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Degos-Like Lesions Associated with Systemic Lupus Erythematosus

View Article: PubMed Central - PubMed

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.

No MeSH data available.


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(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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Figure 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).

Mentions: A 29-year-old woman presented to our clinic with a two-year history of asymptomatic atrophic white lesions. Two years ago, the lesions began as pink dome-shaped papules on her dorsal fingers and knees bilaterally. These papules gradually turned into umbilicated, atrophic porcelain-white lesions with a rim of erythema. Seven years ago, the patient was diagnosed with SLE and was being treated in a rheumatology clinic. On physical examination, atrophic, porcelain-white scars surrounded by an erythematous rim were seen on the dorsum of all fingers and both knees (Fig. 1). The patient also had a malar rash and arthritis, but did not have symptoms of gastrointestinal tract involvement or central nervous system (CNS) vasculitis, such as blurred vision and hemiparesis. Her neurological exam was unremarkable. On dermoscopy, a central whitish, structureless area surrounded by an erythematous rim of short vessels was seen (Fig. 2). Laboratory testing was positive for anti-nuclear antibody (1:1,280), anti-ds-DNA antibody, anti-smith antibody, anti-Ro antibody, and anti-La antibody, but negative for anticardiolipin immunoglobulin (Ig)G, anticardiolipin IgM, and lupus anticoagulant. Histologic findings showed hyperkeratosis, epidermal atrophy, and dermal sclerosis in the central portion. It also showed lymphocytic infiltration around vessels, fibrinoid necrosis of the vessel wall, and thrombus deposition in the lumen (Fig. 3). On the basis of these clinical, dermoscopic, laboratory, and histologic findings, the diagnosis of Degos-like lesions associated with SLE was made. The patient was being treated with hydroxychloroquine (300 mg/day), prednisolone (8 mg/day), and cyclosporine (50 mg/day) for seven years, and beraprost (20 µg/day) and pentoxifylline (400 mg/day) for 2 years for SLE. Because the patient's skin lesions improved gradually during the two-month follow-up period, we continued her treatment as before without further intervention, and no new lesions developed.


Degos-Like Lesions Associated with Systemic Lupus Erythematosus
(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).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC5383749&req=5

Figure 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).
Mentions: A 29-year-old woman presented to our clinic with a two-year history of asymptomatic atrophic white lesions. Two years ago, the lesions began as pink dome-shaped papules on her dorsal fingers and knees bilaterally. These papules gradually turned into umbilicated, atrophic porcelain-white lesions with a rim of erythema. Seven years ago, the patient was diagnosed with SLE and was being treated in a rheumatology clinic. On physical examination, atrophic, porcelain-white scars surrounded by an erythematous rim were seen on the dorsum of all fingers and both knees (Fig. 1). The patient also had a malar rash and arthritis, but did not have symptoms of gastrointestinal tract involvement or central nervous system (CNS) vasculitis, such as blurred vision and hemiparesis. Her neurological exam was unremarkable. On dermoscopy, a central whitish, structureless area surrounded by an erythematous rim of short vessels was seen (Fig. 2). Laboratory testing was positive for anti-nuclear antibody (1:1,280), anti-ds-DNA antibody, anti-smith antibody, anti-Ro antibody, and anti-La antibody, but negative for anticardiolipin immunoglobulin (Ig)G, anticardiolipin IgM, and lupus anticoagulant. Histologic findings showed hyperkeratosis, epidermal atrophy, and dermal sclerosis in the central portion. It also showed lymphocytic infiltration around vessels, fibrinoid necrosis of the vessel wall, and thrombus deposition in the lumen (Fig. 3). On the basis of these clinical, dermoscopic, laboratory, and histologic findings, the diagnosis of Degos-like lesions associated with SLE was made. The patient was being treated with hydroxychloroquine (300 mg/day), prednisolone (8 mg/day), and cyclosporine (50 mg/day) for seven years, and beraprost (20 µg/day) and pentoxifylline (400 mg/day) for 2 years for SLE. Because the patient's skin lesions improved gradually during the two-month follow-up period, we continued her treatment as before without further intervention, and no new lesions developed.

View Article: PubMed Central - PubMed

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.

No MeSH data available.


Related in: MedlinePlus