[Immunohistochemical studies on pathogenetic features of diffuse fasciitis]

Area of Science:

• Rheumatology
• Immunology
• Pathology

Background:

• Diffuse fasciitis, including Shulman syndrome, presents with reduced muscle/fascia extensibility.
• Clinical manifestations include taut skin, edema, and muscle weakness.
• Laboratory findings may show elevated immunoglobulins and positive autoantibodies.

Observation:

• Three patients (24F, 18M, 61M) presented with reduced muscle and/or fascia extensibility.
• Physical findings included taut skin, edema, and varying degrees of muscle weakness.
• Pathological examination revealed fascial thickening and perivascular mononuclear cell infiltration.

Findings:

• Despite the name, eosinophils were scarce in affected tissues.
• Elevated IgG and IgM levels, along with positive antinuclear and anti-ENA antibodies, were noted.
• Histopathology showed significant fascial changes with modest muscle inflammation.

Implications:

• The findings support Shulman syndrome as a distinct entity within diffuse fasciitis.
• A helper T-cell mediated immune mechanism is implicated in the pathogenesis.
• Oral prednisolone demonstrated efficacy in treating these cases.