Dreaded Degos Disease in Childhood.

Degos disease or malignant atrophic papulosis is a rare multisystem vascular disease of unknown etiology most commonly affecting the small- to medium-sized arteries of the dermis of the skin, gastrointestinal tract, and central nervous system. Endothelial cell damage and the activation of the coagulation cascade leading to the obliterative thrombosis of the vessel wall are the main pathogenic mechanisms behind the disease. The characteristic cutaneous features are porcelain white atrophic papules with a peripheral rim of erythema seen commonly over the trunk, chest, and upper and lower limbs. In Degos disease, cutaneous and systemic manifestations typically do not present simultaneously, with skin lesions often appearing first. Systemic involvement, affecting organs such as the gastrointestinal tract or central nervous system, may develop months or years later. Patients should be closely monitored for potential systemic complications, as these significantly impact prognosis, necessitating multidisciplinary care to ensure early detection and management. The disease carries a poor prognosis because of systemic involvement. Degos disease is extremely rare in children with very few cases being reported in literature. We hereby report a rare case of Degos disease in a five-year-old female child with lesions confined to the skin with a lack of systemic involvement.