Malignant atrophic papulosis

Abstract

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.