Is Degos' disease a clinical and histological end point rather than a specific disease?

High, Whitney A.; Aranda, Jennifer; Patel, Samir; Cockerell, Clay J.; Costner, Melissa

doi:10.1016/j.jaad.2003.11.063

Cited by 67 publications

(57 citation statements)

References 33 publications

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“…It has been alternatively regarded as an obliterating arteriolitis, necrotizing vasculitis, endovasculitis with secondary thrombosis, intravascular coagulation disorder or a disorder of fibrinolysis [4] . The exact nature of the disease remains controversial.…”

Section: Discussionmentioning

confidence: 99%

A fatal case of Degos’ disease which presented with recurrent intestinal perforation

Ahmadi

Rafi²,

Faham³

et al. 2011

WJGS

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Degos' disease, otherwise known as "malignant atrophic papulosis" is a rare vasculopathy with an unknown etiology characterized by typical cutaneous lesions. Involvement of the gastrointestinal (GI) tract is observed in approximately half of patients and small infarctions in the mucosa can cause perforation and resulting peritonitis, the leading cause of death. We present a fatal case of Degos' disease with skin and GI involvement, manifesting as recurrent intestinal perforations and peritonitis, in a 15-year-old Iranian boy.

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Section: Discussionmentioning

confidence: 99%

A fatal case of Degos’ disease which presented with recurrent intestinal perforation

Ahmadi

Rafi²,

Faham³

et al. 2011

WJGS

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“…Although there is no specific therapy for MAP, cases have responded well to the use of antiplatelets drugs 8 . Heparin has been utilized in the acutely ill, but other anticoagulants, fibrinolytic and immunosuppressive agents have been tried without success 5,8 .…”

Section: Discussionmentioning

confidence: 99%

“…Heparin has been utilized in the acutely ill, but other anticoagulants, fibrinolytic and immunosuppressive agents have been tried without success 5,8 . Recently, a case report advocated the use of bevacizumab, a recombinant human monoclonal antibody, directed against the vas-…”

Section: Discussionmentioning

confidence: 99%

“…The disease limited to the skin may have a more benign course, but this is not the most common presentation 10 . Only about 15% of patients with MAP enjoy good health, with their lesions limited to the skin without any serious events in the gastrointestinal tract or CNS 4,8 . Furthermore, the disease is usually fatal within three years of the onset of systemic involvement 6 .…”

Section: Fig 1 [A] Abdominal Skin Lesions Characterized By Erythematmentioning

confidence: 99%

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Köhlmeier-Degos Disease (malignant atrophic papulosis) and Neurologic Involvement

Slaviero¹,

Annes

Frighetto³

et al. 2009

Arq. Neuro-Psiquiatr.

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“…6,[19][20][21][22][23] There is no established treatment protocol for Degos' disease and its management is determined by the clinical features and suspected cause. 24 Aspirin and dipyridamole treatment have been used with good results in many patients. 10,[25][26][27] Anticoagulation with heparin was reported to be effective for acutely ill patients, whereas coumadin and fi brinolytics have proven ineffective.…”

Section: Discussionmentioning

confidence: 99%

Degos’ disease (malignant atrophic papulosis) as a fatal cause of acute abdomen: Report of a case

et al. 2008

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Degos' disease, otherwise known as "malignant atrophic papulosis," is a rare condition characterized by typical cutaneous lesions. Its involvement of the gastrointestinal (GI) tract is usually associated with a poor prognosis. We report a case of Degos' disease with GI involvement, which ultimately caused peritonitis, sepsis, and death, despite all treatment measures. A 59-year old woman was admitted to our hospital with acute generalized abdominal pain. The patient had presented initially with multiple skin lesions 2 years earlier, and even with surgery for small-bowel perforation 10 months before this admission, Degos' disease had not been diagnosed. Explorative laparotomy revealed multifocal, ischemic changes in the small bowel with perforation in the mid-jejunum. After the operation, she suffered recurrent small-bowel fistulas and died within 3 months. In a patient with acute abdominal pain and typical atrophic papules, clinicians should retain a high index of suspicion for Degos' disease with GI involvement, even though it is rare.

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Is Degos' disease a clinical and histological end point rather than a specific disease?

Cited by 67 publications

References 33 publications

A fatal case of Degos’ disease which presented with recurrent intestinal perforation

A fatal case of Degos’ disease which presented with recurrent intestinal perforation

Köhlmeier-Degos Disease (malignant atrophic papulosis) and Neurologic Involvement

Degos’ disease (malignant atrophic papulosis) as a fatal cause of acute abdomen: Report of a case

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