Sclerema neonatorum

Hughes, Wilson E.; Hammond, Morton L.

doi:10.1016/s0022-3476(48)80224-6

Cited by 112 publications

(27 citation statements)

References 29 publications

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“…Defective mobilization of these fatty acids from the adipose tissues may result in their thickening. 14,17,21 This also explains why most of the neonates affected by SN have difficulty maintaining body temperature.…”

Section: Pathogenesismentioning

confidence: 99%

“…Hughes and Hammond support the idea that SN occurs under conditions of low peripheral body temperature as a consequence of circulatory collapse, for example, secondary to shock during an underlying grave illness. 14,20 Arguing against this theory, however, is the observation that hardening of subcutaneous fat does not occur until temperatures below freezing point are reached and even then the texture of the hardened skin is quite unlike that of SN. 8 Theory 2 advances that thickening of the subcutaneous layer in the neonate with SN could be due to their decreased ability to mobilize fatty acids from subcutaneous adipose tissue due to either a defect in adipose lipolytic enzymes or in one of the lipid transport mechanisms.…”

Section: Pathogenesismentioning

confidence: 99%

“…8 Theory 2 advances that thickening of the subcutaneous layer in the neonate with SN could be due to their decreased ability to mobilize fatty acids from subcutaneous adipose tissue due to either a defect in adipose lipolytic enzymes or in one of the lipid transport mechanisms. 14,17 Normally, the level of free fatty acids in serum is low at birth and starts to rise within hours after birth due to rapid mobilization of fatty acids from adipose tissues. These are incorporated into triglycerides by the liver and supply metabolic energy and thus contribute toward maintaining body temperature of the neonate.…”

Section: Pathogenesismentioning

confidence: 99%

“…6,15,22,23 Marks 24 has proposed steroids to be beneficial as adjunctive therapy while focusing on intensive therapy for sepsis and correction of fluid and electrolyte imbalance. Hughes and Hammond, 14 Warwick et al 4 and Villacorte and Frank 3 have emphasized the importance of treating the underlying disease.…”

Section: Managementmentioning

confidence: 99%

“…It is not the lowering of the core temperature but the peripheral temperature that predisposes to development of SN. 14 Ghosal and Nag Chaudhuri 12 reported a case series of 15 infants who developed sclerematous skin lesions while suffering from diarrhea. The onset of lesions was within 3 months, all had diarrhea, most had vomiting and dehydration and two survived.…”

Section: Managementmentioning

confidence: 99%

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

Zeb

Darmstadt

2008

J Perinatol

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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.

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Section: Pathogenesismentioning

confidence: 99%

Section: Pathogenesismentioning

confidence: 99%

Section: Pathogenesismentioning

confidence: 99%

Section: Managementmentioning

confidence: 99%

Section: Managementmentioning

confidence: 99%

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

Zeb

Darmstadt

2008

J Perinatol

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The Neonate

Atherton¹,

and²,

Cant³

2004

Rook's Textbook of Dermatology

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Panniculitis

Requena

2016

Rook's Textbook of Dermatology, Ninth Edition

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The panniculitides comprise a group of heterogeneous inflammatory diseases that involve the subcutaneous fat. Histopathological study is required for the specific diagnosis of these disorders, because different panniculitides usually show the same clinical appearance – which typically consists of erythematous nodules on the lower extremities. However, the histopathological study of panniculitis is difficult because of the poor correlation between clinical and pathological findings and the changing microscopic appearances as the lesions evolve. Furthermore, large scalpel incisional biopsies are required in order to evaluate panniculitis fully. From the histopathological point of view, all panniculitides are somewhat mixed, because the inflammatory infiltrate involves both the septa and lobules. However, differentiation between a predominantly septal and a predominantly lobular panniculitis is usually straightforward at scanning magnification on the basis of which subcutaneous structures are the more intensely involved by the inflammatory infiltrate.

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Sclerema neonatorum

Cited by 112 publications

References 29 publications

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

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

The Neonate

Panniculitis

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