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Mixed Connective Tissue Disease Associated with Skin Defects of Livedoid Vasculitis
Authors:Y. B. Oh  J.-B. Jun  C. K. Kim  C. W. Lee  C. K. Park  T.-Y. Kim  D.-H. Yoo  S. Y. Kim
Affiliation:(1) Department of Rheumatic diseases, Tong-inchon Ghil Hospital, Inchon, KR;(2) Department of Internal Medicine, Hanyang University College of Medicine, Seoul, KR;(3) Department of Rheumatology, Dong-eui Medical Center, Pusan, Korea, KR;(4) Department of Dermatology, Hanyang University College of Medicine, Seoul, KR;(5) Department of Pathology, Hanyang University College of Medicine, Seoul, KR;(6) Department of Clinical Pathology, Hanyang University College of Medicine, Seoul, KR
Abstract:
A 21-year-old woman who had a 2-year history of mixed connective tissue disease (MCTD) developed rapidly evolving ulcers consistent with livedoid vasculitis (LV) in all distal extremities. She presented clinically with Raynaud’s phenomenon, polyarthritis and swollen hands; serologically with high titres of ANA and anti-nRNP; and immunogenetically with HLA-DR4 and HLA-DR53. Although there was initial success in treatment except for the skin defects over the ankles, the patient died from disseminated intravascular coagulation. We suggest that LV may be a poor prognostic manifestation in MCTD. Received: 9 August 1999 / Accepted: 25 January 2000
Keywords:: Livedoid vasculitis –   Mixed connective tissue disease
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