[Elevated serum aldolase activity in a patient of non-eosinophilic myofasciitis and synovitis with perifascicular atrophy].

A 35-year-old man suffered from myalgia and joint pain on walking for 5 months. Physical and neurological examinations revealed dermal sclerosis, skin swelling, redness of forearms, Raynaud's phenomenon, joint pain, myalgia and muscle weakness. Eosinophilia was not found and serum creatine kinase activity was normal, while aldolase was markedly elevated. Abnormal signals suggesting synovitis and myofasciitis were found on MRI images. Biopsy of the fascia of quadriceps femoris showed perivascular mononuclear cell infiltration. A muscle biopsy showed mononuclear cell infiltration mainly in the perimysium extending to the endmysium. Eosinophilic cells were not found, Perifascicular atrophy was observed. Corticosteroid therapy improved clinical symptoms and serum aldolase level. We diagnosed him as non-eosinophilic myofasciitis and synovitis with perifascicular atrophy. The serum aldolase activity is usuful for diagnosis and for monitoring the disease activity.

Magnetic resonance imaging and serum aldolase concentration in eosinophilic fasciitis.

We report a 26-year-old man with eosinophilic fasciitis who presented with progressive joint contractures of all four limbs. In this patient, the serum aldolase concentration was elevated while the serum creatinine kinase concentration was normal. Magnetic resonance imaging showed gadolinium enhancement of fascial structures in the lower limbs. With the clinical improvement by the treatment with oral corticosteroid, the aldolase concentration was decreased, and the fascial hyperintensity on magnetic resonance images was reduced. The phenomenon of increased an aldolase concentration accompanied by a normal creatinine kinase concentration may be characteristic of eosinophilic fasciitis. Serial magnetic resonance images and serum aldolase concentration are useful for monitoring the therapeutic response in this condition.

Serum levels of matrix metalloproteinase-13 in patients with eosinophilic fasciitis.

Matrix metalloproteinase-13 (MMP-13), a member of the collagenase family, has been implicated in the pathogenesis of connective tissue diseases characterized by extracellular matrix remodeling. Since serum MMP-13 levels reflect disease severity of systemic sclerosis and localized scleroderma, we evaluated the clinical significance of serum MMP-13 levels in eosinophilic fasciitis (EF). All the EF patients had serum MMP-13 levels lower than the mean - 2SD of healthy controls. Serum MMP-13 levels were also significantly decreased in EF patients compared with diffuse cutaneous systemic sclerosis, limited cutaneous systemic sclerosis, and generalized morphea patients. Although serum MMP-13 levels did not reflect any clinical and serological features of EF, these results indicate that MMP-13 may be involved in the development of this disease.

Tartrate-resistant acid phosphatase containing cells in nodular fasciitis, proliferative fasciitis, and proliferative myositis.

Five cases of nodular fasciitis, three cases of proliferative fasciitis, and one case of proliferative myositis were investigated by tartrate-resistant acid phosphatase (TRAP) histochemistry on conventional paraffin-embedded material. In all cases varying numbers of enzyme-positive cells were detected. Cytological investigations revealed the presence of small round cells, elongated spindle cells, medium-sized mono- or binucleated cells, and multinuclear giant cells, the latter resembling osteoclasts. However, the typical ganglion-like cells were TRAP-negative in almost all tests. The TRAP-pattern may help to differentiate nodular fasciitis and allied lesions from inflammatory pseudotumours or organising haematomas, as the latter do not contain TRAP-positive cells. As TRAP appears to be a marker for a special population of phagocytizing and non phagocytizing histiocytes, nodular fasciitis and its proliferative variants may be assumed to be fibrohistiocytic tumorous conditions.

[A case of eosinophilic fasciitis with excessive increase of muscle enzyme].

A sixty-year old woman had muscle pain of her extremities and stiffness in her fingers after unusual physical exertion. On admission she developed indurative swellings of the upper extremities and flexion contractures of her fingers. Laboratory data showed peripheral blood eosinophilia and excessive increase of muscle enzymes. Electromyography showed a myopathic pattern and magnetic resonance imaging showed findings of myositis. We performed a full thickness biopsy from skin to muscle. In histopathologic studies, collagenous hypertrophy of the fascia and perifascicular atrophy of muscle fibers and infiltrates on eosinophiles in the interstitial tissue were found. She was treated with prednisolone (60 mg/day). Clinical symptoms showed improvement and her peripheral blood eosinophiles and serum muscle enzymes deminished quickly. In this case, we suggest that the characteristic histopathologic findings in eosinophilic faciitis is not fascia alone, but includes muscle. We stress that eosinophilic faciitis and polymyositis are closely related.