Dermatomyositis associated with generalized subcutaneous edema and Evans syndrome.
Although periorbital edema is a common manifestation of dermatomyositis (DM), generalized subcutaneous edema associated with DM is extremely rare. Evans syndrome is an autoimmune disease in which an individual's antibodies attack one's own red blood cells and platelets. Evans syndrome is rarely a presenting feature of DM. DM has been rarely reported to be associated with either generalized edema or Evans syndrome. We report the case of a 52-year-old Korean woman who presented with generalized subcutaneous edema, an erythematous rash, dysphagia, and proximal muscle weakness, and subsequently developed features of Evans syndrome. Treatment with high-dose glucocorticoids and an immunosuppressive agent controlled the DM, the generalized subcutaneous edema, and the Evans syndrome.
Department of Dermatology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea.
Kyu Dong
KD
Jung
Pyoung Su
PS
Kim
Hae Young
HY
Park
Cho Rok
CR
Kim
Ji Yeon
JY
Byun
Dong Youn
DY
Lee
Joo Heung
JH
Lee
Jun-Mo
JM
Yang
Eil-Soo
ES
Lee
D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications* / Q000175:diagnosis; D001379:Azathioprine / Q000627:therapeutic use; D003882:Dermatomyositis / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D004487:Edema / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007155:Immunologic Factors / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D018908:Muscle Weakness / Q000150:complications; D013921:Thrombocytopenia / Q000150:complications* / Q000175:diagnosis
D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D001379:Azathioprine; D008775:Methylprednisolone