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Sclerema Neonatorum

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.


Presentation

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].

Chills
  • Kellum et al. 17 argue against this hypothesis by stating that many more infants sustain major systemic stresses like prematurity, dehydration, shock, chilling, surgical procedures and major infections without developing SN.[nature.com]
  • Kellum et al . 17 argue against this hypothesis by stating that many more infants sustain major systemic stresses like prematurity, dehydration, shock, chilling, surgical procedures and major infections without developing SN.[researchgate.net]
Italian
  • […] introduced “Sclereme” for Endurcissement du tissue cellulaire in 1815 (6,7,8) Parrot called Endurcissement du tissue cellulaire, “Oedema neonatorum” and skin bound, “Sclerema neonatorum”in 1877 (7,8) In 1890, Ballantyne accepted the view of French and Italian[researchgate.net]
  • Neonatal anthropometric charts: The Italian neonatal study compared with other European studies. J. Pediatr. Gastroenterol. Nutr. 2010, 51, 353–361. [ Google Scholar ] [ CrossRef ] [ PubMed ] Moore, T.A.; Wilson, M.E.[mdpi.com]
Hypoxemia
  • Due to deposition of triglyceride crystals in the subcutaneous tissue, which occurs through still undisclosed mechanisms in sepsis, profound hypothermia, hypoxemia, acidosis, and hypocalcemia, neonates suffer from profound hardening of the skin that is[symptoma.com]
Multiple Congenital Anomalies
  • congenital anomalies 7 (39) Khetarpal and Subrahmanyam 15 (1964) 17 Most preterm 11:6 Within first 7 days of birth 15 had no complications Comorbidities: septicemia, jaundice 3 (17.6) Villacorte and Frank 3 (1967) 9 Most preterm 6:3 Within first 7 days[researchgate.net]
Asthmatic Bronchitis
  • Most preterm 11:6 Within first 7 days of birth 15 had no complications Comorbidities: septicemia, jaundice 3 (17.6) Villacorte and Frank 3 (1967) 9 Most preterm 6:3 Within first 7 days of birth Six had complications, including PPROM, cord around neck, asthmatic[researchgate.net]
Heart Disease
  • disease, respiratory distress, or severe dehydration.[medical-dictionary.thefreedictionary.com]
  • Sclerema appear frequently in premature newborn, with associated diseases as sepsis, congenital heart disease, respiratory distress.[accessanesthesiology.mhmedical.com]
  • ., pneumonitis, enteritis, intracranial haemorrhage or congenital heart disease, while in 5 of the reported cases, the mothers were ill during pregnancy, thus accounting for the fact that the majority of infants affected are debilitated or feeble at birth[medigoo.com]
  • It has mostly been reported in low birthweight, premature newborns in the setting of severe illness, including infections and heart disease 2.[ncbi.nlm.nih.gov]
Hyperpigmentation
  • Painful restricted movements of the extremities, hyperpigmentation over swollen joints, and a sclerema-like feel to the skin with increased serum triglyceride was seen in a 5-month-old baby with postnatal CMV infection.[pubfacts.com]
Breast Engorgement
  • engorgement of newborn P83.5 - Congenital hydrocele P83.6 - Umbilical polyp of newborn P83.8 - Other specified conditions of integument specific to fetus and newborn P83.9 - Condition of integument specific to fetus and newborn, unspecified Contact About[icd-code.org]
  • engorgement in newborn 778.8 - Other specified conditions involving the integument of fetus and newborn 778.9 - Unspecified condition involving the integument and temperature regulation of fetus and newborn Browse Medical Billing and Coding Databases[emedcodes.com]
  • engorgement in newborn 778.8 Other specified conditions involving the integument of fetus and newborn 778.9 Unspecified condition involving the integument and temperature regulation of fetus and newborn This page was last updated on: 10/1/2014[healthprovidersdata.com]
  • engorgement of newborn Inclusion term(s): Noninfective mastitis of newborn P83.5 Congenital hydrocele P83.6 Umbilical polyp of newborn P83.8 Other specified conditions of integument specific to newborn P83.81 Umbilical granuloma P83.88 Other specified[icd10coded.com]
  • engorgement of newborn P83.5 Congenital hydrocele P83.6 Umbilical polyp of newborn P83.8 Other specified conditions of integument specific to newborn P83.81 Umbilical granuloma Reimbursement claims with a date of service on or after October 1, 2015 require[icd10data.com]
Uremia
  • An unusual case of neonatal uremia, Arch, Dis, Chiirlh. 27 : 580 (1952). S. DJOJODIGUNO, S,T,S, : Sclerema Neonatorum, Paediat, Indon. 5: 28 (1965). 4.[paediatricaindonesiana.org]

Workup

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].

Treatment

  • Few clinical trials have been performed to address potential treatments. Successful treatment has been achieved via exchange transfusion (ET), but its use in neonates is declining.[ncbi.nlm.nih.gov]
  • The treatment measures may include blood transfusion, steroid therapy, and maintenance of fluid balance in the body However, despite treatment, the prognosis of Sclerema Neonatorum is poor and the condition often results in death of the infant Who gets[dovemed.com]
  • No effective treatment. Good. Spontaneous resolution in 3-5 years, leaving a brown pigmentation. Time is only treatment. Good; may be residual deformities.[kundoc.com]

Prognosis

  • Recent therapeutic modalities and the prognosis are discussed.[ncbi.nlm.nih.gov]
  • The prognosis is poor if it becomes generalized.[medical-dictionary.thefreedictionary.com]
  • Prognosis is poor. Minimal inflammation helps distinguish sclerema neonaturum from subcutaneous fat necrosis of the newborn. See also [ edit ] Panniculitis Skin lesion List of cutaneous conditions References [ edit ] William D. James, Timothy G.[en.wikipedia.org]

Etiology

  • Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology.[icd10coded.com]
  • This review attempts to put into perspective their similarities and differences in light of historical, biochemical, pathologic, and etiologic considerations. Recent therapeutic modalities and the prognosis are discussed.[ncbi.nlm.nih.gov]
  • Although etiology is uncertain, associations include hypoglycemia, sepsis, shock, hypothermia, metabolic abnormalities, and other serious underlying illnesses. Prognosis is poor.[visualdx.com]
  • Dehydration, weakness, low body temperature and acidosis have all been considered as etiological factors. Several theories have been proposed including low oleic acid content of fat plus a low body temperature.[medigoo.com]
  • Wegener granulomatosis - Autoimmune etiology and clinical course. Diabetes - disease and management information, including diagnosis, typical treatment plans and diabetes supplies.[netdoc.com]

Epidemiology

  • Epidemiology and predisposing factors for erythema toxicum neonatorum and transient neonatal pustular melanosis. A case of tetanus neonatorum unsuccessfully treated with tetanus antitoxin.[yogavanahill.com]
  • […] syndrome, congenital heart defects, gastroenteritis, intestinal obstruction, and severe malnutrition. [2, 5, 6, 7] Two case reports have described sclerema neonatorum that developed after therapeutic hypothermia initiated for neonatal asphyxia. [8, 9] Epidemiology[emedicine.medscape.com]
  • Epidemiological study on hypothermia in newborns. Chin Med J (Engl) . 1993;106:428–432. 18. Kellum RE, Ray TL, Brown GR. Sclerema neonatorum . Report of a case and analysis of subcutaneous and epidermal-dermal lipids by chromatographic methods.[journals.lww.com]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology In an infant, fat has a higher saturated-to-unsaturated fatty acid ratio compared to adult fat and thus, a higher melting point.[emedicine.medscape.com]
  • Diagnosis One of the problems in identiT h e pathophysiological occurfying sclerema is its similarity to rences in sclerema are somewhat other diseases of the integumentary obscure: histological studies show system.[kundoc.com]
  • Etiology Pathophysiology SN is likely a final common pathway of many different types of severe stresses leading to crystal formation and solidification of the saturated fats more prevalent in newborns.[clinicaladvisor.com]

Prevention

  • METHODS: Preterm neonates admitted to Dhaka Shishu Hospital in Bangladesh were enrolled in a clinical trial to evaluate the effects of topical treatment with skin barrier-enhancing emollients on prevention of sepsis and mortality.[jhu.pure.elsevier.com]
  • Your skin Holds body fluids in, preventing dehydration Keeps harmful microbes out, preventing infections Helps you feel things like heat, cold, and pain Keeps your body temperature even Makes vitamin D when the sun shines on it Anything that irritates[icdlist.com]
  • Although IVIG did not prevent our patient's death, further studies are needed to determine its clinical utility in the treatment of this rare disorder.[ncbi.nlm.nih.gov]
  • Although IVIG did not prevent our patient’s death, further studies are needed to determine its clinical utility in the treatment of this rare disorder.[mdedge.com]

References

Article

  1. Buster KJ, Burford HN, Stewart FA, Sellheyer K, Hughey LC. Sclerema neonatorum treated with intravenous immunoglobulin: a case report and review of treatments. Cutis. 2013;92(2):83-87.
  2. Zeb A, Darmstadt GL. Sclerema neonatorum: a review of nomenclature, clinical presentation, histological features, differential diagnoses and management. J Perinatol. 2008;28(7):453-460.
  3. Navarini‐Meury S, Schneider J, Bührer C. Sclerema neonatorum after therapeutic whole‐body hypothermia. Arch Dis Child Fetal Neonatal Ed. 2007;92(4):F307.
  4. Afroze F, Pietroni MAC, Chisti MJ. Recurrent Sclerema in a Young Infant Presenting with Severe Sepsis and Severe Pneumonia: An Uncommon but Extremely Life-threatening Condition. J Health Popul Nutr. 2013;31(4):538-542.

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Last updated: 2019-06-28 11:25