Degos disease and spastic paraplegia

Summary Malignant atrophic papulosis (Degos disease) is a rare disorder characterized by a vasculopathy of unknown origin. 1 The cutaneous manifestations comprise erythematous papules, which heal to leave scars with a pathognomonic central porcelain‐white atrophic area and a peripheral telangiectati...

Full description

Saved in:
Bibliographic Details
Published in:Clinical and experimental dermatology 1993-07, Vol.18 (4), p.344-346
Main Authors: LESLIE, T. A., GOLDSMITH, P. C., THOMPSON, A. J., DOWD, P. M.
Format: Article
Language:English
Subjects:
Atrophy
Biological and medical sciences
Dermatology
Erythema - pathology
Female
Humans
Medical sciences
Middle Aged
Paraplegia - etiology
Paraplegia - pathology
Skin - pathology
Skin Diseases, Vascular - complications
Skin Diseases, Vascular - pathology
Skin involvement in other diseases. Miscellaneous. General aspects
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Summary Malignant atrophic papulosis (Degos disease) is a rare disorder characterized by a vasculopathy of unknown origin. 1 The cutaneous manifestations comprise erythematous papules, which heal to leave scars with a pathognomonic central porcelain‐white atrophic area and a peripheral telangiectatic rim. There is usually involvement of the gastrointestinal tract but other organ systems can also be affected, the central nervous system being involved in 20% of cases. 2 It is frequently fatal within 2 or 3 years from onset of systemic involvement, the cause of death usually being intestinal perforation. 3 Our patient is of interest as she has survived an unusually long time despite florid cutaneous and neurological manifestations.
ISSN:0307-6938
1365-2230
DOI:10.1111/j.1365-2230.1993.tb02214.x