Introduction:
Skin hardening might flash through as a familiar term to many of us, but this so-called ‘skin-bound disease’ takes a different turn from this thought. Sclerema neonatorum is a condition that causes generalized hardening of the skin due to inflammation of the subcutaneous fat (the layer present immediately beneath the skin, which is made up of fat) in newborn babies. This condition remains far from understanding and is hardly treatable and is mostly associated with a high mortality rate.
What Happens in Sclerema Neonatorum?
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The subcutaneous layer underneath the skin, predominantly made of fat, undergoes severe panniculitis inflammation.
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This inflammation causes the skin lining, along with the underlying fat, to harden.
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These hardened structures adhere to the underlying structures, like the bone and the muscle. Thus compromising the normal bodily functions of the baby.
What Is Panniculitis?
Panniculitis groups various conditions that involve inflammation of the subcutaneous layer. It usually presents as large bumps under the skin, causing inflammation. These bumps are painful when touched and commonly appear in the lower extremities. Though the cause varies, features are similar in almost every condition.
Who Is Prone to Sclerema Neonatorum?
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This condition is uncommon and usually seen in newborns who are weak by birth, preterm babies, those with low birth weight, and critically ill newborns due to underlying medical conditions.
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This condition commonly appears in the same week as the delivery. But it can also occur anytime, ranging from immediately after birth to two months or later.
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Certain studies show a higher prevalence of this disease in boys compared to girls.
What Are the Significant Features of Sclerema Neonatorum?
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It usually affects the areas with subcutaneous fat, such as the buttocks, thigh, trunk, scapular region, deltoid muscle areas (shoulder region), and cheek areas. It starts appearing on the buttocks and thighs and spreads to the other areas. The spread is as rapid as a day or two.
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This condition does not affect areas without subcutaneous fat, such as the palms, soles, and genitals.
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The skin is usually flat, stiff, cold, tense, and pale; it does not favor pinching or pulling and cannot be pitted.
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This condition usually does not show any edema.
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The newborns cannot flex the joints, including the knee, elbow, wrist, and hip joints.
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They also show a mask-like facial appearance due to their inability to open the mouth completely.
What Are the Symptoms of Sclerema Neonatorum?
The hardening of the skin and the underlying fat makes it hard for the baby to perform normal bodily functions like breathing, feeding, and movement, leading to the following symptoms;
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Breathing difficulty.
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Vomiting immediately after feeding.
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Lethargy.
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Temperature, pulse, and respiratory levels keep decreasing continuously.
What Are the Causes of Sclerema Neonatorum?
The cause of this disease remains unknown. It is said to be associated with the following conditions;
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Septicemia is a life-threatening complication in which the blood circulation is infected.
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Hypothermia (low body temperature).
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Hypocalcemia (low calcium levels).
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Any congenital anomalies.
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Respiratory and gastrointestinal complications.
What Is the Pathogenesis of Sclerema Neonatorum?
Based on the associated conditions, a few theories have been given as the possible causes of this disease.
In this first theory,
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It is stated that the levels of free fatty acids (released from the fat tissues) in the blood are low at birth and increase immediately after birth due to the possible mobility of the fatty acids from the subcutaneous fat into the blood.
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These are incorporated into triglycerides (a type of fat that constitutes the significant fat portion of the body) by the liver and help supply energy for metabolism and maintaining the body temperature.
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As the first theory suggests, any defect in this process could be the cause.
Another theory states that;
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The levels of saturated fats (fats that are said to be bad for health) are higher in neonates when compared to the levels in healthy adults.
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As these saturated fats have a greater tendency to harden when the temperature becomes low, it is considered one of the reasons for hardening.
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According to this theory, sclerema neonatorum occurs due to shock, which lowers the body temperature, causing skin hardening, failing proper circulation, and making the disease fatal.
However, this theory is questioned by a few researchers, as saturated fats can solidify only at temperatures below the freezing point.
What Are the Other Diseases Similar to Sclerema Neonatorum?
There are a few conditions that have features similar to this condition, such as;
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Subcutaneous fat necrosis of newborns (SCFN) only shows skin hardening localized to certain areas. It is usually associated with asphyxia (inability to breathe) or trauma during birth that differentiates it from this condition.
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Scleredema affects preterm babies but differs from sclerema neonatorum by its feature of having generalized firm pitting edema, which does not occur in the latter.
However, the above conditions heal spontaneously with treatment and are not fatal, like sclerema neonatorum.
Can Sclerema Neonatorum Be Treated?
Local therapies to keep the body warm did not show any results.
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The children should be given neonatal intensive care. The careful handling of newborns is mandatory as they are prone to septicemia (infection in the blood), the common cause of fatalities.
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Intravenous immunoglobulin (a separated byproduct of blood injected through veins) and exchange transfusion (replacing the baby's blood with fresh donor blood) have improved the condition, but their use in neonates has declined.
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Steroids are sometimes used as adjunctive therapy, which has shown a limitation in the spread of the hardening.
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Although intensive therapies are given, the prognosis for this condition is poor.
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Written consent is usually obtained from the parents before starting any treatment, as fatality is more common for this condition.
Conclusion:
Sclerema neonatorum is a rare condition in which everything from the cause to treatment is still a mystery. The condition is extremely rare and causes a severe complication in infants who are critically ill in their advanced phase of life. Though this condition has a high fatality rate of 98 percent, various treatment modalities have been tried, some of which have even shown a positive response, indicating light in the tunnel.
