Neurol India Home 
 

CASE REPORT
Year : 2015  |  Volume : 63  |  Issue : 1  |  Page : 72--76

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

Anil Kumar B Patil, AT Prabhakar, Ajith Sivadasan, Mathew Alexander, Geeta Chacko 
 Department of Neurological Sciences, Christian Medical College, Vellore, Tamil Nadu, India

Correspondence Address:
Dr. Mathew Alexander
Prof and Head, Neurology, Department of Neurological Sciences, Christian Medical College, Vellore, Tamil Nadu - 632 004
India

Necrotizing 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«SQ»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.


How to cite this article:
Patil AB, Prabhakar A T, Sivadasan A, Alexander M, Chacko G. An unusual case of inflammatory necrotizing myopathy and neuropathy with pipestem capillaries.Neurol India 2015;63:72-76


How to cite this URL:
Patil AB, Prabhakar A T, Sivadasan A, Alexander M, Chacko G. An unusual case of inflammatory necrotizing myopathy and neuropathy with pipestem capillaries. Neurol India [serial online] 2015 [cited 2021 May 10 ];63:72-76
Available from: https://www.neurologyindia.com/article.asp?issn=0028-3886;year=2015;volume=63;issue=1;spage=72;epage=76;aulast=Patil;type=0