Sclerema adiposum neonatorum P83.0

Uzembenzius, 1772; Underwood, 1784; Underwood described the disease as "skinbound".

Rare, diffuse, indurating panniculitis of immature newborns, usually occurring secondarily as a symptom of a life-threatening underlying disease.

Unexplained. Predisposing problem births, inadequate nutrition, infections, diarrhea, cooling (observed after therapeutic hypothermia) are discussed.

Occurs from birth, especially between the 2nd and 10th day of life, also in older children with severe underlying diseases. There is a slight predominance of males.

Previous case reports and case series show that the disease usually affects premature infants within the first week of life. It may be associated with other neonatal comorbidities such as sepsis, hypothermia, hypocalcemia and congenital anomalies. The mother may experience complications such as pre-eclampsia, eclampsia, prolonged rupture of membranes (PROM), placenta previa and hyperpyrexia.

Leather-like hardened, waxy pale, cold, possibly also cyanotic, non-liftable skin and subcutis. No indentations can be made. Mask-like rigid face. The hardening process usually begins on the buttocks, thighs or trunk and then spreads diffusely to other parts of the body. Fat-free areas such as the palms of the hands, soles of the feet and genitals are usually spared. Movement, food intake and breathing are impaired in such cases due to the hardening. The mobility of the joints is also restricted. Frequently reduced body temperature.

Treatment of SN includes intensive therapy of the underlying sepsis and correction of the fluid and electrolyte imbalance. Despite aggressive treatment, the prognosis is usually poor. Steroids can be used as adjunctive therapy. The use of steroids alone has not improved survival but may limit the spread of skin induration in sclerema neonatorum. ACTH or hydrocortisone or a combination of both have been used in sclerema neonatorum with mixed results.

Similarly, IVIG and exchange transfusions (ET) have been used to improve the outcome of SN. ET has led to better outcomes in neonates with SN. ET increases immunoglobulin and complement levels and improves survival, particularly in preterm infants. IVIG also improves humoral and cellular immunity, but has not improved survival in SN.

Sclerema neonatorum usually affects premature babies with a generalized hardening of the skin. It is associated with sepsis and the prognosis is usually poor.

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