Eosinophilic fasciitis in a child mimicking a myopathy

A 14-year-old boy was suspected of having a myopathy with joint contractures. He presented with progressive painless joint contractures of his right wrist and fingers, and reduced muscle strength of his right arm, without obvious skin changes. Laboratory investigation showed a normal CK, hypergammag...

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Bibliographic Details
Published inNeuromuscular disorders : NMD Vol. 16; no. 2; pp. 144 - 148
Main Authors Pillen, Sigrid, Engelen, Baziel van, Hoogen, Frank van den, Fiselier, Theo, Vossen, Pim van der, Drost, Gea
Format Journal Article
LanguageEnglish
Published England Elsevier B.V 01.02.2006
Subjects
Adolescent
Anti-Inflammatory Agents - therapeutic use
Antirheumatic Agents - therapeutic use
Biopsy
Child
Contracture - diagnosis
Contracture - diagnostic imaging
Contracture - drug therapy
Contracture - pathology
Contractures
Diagnosis, Differential
Eosinophilia - diagnosis
Eosinophilia - drug therapy
Eosinophilia - pathology
Eosinophilic fasciitis
Fascia - diagnostic imaging
Fascia - pathology
Fascia - physiopathology
Fasciitis - diagnosis
Fasciitis - drug therapy
Fasciitis - pathology
Humans
Joints - diagnostic imaging
Joints - pathology
Male
Methotrexate - therapeutic use
Muscular Diseases - diagnosis
Muscular Diseases - pathology
Prednisolone - therapeutic use
Skin - pathology
Ultrasonography
Contractures
Eosinophilic fasciitis
Child
Ultrasonography
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Summary:A 14-year-old boy was suspected of having a myopathy with joint contractures. He presented with progressive painless joint contractures of his right wrist and fingers, and reduced muscle strength of his right arm, without obvious skin changes. Laboratory investigation showed a normal CK, hypergammaglobulinemia and eosinophilia. Ultrasonography revealed thickened fasciae in the forearm. A full thickness biopsy confirmed the diagnosis of eosinophilic fasciitis. The contractures diminished quickly with prednisolone and methotrexate. The ultrasound scans 2 years after diagnosis showed improvement, although some thickening of the fasciae was still present, indicating residual changes. In conclusion, eosinophilic fasciitis has to be regarded as a differential diagnosis of painless joint contractures in children. Ultrasonography can be helpful to suspect the diagnosis.
Bibliography:ObjectType-Case Study-2
SourceType-Scholarly Journals-1
ObjectType-Feature-4
content type line 23
ObjectType-Report-1
ObjectType-Article-3
ISSN:0960-8966
1873-2364
DOI:10.1016/j.nmd.2005.12.001