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Degos syndrome (malignant atrophic papulosis; degos-delort-tricot syndrome; cutaneointestinal syndrome; kolmeier-degos syndrome)  349


Degos Syndrome (Malignant Atrophic Papulosis; Degos-Delort-Tricot Syndrome; Cutaneointestinal Syndrome; Kolmeier-Degos Syndrome)& 444b15e nbsp;   & 444b15e nbsp;   & 444b15e nbsp;   & 444b15e nbsp;   & 444b15e nbsp;   & 444b15e nbsp;   & 444b15e nbsp;   & 444b15e nbsp;   & 444b15e nbsp;   & 444b15e nbsp;   & 444b15e nbsp;   & 444b15e nbsp;   & 444b15e nbsp;   & 444b15e nbsp;   & 444b15e nbsp;   & 444b15e nbsp;   & 444b15e nbsp;   & 444b15e nbsp;   & 444b15e nbsp;   & 444b15e nbsp;   & 444b15e nbsp;   & 444b15e nbsp;   & 444b15e nbsp;   & 444b15e nbsp; 349

General: Rare cutaneovisceral disease; male preponderance; death occurs within a few months after diffuse eruption of skin lesions; multiple cerebral infarcts and/or thrombosis of small arteries; lymphocytic-mediated necrotizing vasculitis with mucin deposits in the dermis.

Ocular: Atrophic skin of eyelids; intermittent diplopia; conjunctiva may be atrophic; telangiectasias of conjunctiva with microaneurysms; peripheral choroiditis; papilledema has occurred with progressive central nervous system involvement; necrotic papules of lids, bulbar conjunctivae, and episcleral tissues.



Clinical: Porcelain-white skin lesions (asymptomatic and diffuse); anorexia and/or weight loss; gastrointestinal involvement; peritonitis; intermittent paresthesias with early central nervous system involvement; signs of progressive cerebral and cerebellar atrophy; peripheral telangiectatic rim.

Degos R, et al. Dermatite papulo-squameuse atrophiante. Bull Soc Fr Dermatol Syphil

Degos R. Malignant atrophic papuloses. Br J Dermatol

Demitsu T, et al. Malignant atrophic papulosis (Degos' syndrome). Int J Dermatol 1992; 31:99-l02.

Howard RO, et al. Malignant atrophic papulosis. Arch Ophthalmol 1968; 79:262.

Kohlmeier W Multiple Hautenekrosen bie Thromboangiitis Obliterans. Arch Dermatol Syphil l941; 181:783-792.

Leslie TA, et al. Degos disease and spastic paraplegia. Clin Exp Dermatol 1993; 18:344-346.

Premalatha S, et al. Malignant atrophic papulosis (Degos syndrome). First case report from India. Clin Exp Dermatol 1980; 5:370-371.




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