Sclerema neonatorum

Abstract

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.

Keywords

• Exchange transfusion
• sclerema neonatorum
• subcutaneous fat