Malignant atrophic papulosis.

[malignant atrophic papulosis]

Malignant atrophic papulosis (MAP ; also known as Degos ' disease ) has a purely cutaneous variant and a systemic variant with cutaneous manifestations . Both have similar cutaneous eruptions . MAP manifests as erythematous , pink or red papules (2 -15 mm ), which evolve into scars with central , porcelain-white atrophic centres . Purely cutaneous MAP is a benign condition that can be life-long . Systemic MAP has a grim prognosis , but is not uniformly fatal . The cause of death is usually intestinal perforation . Death usually occurs within 2 -3 years from the onset of systemic involvement . Systemic MAP can involve the nervous , opthalmological , gastrointestinal , cardiothoracic and hepatorenal systems . No specific laboratory test can be used to aid in diagnosing MAP . Histopathologically , a wedge-shaped degeneration of collagen is present with a prominent interface reaction with squamatization of the dermoepidermal junction , melanin incontinence and epidermal atrophy . No treatment has been shown to be effective in the treatment of MAP .