Looking for a Drop of Porcelain - Video

Dermatology described these as drops of porcelain suggestive of Degos disease, also known as malignant atrophic papulosis (MAP), which was confirmed by skin biopsy. He was started on treprostinil, however after progressive strokes and new encephalopathy, he was transitioned to palliative care. He died after 4 months from his presentation of gastrointestinal hemorrhage. His autopsy showed numerous cerebral strokes, mucin filled lesions on the bowel with perforation and hemorrhage, and hepatomegaly. This rare presentation of a child with cranial neuropathy, multifocal cerebral strokes, porcelain-like depressed infarcts of the skin, and gastrointestinal hemorrhage is classic for Degos disease. MAP is a multisystem obliterative vasculopathy first described in 1942 which has since been described in all ages from infants to adulthood 1,2 with a male predominance of 3:1. 3 Histology shows zones of skin necrosis due to vasculitis with little to no inflammatory reaction differentiating this from angiitis. Mucosal lesions including the bulbar conjunctivae have been reported. 2 The vasculopathy may be from excessive vascular C5b-9 deposition and a type I interferon rich environment, however the causative trigger (virus, autoimmune, genetic, anomalous fibrinolysis) is unknown. 4 There is currently no cure and with multisystem involvement it is fatal within 2 years often despite treatment with steroids, antiplatelet or antithrombin therapy, and biologics.