Abstract:

Wells’ Syndrome (WS), a rare inflammatory condition of unknown etiology, has been associated with arthropod bites, Churg- Straus syndrome, hematologic and solid malignancies, and typically presents with pruritic, erythematous plaques, nodules or bullous lesions. We report a rare presentation of WS with compartment syndrome. A 62-year-old female presented with two days of rapidly progressive right-hand swelling, reduced range of motion, fever and chills, but denied injury. X-ray of the right hand showed soft tissue swelling and a 3 mm calcific or metallic foreign body in the soft tissue between the right thumb and forefinger. Computed tomography showed right lung nodules up to 8 mm in diameter and splenomegaly. Due to concern for infection, intravenous antibiotics were started. However, because of symptom progression she underwent right hand decompressive fasciotomy and debridement. A biopsy of a skin lesion on the right forearm showed intraepidermal vesiculation, superficial and deep interstitial inflammation, abundant eosinophils and flame figures. There was peripheral eosinophilia (9.8 K/ul) and serum immunoglobulins showed IgM: 1300 mg/dl, IgG 4300 mg/dl, IgE: 4900 mg/dl, a normal kappa/lambda ratio, and positive Borrelia burgdorferi IgM. Steroids were withheld until a bone marrow biopsy to evaluate for a lymphoproliferative disorder was negative. Cytogenetic and molecular studies for JAK2 and PDGFRA were inconclusive. After steroids her pain improved, eosinophils began to decrease, and she was discharged to follow up. In summary, this rare presentation of compartment syndrome emphasizes the need to consider WS in the work up of a patient presenting with cellulitis-like symptoms.

Financial Disclosure:
No current or relevant financial relationships exist.

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Jason Pimentel