Degos disease


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ma·lig·nant a·troph·ic pa·pu·lo·sis

[MIM*602248]
a cutaneovisceral syndrome characterized by pathognomonic umbilicated porcelain-white papules with elevated telangiectatic anular borders, followed by the development of intestinal ulcers that perforate, causing peritonitis; arterioles in the lesions are occluded by thrombosis without inflammatory cells, leading to infarction, progressive neurological disability, and death.

malignant atrophic papulosis

A rare, often multisystem disease affecting small blood vessels of the skin, gastrointestinal and genitourinary tracts, central and peripheral nervous systems, heart, lungs, eyes, pancreas, adrenals, and kidneys, which is characterised by proliferation of the vascular intima and thrombosis, and infarction of the regions supplied by the involved arteries.

Clinical patterns
• Malignant systemic form—affects multiple organs and typically causes death within 2 years of presentation due to focal necrosis and perforation of the gastrointestinal tract.
• Benign cutaneous form—limited to the skin.

Management
MAP has been consistently resistant to treatments, including corticosteroids, MTX, sulfonamides, tacrolimus, warfarin, etc.

Degos disease

,

DD

A rare form of vasculitis that damages small blood vessels. The disease initially results in tissue infarction within the skin, but in some instances causes widespread and occasionally fatal tissue infarction in the gastrointestinal tract and other organs.
Synonym: malignant atrophic papulosis

Degos,

Robert, French dermatologist, 1904-1987.
Degos acanthoma - obsolete term for clear cell acanthoma.
Degos disease - a cutaneovisceral syndrome characterized by pathognomonic umbilicated porcelain-white papules followed by peritonitis, progressive neurological disability, and death. Synonym(s): Degos syndrome; malignant atrophic papulosis
Degos syndrome - Synonym(s): Degos disease
Kohlmeier-Degos syndrome - see under Kohlmeier