An unusual case of inflammatory necrotizing myopathy and neuropathy with pipestem capillaries

Anil Kumar B Patil, AT Prabhakar, Ajith Sivadasan, Mathew Alexander, Geeta ChackoNeurology India 2015 63(1):72-76Necrotizing myopathy with pipestem capillaries is a form of chronic inflammatory myopathy, with histopathology showing necrotizing myopathy, minimal cellular infiltration, and microangiopathy. A 30-year-old female presented with progressive limb weakness of 6 months, with skin pigmentation and Raynaud's phenomenon. Serum creatine phosphokinase was 3990 u/L. Muscle biopsy showed necrotic fibers, focal sparse perivascular inflammation/perifascicular atrophy, endomysial/epimysial vessel wall thickening with luminal narrowing. The features were of inflammatory necrotizing myopathy and neuropathy with pipestem capillaries/microangiopathy. She was pulsed with intravenous immunoglobulin, methylprednisolone, and cyclophosphamide and showed a good improvement. In the absence of widespread inflammatory response and classical histopathology findings, it is important to diagnose this condition as it shows a good response to aggressive and prolonged immunotherapy.

Source: Neurology India - March 4, 2015 Category: Neurology Authors: Anil Kumar B PatilAT PrabhakarAjith SivadasanMathew AlexanderGeeta Chacko Source Type: research