Intractable skin necrosis and interstitial pneumonia in amyopathic dermatomyositis, successfully treated with cyclosporin A.
We report a patient with amyopathic dermatomyositis (DM) who mainly showed interstitial pneumonia and intractable skin necrosis in bilateral elbows and soles with a poor response to immunomediated therapy, including corticosteroid and high-dose intravenous immunoglobulin. Soon after starting oral cyclosporin A (CyA) the skin lesions healed completely and the interstitial pneumonia promptly improved in parallel with a decrease in serum KL-6. Since fatal interstitial pneumonia is frequently associated with amyopathic DM as in this case, administration of CyA should be actively considered as a therapeutic option when clinical symtoms are progressive and resistant to conventional treatments.
Shimojima
Yasuhiro
Y
Third Department of Medicine, Shinshu University School of Medicine, Matsumoto.
Ishii
Wataru
W
Kato
Takashi
T
Hoshi
Kenichi
K
Matsuda
Masayuki
M
Hashimoto
Takao
T
Tanaka
Yukio
Y
Ikeda
Shu-ichi
S
D000328:Adult; D016572:Cyclosporine / Q000627:therapeutic use*; D003882:Dermatomyositis / Q000150:complications* / Q000188:drug therapy*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D017563:Lung Diseases, Interstitial / Q000188:drug therapy* / Q000209:etiology*; D008297:Male; D009336:Necrosis; D012867:Skin / Q000473:pathology*
D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D016572:Cyclosporine