IVIg Successful in Scleredema

Eastham AB, Femia AN, Velez NF, et al. Paraproteinemia-Associated Scleredema Treated Successfully With Intravenous Immunoglobulin. JAMA Dermatol (2014). doi:10.1001/jamadermatol.2013.8835. May 21, 2014.

Intravenous immunoglobulin (IVIg) has successfully treated a case of scleredema associated with IgA-κ.

A woman in her 40s presented with a two-year history of progressive erythema and induration of the face, neck, and upper trunk. There was significant limitation of neck flexion, extension, and lateral rotation as well as shoulder abduction and internal rotation. She had no history of infection or diabetes mellitus.

Thalidomide and pegylated interferon were ineffective. Extracorporeal photophoresis was initially successful, but the response plateaued after a year. Neck and shoulder range of motion (ROM) became more restricted.

Based on two recent publications demonstrating the efficacy of IVIg for scleredema associated with diabetes and streptococcal infection, the authors initiated IVIg treatment at 2 g/kg over two consecutive days. After just two cycles, the patient noted significant improvement. After five cycles, her ROM continued to improve. However, IgA-κ levels did not decrease.

The case study does not discuss the cost of the treatment. A recent review in Current Opinion in Allergy and Clinical Immunology discusses why IVIG is so costly, adding that dosages are uncertain for off-label uses in part because  case studies of this kind are rarely followed by rigorous research that determines best practices.