J Korean Rheum Assoc.  2007 Sep;14(3):256-262. 10.4078/jkra.2007.14.3.256.

A Case of Systemic Lupus Erythematosus with Degos' Disease

• Lee JH
• Ryu HJ
• Park YB
• Choi BY
• Lee EY
• Lee YJ
• Lee EB
• Song YW

• KMID: 2202175
• DOI: http://doi.org/10.4078/jkra.2007.14.3.256

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.

Keyword

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

MeSH Terms

Antibodies, Antiphospholipid
Central Nervous System
Erythema
Gastrointestinal Tract
Intestine, Small
Kidney
Lupus Erythematosus, Systemic*
Malignant Atrophic Papulosis
Pathology
Rare Diseases
Skin
Viscera
Antibodies, Antiphospholipid

Figure

• 

  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).

• 

  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).

• 

  Fig. 3. Abdominal CT shows the wall thickening of peritoneum and small bowel with fluid collection around the small bowel, suggesting peritonitis.

• 

  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 x400). Occlusion of the lumen of small vessel by thrombus is seen in this section through small bowel biopsy (B, H&E X400).

Reference

1). Degos R., Delort J., Tricot R. Papulose atrophiante maligne (syndrome cutaneo-intestinal). Bulletin et Memoires de la Societe Medicale des Hospitaux de Paris. 1948. 64:803.

2). Ball E., Newburger A., Ackerman AB. Degos'disease: a distinctive pattern of disease, chiefly of lupus erythematosus, and not a specific disease per se. Am J Dermatol. 2003. 25:308–20.

3). High WA., Aranda J., Patel SB., Cockerell CJ., Costner MI. Is Degos'disease a clinical and histological end point rather than a specific disease? J Am Acad Dermatol. 2004. 50:895–9.

4). 최규철: 황선욱: 김영표. 악성위축성구진증(Degos병) 의1 증례. 대한피부과학회지. 1981. 19:547–51.

5). 김형주: 박희용: 박윤기. 악성위축성구진증(Degos병) 1 예. 대한피부과학회지. 1986. 24:443–6.

6). 이성구: 임인석: 김철하: 이동근: 최응상: 유병훈등. 악성위축성구진증(Degos병) 1 례. 대한소아과학회지. 1991. 34:1724–9.

7). 성용기: 김선훈: 이유신. 악성위축성구진증(Degos병) 1 예. 대한피부과학회지. 1987. 25:443–6.

8). Degos R., Delort J., Tricot R. Dermatite papulosaua-meutse atrophiante. Bull Soc Fr Dermatitol Syphilir. 1942. 148–50.

9). Kohlmeier W. Multiple hausthekrosen bei thromboangiitis obliterans. Arch Dermatol Syphilol. 1941. 181:783–92.

10). Marginat G., Kerwin KS., Gabriel DA. The clinical manifestations of Degos syndrome. Arch Pathol Lab Med. 1989. 113:354–62.

11). Fernandez-Perez ER., Grabscheid E., Scheinfeld NS. A case of systemic malignant atrophic papulosis (Kohlmeier-Degos'disease). J Natl Med Assoc. 2005. 97:421–5.

12). Su WP., Schroeter AL., Lee DA., Hsu T., Muller SA. Clinical and histologic findings in Degos'syndrome. (Malignant atrophic papulosis). Cutis. 1985. 35:131–8.

13). Miyakis S., Lockhin ., Atsumi T., Branch DW., Brey RL., Cervera R, et al. International consensus statement on an update of the classification criteria for definite antiphopholipid syndrome (APS). J Thromb Haemost. 2006. 4:295–306.

14). Asherson RA., Cervera R., de Groot PG., Erkan D., Boff MC., Piette JC, et al. Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus. 2003. 12:530–4.

15). Sanchez-Guerrero J., Reyes E., Alarcon-Segovia D. Primary antiphospholipid syndrome as a cause of intestinal infarction. J Rheumatol. 1992. 19:623–5.