Surgical Treatment in a Case of Neonatal Fulminant Purpura

Abstract

The literature is reviewed and a clinical case is presented in which a severe protein C deficiency was diagnosed and presented a flowery picture of the disease. The surgical treatment of the girl required the performance of gradual necrectomies, the amputation of the hand, the forearm of the right upper limb and some fingers and distal phalanges of the left hand, in addition to daily dressings with the application of temporary cutaneous covering of skin of lyophilized pig (heterograft-xenograft). Definitive closure of the lesions occurred by spontaneous epithelialization and the placement of living donor skin homografts obtained in this case from the father. The recovery of the grafted areas and of the limb amputations was favorable. Conclusion: Due to the severity and torpid evolution of the disease, at the age at which it appears that makes the small caliber and difficult access of the blood vessels produce the gradual loss of accesses for the administration of essential medications, to the serious consequences left in organs and systems due to thrombotic events and finally to the lack of early availability and high cost of Protein C Concentrate; the prognosis for this disease is extremely bleak, leading to death in the majority of neonates who suffer from it.