Abstract

HISTORY: 29 yo male Brazilian jujitsu fighter with a history of Henoch-Schonlein purpura and eczema presents to clinic for 6 months of right anterolateral calf pain without a clear injury. He reports constant, dull, aching pain, worse with dorsiflexion and resisted plantar flexion. He notes associated pitting edema, dry leathery skin, hypopigmentation, and hyperesthesia that began 3-4 months ago. He has stopped jujitsu due to symptoms. Tib-fib xrays and venous duplex were negative. He tried acetaminophen, ibuprofen, physical therapy, topical clobetasol, and oral prednisone with short-term improvement. PHYSICAL EXAMINATION: Skin of the anterolateral calf is indurated with hair loss, a leathery appearance, and areas of depigmentation. The distal lateral calf is warm and erythematous, with swelling posterior to the lateral malleolus. Tinel’s sign is negative at the fibular head. Pulses are normal bilaterally with ankle plantar and dorsiflexion. Ankle ROM is full. Light touch sensation is intact in L2-S2 dermatomes and strength is 5/5 in lower extremities. DIFFERENTIAL DIAGNOSIS: Complex regional pain syndrome, Cellulitis, Scleroderma, Eosinophilic fasciitis, Chronic exertional compartment syndrome, Vascular insufficiency TEST AND RESULTS: Lab work: inflammatory markers and rheumatologic labs negative except for elevated CK (356) of unknown significance. EMG/NCS: low amplitude right vs left sural sensory nerve suggesting sural neuropathy although within normal limits; no fibular neuropathy or radiculopathy. MRI right leg: nonspecific skin thickening of anterolateral shin with fascial edema and mild fibularis longus myositis. Skin biopsy: linear morphea versus eosinophilic fasciitis FINAL WORKING DIAGNOSIS: Segmental Linear Morphea (localized scleroderma) TREATMENT AND OUTCOMES: Dermatology referral led to biopsy; subsequently, treatment with PO methotrexate (MTX), high dose IV methylprednisolone for three cycles, and topical clobetasol ointment BID. UVA1 phototherapy was also started due to extensive disease and ankle and knee joint involvement. Subjective improvement in ROM, skin stiffness, and strength per patient after 2 cycles of methylprednisolone. Switched to SQ MTX for better absorption and lower cost. Patient continues to improve as he remains on the above treatment protocol.

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