Ann Dermatol.  2017 Apr;29(2):215-218. 10.5021/ad.2017.29.2.215.

Degos-Like Lesions Associated with Systemic Lupus Erythematosus

• Jang MS
• Park JB
• Yang MH
• Jang JY
• Kim JH
• Lee KH
• Kim GT
• Hwangbo H
• Suh KS

• KMID: 2394847
• DOI: http://doi.org/10.5021/ad.2017.29.2.215

Abstract

Degos disease, also referred to as malignant atrophic papulosis, was first described in 1941 by Köhlmeier and was independently described by Degos in 1942. Degos disease is characterized by diffuse, papular skin eruptions with porcelain-white centers and slightly raised erythematous telangiectatic rims associated with bowel infarction. Although the etiology of Degos disease is unknown, autoimmune diseases, coagulation disorders, and vasculitis have all been considered as underlying pathogenic mechanisms. Approximately 15% of Degos disease have a benign course limited to the skin and no history of gastrointestinal or central nervous system (CNS) involvement. A 29-year-old female with history of systemic lupus erythematosus (SLE) presented with a 2-year history of asymptomatic lesions on the dorsum of all fingers and both knees. The patient had only skin lesions and no gastrointestinal or CNS vasculitis symptoms. Her skin lesions were umbilicated, atrophic porcelain-white lesions with a rim of erythema. On the basis of clinical, histologic, and laboratory findings, a diagnosis of Degos-like lesions associated with SLE was made. The patient had been treated for SLE for 7 years. Her treatment regimen was maintained over a 2 month follow-up period, and the skin lesions improved slightly with no development of new lesions.

Keyword

Degos disease; Degos-like lesions; Systemic lupus erythematosus

MeSH Terms

Adult
Autoimmune Diseases
Central Nervous System
Diagnosis
Erythema
Female
Fingers
Follow-Up Studies
Humans
Infarction
Knee
Lupus Erythematosus, Systemic*
Malignant Atrophic Papulosis
Skin
Vasculitis
Vasculitis, Central Nervous System

Figure

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  Fig. 1 (A~C) Multiple erythematous papules with porcelain-white scars surrounded by an erythematous rim were seen on dorsum of all fingers and both knees.

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  Fig. 2 On dermoscopy, a central, whitish, structureless area surrounded by an erythematous rim is seen (×10).

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  Fig. 3 (A) Histologic findings showed hyperkeratosis, epidermal atrophy, and dermal sclerosis in the central portion (H&E, ×40). (B) Lymphocytic infiltration around vessels, fibrinoid necrosis of the vessel wall, and thrombus deposition in the lumen were seen (H&E, ×400).

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