Department of Rheumatology, Higashi-Hiroshima Memorial Hospital, Higashi-Hiroshima and
1 Second Department of Internal Medicine, Hiroshima University School of Medicine, Hiroshima, Japan
Sir, Eosinophilic fasciitis is a distinct clinicopathological disorder, characterized by peripheral eosinophilia and histological evidence of thickening and inflammatory cell infiltration of the deep fascia [1, 2]. The aetiology and pathogenesis of eosinophilic fasciitis remain unknown. We describe a patient with bronchial asthma who developed relapsing eosinophilic fasciitis.
A 50-yr-old woman with a 16-yr history of bronchial asthma was admitted to our hospital complaining of right upper arm swelling. Eleven and 3 months before admission, she had developed swelling and muscle weakness of the bilateral upper extremities (from shoulder to wrist) associated with marked peripheral blood eosinophilia (58 and 88%, respectively), leucocytosis (27 000 and 48 000/mm3) and increased creatine phosphokinase (548 and 125 IU/l; normal 170) and aldolase (11.2 and 17.8 IU/l; normal
7.6) activity. These symptoms and abnormal test data had normalized within 4 weeks after oral administration of prednisolone (PSL, initial dose of 30 or 40 mg/day) under a suspected diagnosis of eosinophilic fasciitis. After remission of these conditions, she had been maintained on theophylline 400600 mg/day and PSL 5 mg/day orally, both of which were used as the maintenance doses for several years before admission to treat bronchial asthma. She has no history of taking L-tryptophan or ChurgStrauss syndrome-related diseases, such as allergic rhinitis and nasal polyposis.
Examination showed moderate swelling of the right upper arm without cutaneous sclerosis, normal pulmonary vesicular sounds on auscultation, and normal chest X-ray findings. Laboratory blood testing revealed leucocytosis 14 000/mm3 and peripheral blood eosinophilia 28%. Levels of muscle-associated enzymes and inflammatory indicators were within normal limits. Haematological malignancy was ruled out by bone marrow aspiration. Serological tests for parasites, and antinuclear, anti-Jo-1 and anti-Scl-70 antibodies were negative. En bloc biopsy specimens of the right deltoid muscle revealed marked thickening of the fascia and infiltration of inflammatory cells, mainly eosinophils, into the fascia and associated tissues, including the deep subcutis and skeletal muscle, with mild atrophy and degeneration of the muscle cells (Fig. 1). Skin biopsy showed no characteristic findings of scleroderma. There was no evidence of vasculitis histopathologically or clinical symptoms suggesting the presence of vasculitis, such as fever, neuropathy, body weight loss and nephritis, and ChurgStrauss syndrome was ruled out. Eosinophilic fasciitis was diagnosed based on the clinical, laboratory and specific histopathological findings [1], and oral administration of PSL 40 mg/day was started. The right upper arm swelling and peripheral blood eosinophilia improved rapidly without residual induration, and the PSL was tapered to 5 mg/day. During admission and after discharge, we recommend regular steroid (beclomethasone dipropionate, 300400 µg/day) inhalation, which this patient previously performed only for bronchial asthma attacks, in addition to oral steroids (PSL, 5 mg/day), to reduce inflammation of the bronchial epithelium associated with asthma [3]. During the subsequent 6 yr of follow-up, the patient was totally free of not only attacks of bronchial asthma, for which she had been hospitalized several times a year despite low-dose administration of oral steroids before the addition of regular steroid inhalation, but also eosinophilia, and fascicular and muscular symptoms.
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Notes
Correspondence to: S. Ishioka.
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