Case report of Sleromyxedema with sensory motor axonal polyneuropathy treated with immunoglobulin therapy

Sleromyxedema is a rare disease characterised by extensive mucin deposition with fibrosis in dermis. It is often associated with monoclonal gammopathy. Its true prevalence and/or cause is unknown due to its rarity. Various extra-cutaneous manifestations can be associated with sleromyxedema such as dermoneuro syndrome, neuropathy, rheumatological problems or various malignancies. This case report showed that a patient with sleromyxedema and neuropathy had positive outcomes when treated with intravenous immunoglobulin (IVIg) and steroids pulses. The underlying cause of the patient’s condition was unknown.


Keywords: Sleromyxedema, Immunoglobulin therapy, Dermoneuro syndrome, Sensory Motor Axonal Polyneuropathy, Monoclonal Gannopathey