An unusual case of inflammatory necrotizing myopathy and neuropathy with pipestem capillaries.
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'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.
Anil Kumar B
AK
Patil
A T
AT
Prabhakar
Ajith
A
Sivadasan
Department of Neurological Sciences, Christian Medical College, Vellore, Tamil Nadu, India.
Mathew
M
Alexander
Geeta
G
Chacko
D000328:Adult; D002196:Capillaries / Q000473:pathology*; D003402:Creatine Kinase / Q000097:blood; D003520:Cyclophosphamide / Q000627:therapeutic use; D003882:Dermatomyositis / Q000175:diagnosis* / Q000628:therapy; D005260:Female; D006801:Humans; D007136:Immunoglobulins / Q000627:therapeutic use; D007249:Inflammation / Q000175:diagnosis / Q000628:therapy; D008775:Methylprednisolone / Q000627:therapeutic use; D018482:Muscle, Skeletal / Q000098:blood supply / Q000473:pathology; D009336:Necrosis / Q000175:diagnosis / Q000628:therapy; D010525:Peripheral Nerves / Q000473:pathology*; D011928:Raynaud Disease / Q000175:diagnosis / Q000628:therapy
D007136:Immunoglobulins; D003520:Cyclophosphamide; D003402:Creatine Kinase; D008775:Methylprednisolone