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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

Authors
  • Baldo, Francesco1, 2
  • Brena, Michela3
  • Carbogno, Simone1, 2
  • Minoia, Francesca1
  • Lanni, Stefani1
  • Guez, Sophie1
  • Petaccia, Antonella1
  • Agostoni, Carlo1, 2
  • Cimaz, Rolando4, 2
  • Filocamo, Giovanni1
  • 1 Fondazione IRCCS Cà Granda, Ospedale Maggiore Policlinico, Via della Commenda, 9, Milan, 20122, Italy , Milan (Italy)
  • 2 University of Milan, Milan, Italy , Milan (Italy)
  • 3 Dermatology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy , Milan (Italy)
  • 4 ASST G.Pini-CTO, Milan, Italy , Milan (Italy)
Type
Published Article
Journal
Pediatric Rheumatology
Publisher
Springer Science and Business Media LLC
Publication Date
Jun 03, 2021
Volume
19
Issue
1
Identifiers
DOI: 10.1186/s12969-021-00571-9
Source
Springer Nature
Keywords
License
Green

Abstract

BackgroundHarlequin 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 presentationWe 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.ConclusionThe 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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