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Author Spotlight: Investigating the Pathophysiology of Eosinophilic Esophagitis
Published on: May 10, 2024
Juvenile eosinophilic fasciitis: three case reports with review of the literature
Riccardo Papa1, Paolo Nozza2, Claudio Granata3
1UOC Pediatria II, Reumatologia, Istituto Giannina Gaslini, Genoa, Italy. papariccardo86@gmail.com.
Objectives:
Eosinophilic fasciitis is an uncommon scleroderma-like disorder characterised by induration and thickening of skin and soft tissue, usually associated with peripheral eosinophilia, poorly characterised in childhood.
Methods:
We report 3 paediatric cases of eosinophilic fasciitis showing unusual clinical and histopathological features with a review of the literature.
Results:
All cases presented progressive motility impairment started from upper limbs with no skin abnormalities. All cases showed systemic inflammatory involvement and 2 patients had acute complications. Two patients developed disabling outcomes despite appropriate treatments.
Conclusions:
Eosinophilic fasciitis may present unusual clinical and histopathological features during childhood and requires early recognition in order to prevent acute complications and disabling outcomes.
Insights
Eosinophilic fasciitis (EF) in children can present unusually, without skin changes but with motility issues and inflammation. Early diagnosis is crucial to prevent severe complications and long-term disability.
Area of Science:
- Pediatric rheumatology
- Dermatology
- Pathology
Background:
- Eosinophilic fasciitis (EF) is a rare scleroderma-like condition.
- It is characterized by tissue induration and thickening, often with peripheral eosinophilia.
- EF is poorly understood in pediatric populations.
Observation:
- Three pediatric cases of EF with atypical presentations were analyzed.
- Cases exhibited unusual clinical and histopathological features.
- Motility impairment, primarily in upper limbs, was a common feature, often without skin abnormalities.
Findings:
- All patients presented with systemic inflammatory involvement.
- Two patients experienced acute complications.
- Despite treatment, two patients had disabling outcomes.
Implications:
- Pediatric eosinophilic fasciitis can manifest atypically.
- Early recognition of unusual clinical and histopathological signs is vital.
- Prompt diagnosis and intervention may prevent severe complications and long-term disability in children.
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