Juvenile eosinophilic fasciitis presenting with polyarthriti
The present case has been reported in the Indian Journal of Rheumatology.

A 5-year-old girl presented with a history of the inability to bear weight for 3 months, accompanied with swollen hands and feet, along with morning stiffness which usually lasted for 2–3 h daily. Physical examination of the skin revealed bilateral nonpitting edema involving the dorsum of hands, proximal and distal phalanges, extending to mid-forearms with tethering of skin to the underlying tissue.

There was difficulty in flexion and extension of her fingers, as well as an inability to adopt a praying position. Koebner's phenomenon (linear erythema in the arms after applying blood pressure cuff) was positive. Similar findings were noted on the feet, with nonpitting edema and taut skin which involved the metatarsophalangeal joints and ankles.

Musculoskeletal examination (MSK) showed contracted knuckles, warm wrists with tenderness, and restricted range of movement (ROM). There was also tenderness of both tibialis and quadriceps muscles, both hip and knee joints appeared contracted with restricted movements, and she had toe-walking gait. On further MSK examination, there was remarkable tenderness over the cervical and thoracolumbar spine with significant restriction in the ROM.

Rheumatology screening resulted in antinuclear antibody, rheumatoid factor, anti-cyclic citrullinated peptide, antineutrophil cytoplasmic antibody, and Extractable nuclear antigen (ENA) antibody were all negative and C3 and C4 were normal. Plain X-rays of hands and knees revealed soft-tissue swellings and normal bones.

A full-thickness skin biopsy was performed which showed collagenization of the subcutaneous fat dividing it into lobules with broad collagen bands in between. In addition, there was diffuse chronic inflammatory cell infiltrate composed of mainly lymphocytes, plasma cells, and few eosinophils in the thick fascia.

The patient was started on pulses of methylprednisolone, 10 mg/kg/day was given for 3 days, along with occupational therapy sessions. Within 48 h, both the edema and skin thickening improved significantly. With the treatment, morning stiffness and joint pain regressed dramatically.

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