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Degos disease and spastic paraplegia
Author(s) -
LESLIE T. A.,
GOLDSMITH P. C.,
THOMPSON A. J.,
DOWD P. M.
Publication year - 1993
Publication title -
clinical and experimental dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.587
H-Index - 78
eISSN - 1365-2230
pISSN - 0307-6938
DOI - 10.1111/j.1365-2230.1993.tb02214.x
Subject(s) - pathognomonic , medicine , scars , gastrointestinal tract , dermatology , perforation , pathology , disease , materials science , punching , metallurgy
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.