Complete remission of relapsing eosinophilic fasciitis associated with bronchial asthma following regular steroid inhalation

Y. Yamanishi, S. Ishioka1, and M. Yamakido1

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 400–600 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 Churg–Strauss 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. 1Go). 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 Churg–Strauss 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, 300–400 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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FIG. 1. Photomicrograph of the right deltoid muscle. Severe thickening of the fascia and infiltration of inflammatory cells into the fascia and associated tissues including the deep subcutis and skeletal muscle can be clearly seen.

 
Gabrielli et al. [4] reported a progressive case of eosinophilic fasciitis with simultaneous development of bronchial asthma, and proposed a significant role of mast cells in the pathogenesis of eosinophilic fasciitis. Their case differed from ours in that many mast cells, not eosinophils, infiltrated the fascia. To our knowledge, there is no report of eosinophilic fasciitis associated with any other allergic diseases and this is the first case of relapsing eosinophilic fasciitis associated with bronchial asthma. The fact that regular inhalation of low-dose steroid, which is considered to affect only the bronchial epithelium [5], prevented the relapse of eosinophilic fasciitis, suggests that activated inflammatory cells including eosinophils in the bronchial epithelium might have infiltrated the fascia to produce fasciitis.

Notes

Correspondence to: S. Ishioka. Back

References

  1. Shulman LE. Diffuse fasciitis with eosinophilia: a new syndrome? Trans Assoc Am Phys1975;88:70–86.[Medline]
  2. Barnes L, Rodnan GP, Medsger TA, Short D. Eosinophilic fasciitis. Am J Pathol1979;96:493–507.[Abstract]
  3. Djukanovic R, Wilson JW, Britten KM et al. Effect of an inhaled corticosteroid on airway inflammation and symptoms in asthma. Am Rev Respir Dis1992;145:669–74.[ISI][Medline]
  4. Gabrielli A, De Nictolis M, Campanati G, Cinti S. Eosinophilic fasciitis: a mast cell disorder? Clin Exp Rheumatol1983;1:75–80.[ISI][Medline]
  5. Svendsen UG, Frolund L, Heinig JH, Madsen F, Nielsen NH, Weeke B. High-dose inhaled steroids in the management of asthma: a comparison of the effects of budesonide and beclomethasone dipropionate on pulmonary function, symptoms, bronchial responsiveness and the adrenal function. Allergy1992;47:174–80.[ISI][Medline]
Accepted 8 October 1999





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