Journal List > J Korean Rheum Assoc > v.14(3) > 1003569

J Korean Rheum Assoc. 2007 Sep;14(3):256-262. Korean.
Published online September 30, 2007.  https://doi.org/10.4078/jkra.2007.14.3.256
Copyright © 2007 The Korean Rheumatism Association
A Case of Systemic Lupus Erythematosus with Degos' Disease
Jung Hwa Lee, M.D., Hee Jung Ryu, M.D., Young Bae Park, M.D., Byoung Yong Choi, M.D., Eun Young Lee, M.D., Yun Jong Lee, M.D., Eun Bong Lee, M.D. and Yeong Wook Song, M.D.
Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea.
Received March 13, 2007; Accepted May 25, 2007.

Abstract

Degos' disease, malignant atrophic papulosis, is a rare disease characterized by a pathognomonic appearance of porcelain-white, atrophic papules with peripheral erythema and multiple occlusive thrombotic vascular pathology. It sometimes involves internal viscera such as the gastrointestinal tract, the central nervous system and kidney which may bring to death. Although several reports showed the associations between Degos' disease and antiphospholipid antibody or systemic lupus erythematosus, its pathogenetic significance remains elusive. The previous 4 Korean cases of Degos's disease involved only skin and/or the gastrointestinal tract. We report a case of systemic lupus erythematosus with Degos' disease involving skin, kidney and small intestine.

Keywords: Malignant atrophic papulosis; Degos' disease; Systemic lupus erythematosus; Antiphospholipid antibody

Figures


Fig. 1
Photographs of skin lesion. Multiple discrete papules are seen on both legs (A) and papules have white atrophic center with thin erythematous rim (B).
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Fig. 2
Histopathology of skin lesion and kideny. Biopsy specimen from papule in the trunk shows focal dermal sclerosis and perivascular lymphocyte infiltration (A, H&E ×400). A glomerulus reveals fibrillar appearance of mesangium and tubules reveal focal atrophy with infiltration of mononuclear cells in kidney biopsy specimen (B, H&E ×400).
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Fig. 3
Abdominal CT shows the wall thickening of peritoneum and small bowel with fluid collection around the small bowel, suggesting peritonitis.
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Fig. 4
Histopathology of small bowel. Small bowel biopsy specimen from segmental resection shows perivascular lymphocyte infiltration and narrowing of the lumen of small vessel by intimal proliferation (A, H&E ×400). Occlusion of the lumen of small vessel by thrombus is seen in this section through small bowel biopsy (B, H&E ×400).
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