@article { author = {Javinani, Ali and Gharibdoost, Farhad and Jamshidi, Ahmadreza and Kavosi, Hoda}, title = {Scleromyxedema associated with ankylosing spondylitis treated successfully with cyclosporine and high dose corticosteroids}, journal = {Rheumatology Research}, volume = {2}, number = {4}, pages = {145-149}, year = {2017}, publisher = {Rheumatology Research}, issn = {2476-5856}, eissn = {2476-5856}, doi = {10.22631/rr.2017.69997.1034}, abstract = {Scleromyxedema (SM) is a rare disorder which initially presents with waxy skin stiffness and maculopapular lesions. It also has non-dermatologic manifestations, such as serum paraproteinemia and myopathies, and is sometimes associated with other autoimmune disorders. A 52-year-old man was referred for treatment because of torso-dominant skin stiffness. He also had a neglected history of bilateral inflammatory back pain and chronic Helicobacter pylori-negative gastritis. Skin histopathology confirmed a diagnosis of SM. A grade 4 bilateral sacroilitis and a positive human leukocyte antigen B27 led to the patient also being diagnosed with ankylosing spondylitis (AS). Upon further analysis, monoclonal gammopathy of immunoglobulin (Ig) G/kappa (lower than 3 g/dl) and a normal percentage of plasma cells in his bone marrow aspiration sample were discovered. Due to the patient’s IgA deficiency, intravenous immunoglobulin (IVIG) could not be used to treat his SM; due to his positive tuberculin skin test (25 mm) and history of gastritis, anti-tumor necrosis factor alpha and non-steroid anti-inflammatory drugs were also withheld. The patient received a drug regimen of cyclosporine (3mg/kg/day) and high-dose prednisolone (0.5mg/kg/day) which successfully controlled both his SM and AS disorders. In this paper, we report a previously unreported case of SM-associated gammopathy and AS. We also show the efficacy of cyclosporine and high-dose prednisolone in the treatment of both of these conditions.}, keywords = {ankylosing spondylitis,cyclosporine,Prednisolone,scleromyxedema}, url = {https://www.rheumres.org/article_44446.html}, eprint = {https://www.rheumres.org/article_44446_89f9689c97daa051b284bb5a16399312.pdf} }