Neonatal Purpura Fulminans, a rare genetic disorder due to protein C deficiency: A case report

Autor: Sayyeda Ghazala, Irfan Kazi, Emaduddin, Siddiqui, Irfan, Habib, Saadia, Tabassum, Badar, Afzal, Irum Qamar, Khan
Rok vydání: 2018
Předmět:
Zdroj: JPMA. The Journal of the Pakistan Medical Association. 68(3)
ISSN: 0030-9982
Popis: Neonatal Purpura Fulminans is a rare and fatal disorder associated with perivascular haemorrhage and disseminated intravascular coagulation. Early clinical recognition, timely investigation and treatment is utmost important. A 6 days old baby boy was brought to emergency with blackish ulcers all over the body. Initially these were over the feet and scalp but later appeared on the abdomen. On examination, child was vitally stable, mildly icteric and had multiple erythematous large bullous blackish lesions on scalp, lower abdomen, perineum, back and soles. Neonatal reflexes and systemic examination was normal. Laboratory investigations showed normal CBC, PT/APTT and Protein S level while Protein C and Antithrombin III levels were low. Neonatal Purpura Fulminans is a life threatening condition and family screening is also mandatory for early recognition of disease in the siblings.
Databáze: OpenAIRE