Irfan Kazi Sayyeda Ghazala, Siddiqui Emaduddin, Habib Irfan, Tabassum Saadia, Afzal Badar, Khan Irum Qamar
The Aga Khan University Hospital.
National Institute of Child Health, Child Life Foundation, Karachi, Pakistan.
J Pak Med Assoc. 2018 Mar;68(3):463-465.
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.
新生儿暴发性紫癜是一种罕见的致命性疾病，与血管周围出血和弥散性血管内凝血有关。早期临床识别、及时检查和治疗至关重要。一名6天大的男婴因全身出现黑色溃疡被送往急诊。最初这些溃疡出现在足部和头皮，后来出现在腹部。检查时，患儿生命体征稳定，轻度黄疸，头皮、下腹部、会阴、背部和足底有多个红斑性大疱性黑色病变。新生儿反射和全身检查正常。实验室检查显示血常规、凝血酶原时间/活化部分凝血活酶时间和蛋白S水平正常，而蛋白C和抗凝血酶III水平较低。新生儿暴发性紫癜是一种危及生命的疾病，对家庭成员进行筛查对于早期识别其兄弟姐妹中的疾病也很有必要。
