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

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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.