Juvenile Idiopathic Arthritis in Harlequin Ichthyosis, a Rare Combination or the Clinical Spectrum of the Disease? Report of a Child Treated with Etanercept and Review of the Literature

Overview

Journal Pediatr Rheumatol Online J

Publisher Biomed Central

Specialty Pediatrics

Date 2021 Jun 4

PMID 34082764

Citations 3

Authors

Francesco Baldo

Michela Brena

Simone Carbogno

Francesca Minoia

Stefano Lanni

Sophie Guez

Antonella Petaccia

Carlo Agostoni

Rolando Cimaz

Giovanni Filocamo

Affiliations

Soon will be listed here.

Abstract

Background: Harlequin ichthyosis (HI) is the most severe phenotype of autosomal recessive congenital ichthyosis. Juvenile Idiopathic Arthritis (JIA) represents a heterogenous group of disorders all sharing the clinical manifestation of chronic arthritis. Association of HI and chronic arthritis has been reported in few cases.

Case Presentation: We report the case of a child with HI who developed a severe form of chronic polyarthritis during the first years of life, treated with repeated multiple joint injections, methotrexate and etanercept with good response and without any adverse events.

Conclusion: The reported case and the literature review highlighted the presence of a peculiar severe seronegative polyarthritis with early onset in a series of patients with HI, suggesting that polyarthritis may be a specific manifestation of HI, rather than a rare combination of two separate conditions.

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