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Year : 2019  |  Volume : 20  |  Issue : 4  |  Page : 302-305

Sclerema neonatorum


1 Department of Dermatology, Dr Ram Manohar Lohia Hospital and Post Graduate Institute of Medical Education and Research, New Delhi, India
2 Department of Paediatric, North DMC Medical College and Hindu Rao Hospital, New Delhi, India
3 Department of Dermatology, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India
4 Department of Neonatology, LNJP Hospital, New Delhi, India

Correspondence Address:
Dr Konchok Dorjay
Dr Ram Manohar Lohia Hospital and Post Graduate Institute of Medical Education and Research, New Delhi - 110 001
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.IJPD_12_18

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Sclerema neonatorum (SN) is a rare clinical condition usually seen during the 1st week of life. It presents as hardening of the skin and has a high mortality rate. It is usually associated with congenital anomalies, hypothermia, respiratory illnesses, and sepsis. Defective lipolytic enzymes, a high melting point, and resultant low solidification point of saturated fatty acids of subcutaneous fat, edema of connective tissue septae, and signs of underlying systemic diseases are the various theories proposed for the development of SN. The skin biopsy shows the thickening of trabeculae, sparse inflammatory infiltrates of lymphocytes, histiocytes and multinucleate giant cells, and X-ray diffraction of a biopsy sample of SN shows crystals in affected sites. The systemic corticosteroids have been used by various authors with variable response. Use of exchange transfusion has recently shown good results in improving the outcome of SN.


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