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Malignant Atrophic Papulosis

Variously described as a vasculopathy, endovasculitis, or occlusive arteriopathy, this condition occurs in a benign cutaneous form and a lethal multiorgan systemic variant. It is characterized by a narrowing and occlusion of the lumen of small to medium-sized blood vessels, leading to ischemia and infarction in the involved organ systems. The etiology and pathophysiology are unknown.
Also Known As:
Degos Syndrome; Degos's Malignant Atrophic Papulosis; Erythrokeratoderma en cocardes; Papulosis, Malignant Atrophic; Atrophic Papuloses, Malignant; Atrophic Papulosis, Malignant; Disease, Degos; Disease, Kohlmeier-Degos; Malignant Atrophic Papuloses; Papuloses, Malignant Atrophic; Syndrome, Degos; Degos Disease; Kohlmeier-Degos Disease
Networked: 73 relevant articles (3 outcomes, 1 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Malignant Atrophic Papulosis
2. Cicatrix (Scar)
3. Dermatomyositis (Dermatopolymyositis)
4. Vascular System Injuries
5. Vasculitis (Vasculitides)

Experts

1. Magro, Cynthia M: 5 articles (11/2020 - 11/2002)
2. Shapiro, Lee: 3 articles (08/2022 - 04/2011)
3. Magro, Cynthia: 2 articles (08/2022 - 02/2020)
4. Zouboulis, Christos C: 2 articles (01/2022 - 01/2013)
5. Shapiro, Lee S: 2 articles (11/2013 - 04/2013)
6. Horai, Tetsuya: 1 article (04/2022)
7. Kitamura, Tadashi: 1 article (04/2022)
8. Miyaji, Kagami: 1 article (04/2022)
9. Tadokoro, Yuki: 1 article (04/2022)
10. Becker, Lena-Luise: 1 article (01/2022)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Malignant Atrophic Papulosis:
1. eculizumabFDA Link
2. treprostinilFDA Link
3. AntibodiesIBA
4. Dental Porcelain (Porcelain)IBA
5. Aspirin (Acetylsalicylic Acid)FDA LinkGeneric
6. Adrenal Cortex Hormones (Corticosteroids)IBA
7. Anticardiolipin AntibodiesIBA
8. Intravenous Immunoglobulins (IVIG)FDA Link
9. Lupus Coagulation Inhibitor (Lupus Anticoagulant)IBA
10. Proteins (Proteins, Gene)FDA Link

Therapies and Procedures

1. Therapeutics
2. Laparotomy
3. Immunomodulation
4. Secondary Prevention
5. Parathyroidectomy