Furkhunda Bahar, Arshalooz Rehman, Yasmeena Khan, K Z Hashmi.
Purpura Fulminans with Protein C Deficiency.
J Pak Assoc Derma Jan ;12(1):29-33.

A 4 months old boy, presented to our department with recurrent ecchymotic patches on different parts of the body since the age of 1112 month. He was diagnosed as neonatal purpura fulminans and the etiology came out to be protein C deficiency. He responded to our initial management and now he is on regular follow-up.

Case Report: A 4 months old boy was admitted in our hospital with history of recurrent ecchymotic patches on different parts of the body since the age of 1/2 month. He was a frill term baby, born after an uneventful pregnancy and uncomplicated delivery. He was the first offspring of his symptom free parents, who were first cousins. These lesions started as ecchymotic patches which within few hours turned deep purple areas and then progressed to necrosis followed by healing by scaring, the first episode was diagnosed as an allergic reaction and was treated by anti histamines by some local GP, after that patches faded off. The second episode was diagnosed as a case of DIC at a local hospital for which he was transfused FFP & packed red cell. At the age of 2 months the patient developed hydrocephalus. It was gradual in onset, A ventriculo-peritoneal shunt was placed in that hospital. Postoperative period was uncomplicated. At the time of admission to LNH he had blue areas with raised margins on the posterior aspect of left thigh & on abdomen. Baby appeared well and continued to feed well. Relevant laboratory values are given in the table. Blood samples were also taken for protein C & S levels and the patient was put on antibiotics & FFP & packed red cells were transfused. Protein S level was normal while protein C level was found to be 20% (Normal 70-140%). Family History: Parents were also checked for protein C & S levels. Both had normal levels of protein S. Protein C level was 70.80% in the mother and in father it was 50% (70- 140). Paternal grandmother had a history of swollen painful leg (?DVT). Father`s brother was married to mother`s sister. Their two sons died few days after birth with history of having necrotic patches, similar to the patient. On the basis of presenting complains, lab. findings and family history, we diagnosed the child as a case of purpura fulminans due to protein C deficiency. Treatment: After healing of lesions we heparinized the patient with the loading dose of 50mg/kg. It was maintained at the dose of 10 12 mg/kg/hour. Oral anticoagulant was then started with the dose of 0.2mg/kg.

PakMediNet -Pakistan's largest Database of Pakistani Medical Journals - http://www.pakmedinet.com