ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferencesView all Images (10)
Malignant atrophic papulosis - Skin
Print
Other Resources UpToDate PubMed

Malignant atrophic papulosis - Skin

Print Images (10)
Contributors: Alexis Perkins MD, Nikki Levin MD, Jeffrey D. Bernhard MD, Noah Craft MD, PhD, Lindy P. Fox MD, Lowell A. Goldsmith MD, MPH, Michael D. Tharp MD
Other Resources UpToDate PubMed

Synopsis

Malignant atrophic papulosis (MAP), or Degos disease, is a rare (200 reported cases) 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 three 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 the anti-phospholipid 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. 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 a vascular coagulopathy and/or 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

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

References

Subscription Required

Last Updated: 03/29/2017
Copyright © 2019 VisualDx®. All rights reserved.
Malignant atrophic papulosis - Skin
Print 10 Images
View all Images (10)
(with subscription)
Malignant atrophic papulosis : Abdominal pain, Dizziness/seizures, Headache, Nausea, Diplopia, Painful skin lesions, Sunken scar
Clinical image of Malignant atrophic papulosis
Copyright © 2019 VisualDx®. All rights reserved.