A case of cutaneous sensory neuropathy associated with Churg-Strauss syndrome

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“…Multiplex mononeuritisis as acute neuropathy resembling Guillain Barré syndrome [6][7][8], may be the initial symptom of CSS, usually evolving in a distal sensorimotor polyneuropathy [5,9,10]. Cranial nerves neurop- athy is uncommon [8]. Studies of biopsied sural nerves indicate axonal degeneration as the major pathological process.…”

“…Studies of biopsied sural nerves indicate axonal degeneration as the major pathological process. Neurological symptoms can improve, but the sequelae of axonal damage may become persistent [8].…”

Neurological manifestations of Churg–Strauss syndrome

“…Neurological involvement occurs in 59–86% of the cases during the vasculitic phase with the peripheral nervous system (PNS) more frequently affected than the central nervous system (CNS) [2]. CSS responds favourably to steroids, cyclophosphamide, azathioprin, mycophenolate mofetil, or rituximab [3].…”

“…Peripheral nervous system manifestations occur in up to three quarters of the patients and include most frequently mononeuritis multiplex, followed by symmetrical polyneuropathy [2], cranial or peripheral mononeuropathy, cutaneous sensory neuropathy [3] or myositis (Table). In a single patient, the phrenic nerve was exclusively affected.…”

“…The characteristic axonal loss occurs in myelinated as well as unmyelinated fibres. Cutaneous sensory neuropathy is caused by multifocal inflammation of the small sensory nerves of the skin [3]. Particularly, polyneuropathy may be associated with a positive ANCA status [1].…”