A CASE REPORT ON CONGENITAL PROTEIN S DEFICIENCY PRESENTING AS NEONATAL PURPURA FULMINANS
Journal Title: Journal of Evidence Based Medicine and Healthcare - Year 2018, Vol 5, Issue 48
Abstract
PRESENTATION OF CASE A newborn delivered at 34 weeks of gestation was brought at 20 hrs. of life in view of development of 2 large ecchymotic patches over lower limbs. The newborn was preterm 2.2kgs and was delivered by LSCS in view of oligohydramnios, and born to third degree consanguineous marriage, no significant family history of similar complaints were noted. The neonate cried immediately after birth and lesions started appearing around 2hrs. of life. Lesions were sudden in onset purplish black in colour, surrounded by erythematous halo. On examination, the lesions were extensive and spread across upper and lower limbs especially the dependent and weight bearing areas. The lesions began as purplish discoloration followed by necrosis of the underlying area and finally presenting as bleeding haemorrhagic areas. The ecchymotic lesions progressed over a period of few days into sharply demarcated necrotic patches, which finally got shed leaving behind an ulcer base. The neonate was screened for congenital anomalies, 2d-echo was normal, no external vertebral or neural tube defects seen. The neonate was fed through NGT feeds and passed stools and urine within first 6 hrs. of life. However, the urine was dark cola coloured and stools were black-brown in colour suggesting disseminated intravascular coagulation. Systemic screening however did not reveal any external symptoms and signs, yet the neonate was evaluated for ophthalmic examination and MRI brain to note down any internal bleeding. An elder sibling of the propositus developed similar lesions but more extensive and succumbed to death on the seventh day of her life, as per the history from parents and there was no conclusive evidence as the sibling was not investigated. Lab reports revealed and elevated PT, APTT and ddimer and decreased fibrinogen levels, thus resembling a picture of DIC. Septic screen was negative.
Authors and Affiliations
Morishetty Arjun Kumar, Koneru Vamsi Krishna, Shailaja Mane, Sharad Agarkhedkar, Sanket Nale
Keywords
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- EP ID EP607577
- DOI 10.18410/jebmh/2018/679
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