Major criteria:
(1) swelling, induration and thickening of the skin and subcutaneous tissue
(2) fascial thickening with accumulation of lymphocytes and macrophages with or without eosinophilic infiltration
where:
• Fascia thickening is determined in a full-thickness wedge biopsy of clinically affected skin.
• Most of the lymphocytes involving the fascia should be CD8+.
• Swelling and induration may be symmetrical or nonsymmetrical.
• Swelling and induration may be diffuse or localized.
• Swelling and induration spare the acral zones.
Minor criteria:
(1) eosinophilia (absolute eosinophil count > 500 per µL)
(2) hypergammaglobulinemia (> 1.5 g/L)
(3) muscle weakness and/or elevated serum aldolase activity
(4) groove sign and/or peau d'orange
(5) hyperintense fascia on T2-weighted images in MRI
Diagnosis:
(1) presence of both major criteria
(2) >= 2 minor criteria
(3) exclusion of systemic sclerosis.
