Sep

REPOSITORIES: biostudies-literature