[Immunohistochemical studies on pathogenetic features of diffuse XXXX ].

@entity1712 @entity4 @entity30148 @entity9156 @entity617
Three cases of diffuse @entity9156 were reported: case 1, a 24-year-old female, case 2, an 18-year-old male and case 3, a 61-year-old male. Their prominent features were reduced extensibility of the muscle and/or fascia. Skin was taut and bound underneath in cases 1 and 3, and focal @entity4 was noted in the left forearm of case 2. Firmness of the muscle and/or fascia was dominantly seen in four extremities in case 1, proximal limbs in case 2 and in the trunk and proximal limbs in case 3. Muscle weakness was not seen in case 1, slight in the limbs of case 2 and moderate in the proximal limbs of case 3. Transient @entity1712 was observed in case 1. IgG was elevated in all 3 cases and IgM in cases 1 and 3. Antinuclear antibody was positive in case 1 and 2, and anti-ENA antibody in case 3. Remarkable thickening of the fascia and perivascular mononuclear cell infiltration in the fascia were main pathological features in all three cases. Inflammatory alteration of the muscle was modest and limited to the perifascicular and epimysial sites underneath the fascia. In these 3 cases, a diagnosis of @entity30148 or diffuse @entity9156 with @entity1712 was made, although eosinophils were scarce in the fascia or muscle tissue. All 3 cases well responded to the oral @entity617 . Mononuclear cells clustered mainly in the perivascular sites, comprising mostly T cells, helper T cells, and partly B cells. These findings suggest that helper T cell mediated immune mechanism may play a pathogenetic role in this disease.(ABSTRACT TRUNCATED AT 250 WORDS)
