Degos disease with an intestinal perforation: a case report

Zhu, Ming; Jiang, Zhongmin; Jiang, Rihua; Chong, Xianling; Sun, Jing Ping

doi:10.1111/j.1365-4632.2012.05602.x

Cited by 6 publications

(2 citation statements)

References 7 publications

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“…Anti‐platelet monotherapies were the most and second most common treatments in BAP (19.3%, n = 26/135) and MAP (8.6%, n = 35/405), respectively, with CR rates of 42.3% (n = 11/26) and 20.0% (n = 7/35%). This is consistent with the coagulopathy aetiology hypothesis; 1 anti‐platelet therapies may combat the high platelet counts or platelet adhesiveness reported in many cases that can contribute to vascular thrombosis 1,54,63,64,104,190,210,251,255,256,270 . When combined with anti‐platelet combination therapies, all treatment regimens involving anti‐platelet therapies had CR and PR rates of 42.9% (n = 15/35) and 17.1% (n = 6/35) for BAP and 8.9% (n = 9/101) and 32.7% (n = 33/101) for MAP.…”

Section: Discussionsupporting

confidence: 82%

“…This is consistent with the coagulopathy aetiology hypothesis; 1 anti-platelet therapies may combat the high platelet counts or platelet adhesiveness reported in many cases that can contribute to vascular thrombosis. 1,54,63,64,104,190,210,251,255,256,270 When combined with antiplatelet combination therapies, all treatment regimens involving anti-platelet therapies had CR and PR rates of 42.9% (n = 15/ 35) and 17.1% (n = 6/35) for BAP and 8.9% (n = 9/101) and 32.7% (n = 33/101) for MAP. This significant decrease in CR rate in MAP but not BAP may suggest that anti-platelet monotherapies were more often prescribed for milder cases of the disease, while more complex cases received multiple therapies that were not able to achieve complete resolution.…”

Section: Treatments and Outcomesmentioning

confidence: 99%

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Clinical manifestations and treatment outcomes in degos disease: a systematic review

Kim

Kashetsky

Bagit

et al. 2021

Acad Dermatol Venereol

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Degos disease (atrophic papulosis) is a rare vasculopathy with cutaneous and systemic manifestations. Although potentially fatal, the characteristics of and treatments for Degos disease variants are not adequately described. We conducted a systematic review to summarize cutaneous and systemic presentations, treatments and outcomes of malignant (MAP) and benign (BAP) variants of Degos disease. A comprehensive search was conducted on Embase, MEDLINE, CINAHL and CENTRAL on 27 October 2020, which yielded 254 original studies reporting cases of Degos disease. A total of 357 patients were included in the analysis. Mean age of onset was 33.9 years. MAP was most commonly reported (63.8%, n = 228/357), with 56.6% (n = 129/228) mortality. Cutaneous lesions were usually asymptomatic (26.3%, n = 81/308) and localized to the trunk (57.7%, n = 206/357) and extremities (56.8%, n = 203/357). Systemic involvement developed within 2 years on average, ranging from 0 to 28 years. Anti-platelet monotherapy had a complete resolution rate of 42.3% (n = 11/26) in BAP and 20.0% (n = 7/35) in MAP. Based on the findings of the study, most cases of Degos disease are malignant with high mortality, and even benign cutaneous cases may develop systemic disease in as late as 28 years. Anti-platelet monotherapies may prove effective against both variants. Further studies are needed to confirm these findings.

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Section: Discussionsupporting

confidence: 82%