Sclerema neonatorum is an extremely rare type of panniculitis characterized by hardening of the skin and subcutaneous tissue in newborns, with skin hardening being severe enough to impair respiration and feeding. The diagnosis is made based on clinical findings and the presence of risk factors. High mortality rates are observed in the absence of adequate therapy.
Sclerema neonatorum is very rarely encountered in clinical practice. Reports have shown preterm newborns that suffer from respiratory distress or other delivery complications are at greatest risk for this rare form of panniculitis, whereas eclampsia has also been considered as a risk factor [1]. Symptoms most frequently appear during the first few days after birth but the period of symptom onset may range from immediately after birth to late neonatal period [2]. Due to deposition of triglyceride crystals in the subcutaneous tissue, which occurs through still undisclosed mechanisms in sepsis, profound hypothermia, hypoxemia, acidosis, and hypocalcemia [3], neonates suffer from profound hardening of the skin that is smooth, hard, and purplish in color [2]. The proximal parts of the lower limbs (buttocks or thighs) are sites where skin changes initially develop, but when such changes are seen on the thorax, breathing and feeding difficulties may ensue, as the skin cannot be picked up and is firmly bound to the subcutaneous tissue overlying the muscles and bones [2] [4]. Changes often spread to the entire body, sparing the fat-free soles, palms and genitalia [1], and mortality rates range from 30-100%, due to a progressive decline in body temperature, pulse and respiratory rate [2] [4].
The diagnosis of sclerema neonatorum should be easy to make based on clinical criteria and the observation of typical skin changes within the first few days or weeks of life. Having in mind this fact, a thorough but rapid examination of the newborn is the most important step in the workup. Furthermore, establishing risk factors can aid in solidifying the diagnosis of sclerema neonatorum as the underlying cause. Imaging or laboratory studies are used primarily for evaluation of conditions associated with this rare skin disorder. Chest X-rays or computed tomography (CT) are essential for the assessment of the cardiorespiratory system, whereas inflammatory markers, a complete blood count (CBC), and a full biochemical panel (bilirubin, liver and kidney function tests, etc.) are vital if valid clinical suspicion toward sepsis exists. Histopathological studies of the skin in sclerema neonatorum show completely normal epidermis and dermis, with broadened trabeculae and diminished fat spaces, and an inflammatory infiltrate without signs of necrosis[2]. However, the presence of needle-shaped crystals (formed from triglycerides, stearic and palmitic acids) in adipocytes is considered to be a hallmark of this condition [2].