Nervous system involvement in Degos disease.

[malignant atrophic papulosis]

Degos disease , or malignant atrophic papulosis , is a rare obstructive vasculopathy of unknown origin , characterized by distinctive skin lesions , visceral involvement , and an unfavorable outcome . The gastrointestinal tract and the central nervous system are most frequently affected , but cases limited to benign skin lesions have also been described . Neuroradiologic reports of this condition are exceptionally rare . We report the case of a 29 -year -old woman with central and peripheral nervous system involvement who presented with progressive clinical deterioration and a meningovascular pattern at cerebral MR imaging ; other organs were spared in this patient .

Diseases
Validation

Diseases presenting "skin lesions" symptom

child syndrome

cowden syndrome

cutaneous mastocytosis

cystinuria

dystrophic epidermolysis bullosa

epidermolysis bullosa simplex

erdheim-chester disease

erythropoietic protoporphyria

familial mediterranean fever

focal myositis

gm1 gangliosidosis

heparin-induced thrombocytopenia

hirschsprung disease

junctional epidermolysis bullosa

lamellar ichthyosis

liposarcoma

lymphangioleiomyomatosis

malignant atrophic papulosis

oligodontia

omenn syndrome

papillon-lefèvre syndrome

primary effusion lymphoma

proteus syndrome

severe combined immunodeficiency

sneddon syndrome

waldenström macroglobulinemia

werner syndrome

wiskott-aldrich syndrome

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