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Year : 2020  |  Volume : 9  |  Issue : 4  |  Page : 286-288

Pancytopenia with atypical skin manifestation in a term newborn

Department of Neonatology, PGIMER and Dr. RML Hospital, New Delhi, India

Correspondence Address:
Prof. Arti Maria
PGIMER and Dr. RML Hospital, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcn.JCN_109_19

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Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder in neonates characterized by hyperinflammation along with abnormal proliferation and accumulation of mononuclear cells within various tissues due to pathologic immune activation. Cutaneous manifestations in HLH are rare and have been reported between 6% and 65% of the cases in the literature. We describe the case of a term newborn with HLH and atypical skin manifestations. The baby was diagnosed as a case of primary HLH according to HLH-2004 guidelines based on typical clinical and laboratory findings. No specific genetic defect was identified. Subsequently, during hospital stay, the baby developed atypical skin lesions suggestive of “Noma Neonatorum.” He was managed aggressively with antibiotics as per the culture sensitivity report. However, the baby succumbed to rapidly progressive HLH. This is the first case report we are aware of “Noma Neonatorum” occurring in an infant with HLH. Although the cutaneous manifestations are not common, “Noma Neonatorum” should be kept in mind as an accompanying condition in neonates with HLH.

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