Sclerema neonatorum: a review of nomenclature, clinical presentation, histological features, differential diagnoses and management

Abstract

Objective: To review published literature on sclerema neonatorum (SN) in order to clarify its clinical presentation, histological features and management compared with two other diseases: subcutaneous fat necrosis of the newborn (SCFN) and scleredema.Study Design: PubMed database was searched using the key words Sclerema neonatorum. A total of 55 articles from peer-reviewed journals were reviewed and summarized.Result: SN, SCFN and scleredema are diseases of the subcutaneous adipose tissue. SN is characterized by hardening of the skin that gets bound down to the underlying muscle and bone, hindering respiration and feeding and is associated with congenital anomalies, cyanosis, respiratory illnesses and sepsis. Histology of the skin biopsy shows thickening of the trabeculae supporting the subcutaneous adipose tissue and a sparse inflammatory infiltrate of lymphocytes, histiocytes and multinucleate giant cells. SCFN has circumscribed hardening of skin on bony prominences with necrosis of adipocytes in subcutaneous tissue and a dense granulomatous infiltrate on histology. Scleredema is characterized by hardening of the skin along with edema; histology shows inflammatory infiltrate and edema in skin and subcutaneous tissues. SN has a high case fatality rate whereas SCFN and scleredema are self-limiting and lesions resolve within a few weeks to months. Exchange transfusion may improve survival in SN. Conclusion:The histological features of skin biopsy should be used to establish diagnosis of SN, SCFN and scleredema as disease-specific treatment is imperative in SN due to high fatality.

Neonatal hypothermia in low resource settings: a review

“…1 In severe cases, associated findings and sequelae include cold panniculitis, sclerema neonatorum and subcutaneous fat necrosis. 52 Cold panniculitis presents with circumscribed red lesions on the skin of infants and children who have been exposed to cold. These lesions disappear on their own after a period of weeks or months.…”

“…53 Sclerema neonatorum, a generalized waxy hardening of the skin, has been associated with hypothermia, and is considered a sign of severe underlying morbidity, including sepsis, cardiopulmonary disease, diarrhea or dehydration. 52,53 A report from China documented an incidence of sclerema of 6.7% among all infants born in the cold season in six middle-income rural and urban counties. 54 The case fatality rate reported for sclerema neonatorum is >50%.…”

Predictors of mortality in infants with sclerema presenting to the Centre for Diarrhoeal Disease, Dhaka

“…[1][2][3] It is characterised by deposition of crystals, primarily of triglycerides, in the subcutaneous fat as determined by X-ray diffraction. [2][3][4][5][6] Signs of sclerema are more frequently observed in neonates (sclerema neonatorum) but have also been reported in older infants.…”

Paniculitis como manifestación de síndrome febril prolongado en pediatría: Caso clínico

“…cording to etiology. Childhood panniculitis are described, such as neonatal subcutaneous fat necrosis, neonatal scleroderma, post-steroidal panniculitis and cold panniculitis [12][13][14][15][16] . There are very infrequent reports on pediatric presentations of panniculitis that are well described in adults, being the most frequent, the erythema nodosum, a septal panniculitis without vasculitis, clinically characterized as the appearance of inflammatory and painful cutaneous nodules that mainly compromise the pretibial region of the lower limbs, which usually solve spontaneously in 1 to 6 weeks, without ulceration or cutaneous atrophy.…”