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Malignant atrophic papulosis
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Malignant atrophic papulosis

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Contributors: Alexis Perkins MD, Nikki Levin MD, Jeffrey D. Bernhard MD
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Synopsis

Malignant atrophic papulosis (MAP), or Degos disease, is a rare primary vaso-occlusive disorder presenting on the trunk or extremities as rose-colored papules that become umbilicated, healing with a porcelain white center with surrounding telangiectasia.

The cutaneous and systemic manifestations of MAP may cause significant pain.

The disease is thought to exist in 3 forms. The classic systemic form affects the skin first and later may involve the eyes, gastrointestinal tract, heart, and central nervous system (CNS), generally resulting in death within several years because of sepsis from peritonitis (61%), CNS bleeding (18%), and pleural or pericardial involvement (16%). A benign, purely cutaneous form is seen in up to 15% of patients and is defined by no signs of systemic involvement within 3 years of diagnosis. Finally, MAP may be seen in association with connective tissue diseases such as antiphospholipid syndrome, lupus erythematosus, dermatomyositis, and systemic sclerosis.

MAP typically occurs between the second and fourth decades of life. Cases of MAP have been reported in teenagers and children. The incidence in men may be greater than in women. Although most cases are sporadic, there are reports of familial cases.

The pathogenesis of MAP is unknown, although vascular coagulopathy and vasculopathy are possible pathogenic mechanisms. Other theories implicate a viral or autoimmune pathogenesis. A combination of prothrombotic factors possibly plays a role; however, in studies of MAP patients, no single abnormality was repeatedly identified.

For more information, see OMIM.

Codes

ICD10CM:
I77.89 – Other specified disorders of arteries and arterioles

SNOMEDCT:
400171002 – Malignant atrophic papulosis

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Last Updated: 11/18/2019
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Malignant atrophic papulosis
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Malignant atrophic papulosis : Abdominal pain, Dizziness/seizures, Headache, Nausea, Diplopia, Painful skin lesions, Sunken scar
Clinical image of Malignant atrophic papulosis
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