Elsevier

Neuromuscular Disorders

Volume 16, Issue 2, February 2006, Pages 144-148
Neuromuscular Disorders

Case report
Eosinophilic fasciitis in a child mimicking a myopathy

https://doi.org/10.1016/j.nmd.2005.12.001Get rights and content

Abstract

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.

Introduction

In 1974, Shulman first described a new clinical syndrome of diffuse fasciitis with hypergammaglobulinemia and eosinophilia [1]. This syndrome, later termed eosinophilic fasciitis (EF), is a rare disorder of unknown aetiology. In the revised classification system of morphea (localized scleroderma) proposed by Peterson et al. [2], EF belongs to the subtype of deep morphea. The fascia is the predominant level of involvement. Characteristics of EF include painful induration of the skin and soft tissues with peau d'orange appearance, and inflamed and later on sclerotic superficial fascia underlying cutaneous lesions. These changes can appear at any part of the body, but are mainly limited to the extremities. Characteristic laboratory findings include an elevated erythrocyte sedimentation rate (ESR), peripheral eosinophilia and hypergammaglobulinemia. Full thickness biopsy shows thickening of the fascia and fasciitis with inflammatory infiltration of lymphocytes, plasmacells, histiocytes and eosinophils in variable degree.

In children, EF usually has the same presentation as in adults, however, it predominantly occurs in girls, and arthritis or haematological disorders (like aplastic anaemia or thrombocytopenic purpura) are less common [3], [4]. Two thirds of 21 paediatric patients described by Farrington et al. with biopsy proven EF had progressive cutaneous fibrosis resulting in flexion contractures [4]. Furthermore, specific clinical presentations, not seen in adults, have been reported. For example, the development of painless contractures without involvement of the skin might be a phenotype of EF specific to childhood [5].

In this paper, we discuss a 14-year-old boy, in whom a myopathy was suspected because of painless joint contractures and muscle weakness in the absence of skin changes. In such a case, EF has to be considered as a differential diagnosis of a neuromuscular disease in childhood.

Section snippets

Case report

A right-handed 14-year-old boy was referred to our neuromuscular centre because of a suspected neuromuscular disorder with joint contractures. For 8 months he had noticed a painless limitation of his right forefinger with progressive stiffness of other fingers, right wrist and elbow. He had not suffered from fever, skin changes, joint swelling or pain, general malaise or decreased muscular strength, nor signs of Raynaud's phenomenon. He could play handball without any difficulties until a few

Discussion

The clinical presentation of EF is diverse. Usually it presents with painful thickening of the skin and eventually progresses to cutaneous fibrosis and joint contractures. Swelling and stiffness of the extremities may mimic muscle weakness. However, muscle strength is hardly impaired and muscle enzyms are normal. Although the inflammation predominantly involves the fascia, it also may involve the deep dermis, subcutaneous tissue and superficial muscle [2]. Clinically and histologically EF

Acknowledgements

We thank Dr Martin M.Y. Lammens for preparing and photographing the biopsy specimen and Dr Ton M. van der Vliet for the MRI data.

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