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Monogenic Autoimmune Diseases

Jeong DC

Monogenic autoimmune diseases (AD) present as lupus-like clinical manifestations with recurrent fever or various vasculopathies. Recurrent fever with an elevation of acute phase reactants and various skin lesions are similar...
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A Case of Rheumatoid Vasculitis Involving Hepatic Artery in Early Rheumatoid Arthritis

Lee JE, Kim IJ, Cho MS, Lee J

Rheumatoid vasculitis is a rare, but most serious extra-articular complications of long-standing, seropositive rheumatoid arthritis (RA). Vasculitis of hepatic artery is an extremely rare but severe manifestation of rheumatoid vasculitis....
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Dermatological Manifestations of Postural Tachycardia Syndrome Are Common and Diverse

Huang H, Deb A, Culbertson C, Morgenshtern K, DePold Hohler A

BACKGROUND AND PURPOSE: Postural tachycardia syndrome (POTS) is a syndrome of orthostatic intolerance in the setting of excessive tachycardia with orthostatic challenge, and these symptoms are relieved when recumbent. Apart...
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Gangrenous Cryoglobulinemic Vasculitis in a Patient with Multiple Myeloma

Ryu H, Park B, Moon JY, Lee MW, Choi YS, Song IC, Jo DY

  • KMID: 2267752
  • Korean J Med.
  • 2013 Dec;85(6):634-638.
We report a 62-year-old woman with multiple myeloma associated with cryoglobulinemia accompanied by gangrene of the digits. She presented with generalized purplish net-like discoloration (livedo reticularis), which was more prominent...
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Livedo Racemosa Associated with Epstein-Barr Virus-induced Post-transplant Lymphoproliferative Disorder

Kim TW, Mun JH, Jwa SW, Song M, Kim HS, Ko HC, Kim BS, Kim MB

  • KMID: 1427486
  • Korean J Dermatol.
  • 2013 Apr;51(4):261-264.
Livedo racemosa (LR) is characterized by a striking violaceous netlike patterning of the skin similar to the livedo reticularis, from which it differs by its localization (more generalized), and shape...
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Erythromelalgia and Livedo Reticularis in a Patient with Essential Thrombocythemia, Acquired von Willebrand Disease, and Elevated Anti-Phospholipid Antibodies

Gambichler T, Matip R

  • KMID: 2434691
  • Ann Dermatol.
  • 2012 May;24(2):214-217.
Essential thrombocythemia (ET) is a clonal stem cell disease characterized by isolated thrombocytosis and thrombohemorrhagic complications. We describe an unusual case of ET primarly presenting with skin symptoms including erythromelalgia...
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Blue Toe Syndrome: A Case Report

Kim HS, Kim CH

  • KMID: 2322458
  • J Korean Soc Plast Reconstr Surg.
  • 2011 Jul;38(4):508-511.
PURPOSE: Blue toe syndrome consists of blue or purplish toes in the absence of a history of obvious trauma, serious cold exposure, or disorders producing generalized cyanosis. It is a...
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A Case of Pulmonary Fibrosis with Microscopic Polyangiitis

Jeong JH, Kang SH, Park SJ, Kim DY, Kim WS, Kim DS, Song JW

A 65-year-old woman was admitted due to poor oral intake and a dry cough over the previous 3 months. The physical examination was remarkable for bibasilar crackles, and plain chest...
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Livedoid Vasculopathy Associated with Hepatitis C

Won JH, Yun SJ, Kim SJ, Lee SC, Won YH, Lee JB

  • KMID: 2301697
  • Korean J Dermatol.
  • 2009 Feb;47(2):239-241.
Livedoid vasculopathy is a chronic disease which appears with recurrent painful livedo reticularis patches and ulcers. The exact cause is unknown but it is known to be associated with some...
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A Case of Systemic Lupus Erythematosus and Secondary AntiphospholipidSyndrome Presenting as Livedo Reticularis

Kim JW, Oh DJ, Cho KJ, Hong SK, Lee D, Hwang SW, Park SW

  • KMID: 2302201
  • Korean J Dermatol.
  • 2007 Jun;45(6):622-625.
Antiphospholipid syndrome is defined as the presence of lupus anticoagulant antibodies or anticardiolipin antibodies with vascular thrombosis or specific pregnancy complications. Antiphospholipid syndrome can be associated with autoimmune, malignant or...
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Cerebral Venous Thrombosis and Livedo Reticularis in a Case with MTHFR 677TT Homozygote

Lee JY, Kim M

Hyperhomocysteinemia associated with methylene terahydrofolate reductase (MTHFR) mutation can be a risk factor for idiopathic cerebral venous thrombosis. We describe the first case of MTHFR 677TT homozygote with cerebral venous...
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Three Cases with Cutaneous Polyarteritis Nodosa

Bang KT, Herr H, Kim MJ, Lee BH, Bae KW, Kim YC, Chang HK

  • KMID: 2202338
  • J Korean Rheum Assoc.
  • 2004 Dec;11(4):447-452.
Systemic polyarteritis nodosa is a necrotizing vasculitis that involves small and medium-sized muscular arteries in the multiple organ systems, whereas cutaneous polyarteritis nodosa (CPAN) is a localized disease characterized by...
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A Case of Cutaneous Polyarteritis Nodosa

Kim CD, Kim BC, Lee KS

Cutaneous polyarteritis nodosa(CPAN) is a benign form of rare vasculitis of small and medium-size arteries with a recurrent but benign course without systemic involvement. We experienced a 61-year-old male who had...
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A Case of Rheumatoid Vasculitis

Han BG, Jung JH, Kim YC, Park HJ, Cinn YW, Pai HJ

  • KMID: 2250791
  • Korean J Dermatol.
  • 2002 Jul;40(7):829-831.
Rheumatoid vasculitis usually occurs in patients with long standing, seropositive, erosive rheumatoid arthritis. It involves typically small to medium-sized vessels and is associated with peripheral neuropathy, digital gangrene, nail fold...
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Two cases of severe cholesterol embolism after coronary angiography : Proven by skin biopsy

Jang SH, Jin SW, Park HC, Jang YS, Yoon HJ, Lee HK, Kim HY, Kim CJ, Park JC, Chae JS, Kim JH, Hong SJ, Choi KB

  • KMID: 2080864
  • Korean J Med.
  • 2002 Jul;63(1):79-84.
Cholesterol embolism is due to dislodgment of cholesterol crystals from the atheromatous aorta resulting in an occlusion of small arteries. Cholesterol emboli may occur spontaneously but usually develops as a...
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A Case of Sneddon's Syndrome

Lim TG, Lee SG, Lee SJ, Kim YK, Choi GS

  • KMID: 2251027
  • Korean J Dermatol.
  • 2001 Sep;39(9):1022-1024.
Sneddon's syndrome is an infrequent neurocutaneous disorder of unknown origin. It is characterized by the combination of livedo reticularis and cerebrovascular accident. We present a 57-year-old male patient with livedo...
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A case of primary antiphospholipid syndrome with renal microangiopathy

Rhee CH, Koh JH, Cha HS, Kim J, Huh W, Koh EM, Kim MK

  • KMID: 2143083
  • Korean J Med.
  • 2001 Jul;61(1):86-90.
Antiphospholipid syndrome is a disease that has continuous high titer of antibodies directed against either phospholipids or plasma proteins bound to anionic phospholipids in serum and shows a variety of...
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A Case of Relapsing Polychondritis Associated with Thrombophlebitis

Chung EC, Choi JH, Sung KJ, Moon KC, Koh JK

  • KMID: 2086301
  • Korean J Dermatol.
  • 2000 Jan;38(1):79-83.
Relapsing polychondritis is a rare systemic disorder characterized by recurrent inflammation of cartilaginous structures, the eye, the audiovestibular and cardiovascular systems. Skin lesions, found in 17% to 38% of cases,...
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Primary Antiphospholipid Antibody Sy n d rome with Adrenal Hemorrhage in a Child: A Case Report

Kim DH, Lee SH, Kim HJ, Yoo HW, Yoon CH

Primary antiphospholipid antibody syndrome is a disease that is clinically diagnosed if a patient suffers recurrent thromboses, stroke, recurrent fetal loss, livedo reticularis, and thrombocytopenia, without evidence of systemic lupus...
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A Case of Sneddon's Syndrome

Song JS, Oh YM, Lim SB, Kim JS

  • KMID: 1930652
  • Korean J Dermatol.
  • 1999 Oct;37(10):1518-1520.
Sneddon's syndrome is a disease characterized by livedo reticularis and cerebrovascular lesions with unknown etiopathogenesis. We report a 16-year-old male patient who had been suffered from livedo reticularis for 10...
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