Idiopathic hypocomplementemic urticarial vasculitis-linked neuropathy.
Hypocomplementemic urticarial vasculitis (HUV) is a rare form of cutaneous small-vessel vasculitis characterized by recurrent episodes of urticaria and painful, tender, burning or itchy skin lesions, often associated with extracutaneous involvement but usually with no significant peripheral nerve damage. We describe a patient with an HUV of undetermined cause that developed a progressive multifocal sensory neuropathy whose symptoms were temporarily relieved by intravenous immunoglobulin treatment. Sural nerve biopsy showed asymmetrical multifocal nerve fiber loss and axon degeneration in nerve fascicles, a picture suggestive of ischemic damage as a likely result of a vasculitic process. We point out that an axonal neuropathy may complicate idiopathic HUV and suggest looking for peripheral nerve involvement in HUV patients.
Filosto
Massimiliano
M
Clinical Neurology, Section for Neuromuscular Diseases and Neuropathies, University Hospital Spedali Civili, Brescia, Italy.
Cavallaro
Tiziana
T
Pasolini
Giorgio
G
Broglio
Laura
L
Tentorio
Marta
M
Cotelli
Mariasofia
M
Ferrari
Sergio
S
Padovani
Alessandro
A
D000893:Anti-Inflammatory Agents / Q000008:administration & dosage / Q000627:therapeutic use; D001369:Axons / Q000473:pathology; D001379:Azathioprine / Q000008:administration & dosage / Q000627:therapeutic use; D001706:Biopsy; D003165:Complement System Proteins / Q000172:deficiency*; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use; D008775:Methylprednisolone / Q000008:administration & dosage / Q000627:therapeutic use; D008875:Middle Aged; D010525:Peripheral Nerves / Q000098:blood supply* / Q000473:pathology; D010523:Peripheral Nervous System Diseases / Q000209:etiology* / Q000473:pathology; D013497:Sural Nerve / Q000473:pathology; D014581:Urticaria / Q000188:drug therapy / Q000209:etiology*; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D014855:Wallerian Degeneration / Q000209:etiology*
D000893:Anti-Inflammatory Agents; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D003165:Complement System Proteins; D001379:Azathioprine; D008775:Methylprednisolone