A special subtype of POEMS syndrome: IgG4 subtype.

Both POEMS syndrome and IgG4 related diseases are uncommon and clinical manifestations have been multifarious and nonspecific. There is still no literature describing the relationships between these two diseases. This study describes a case of a 33-year-old woman with POEMS syndrome, who had high serum IgG4 concentration and monoclonal IgG4 positive plasmacyte tissue infiltration. This is the first available report of the IgG4 subtype of POEMS syndrome. It is found that the best treatment is to find a novel therapy that targets the plasma cells directly.