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Year : 2021  |  Volume : 2  |  Issue : 3  |  Page : 94-96

Sclerema neonatorum associated with early onset neonatal sepsis

1 Dr. NTR University of Health Sciences, Vijayawada, Andhra Pradesh, India
2 Department of Paediatrics, Government Medical College, Anantapur, Andhra Pradesh, India
3 Konaseema Institute of Medical Sciences and Research Foundation, Amalapuram, Andhra Pradesh, India

Date of Submission15-May-2021
Date of Decision18-Jun-2021
Date of Acceptance27-Jun-2021
Date of Web Publication29-Sep-2021

Correspondence Address:
Mr. Tarun Kumar Suvvari
Dr. NTR University of Health Sciences, Vijayawada, Andhra Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jopcs.jopcs_12_21

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Sclerema neonatorum (SN) is a rare condition with high mortality, usually prevalent in preterm newborns. SN is an infant panniculitis, in which adherence of skin, subcutaneous tissues with underlying muscles, and bones are seen. SN is more common among preterm newborns with high sepsis and the survival of the infants diagnosed with SN was very low. In this case report, we are presenting a case of SN in a preterm baby associated with early-onset neonatal sepsis.

Keywords: Atrial septal defect, early-onset neonatal sepsis, sclerema neonatorum

How to cite this article:
Suvvari TK, Salibindla DB, Narayana C S, Kutikuppala LV, Mantha K. Sclerema neonatorum associated with early onset neonatal sepsis. J Prim Care Spec 2021;2:94-6

How to cite this URL:
Suvvari TK, Salibindla DB, Narayana C S, Kutikuppala LV, Mantha K. Sclerema neonatorum associated with early onset neonatal sepsis. J Prim Care Spec [serial online] 2021 [cited 2023 May 28];2:94-6. Available from: https://www.jpcsonline.org/text.asp?2021/2/3/94/327052

  Introduction Top

Neonatal sepsis among newborns is a significant cause of mortality and morbidity. Early-onset neonatal sepsis (EOS) is defined as sepsis occurring before the first 3 days of life. It is caused by pathogens that are transmitted vertically from mother to infant before/during delivery. Late-onset neonatal sepsis is defined as sepsis occurring after 72 h in newborn intensive care unit (NICU) infants and after 7 days of birth in term infants, which are transmitted horizontally or vertically by pathogens.[1]

Sclerema neonatorum (SN) is a rare type of infant panniculitis, in which skin and subcutaneous adipose tissue harden with poor prognosis.[2] SN is generally seen in 1st week of preterm infants. In most cases, death occurs because of the hindrance of respiration and feeding due to adherence to skin and subcutaneous tissues with muscles and bone.[3]

  Case Report Top

A preterm (33 weeks) single tone live male baby was born by emergency lower segment cesarean section in view of oligohydramnios with fetal distress and born through clear liquor with a spontaneous cry at birth. The weight of the baby was 2.1 kg, as the baby has developed respiratory distress with grunting and was admitted in the NICU and supported by continuous positive airway pressure (CPAP). The baby was given empirical intravenous (IV) antibiotics along with IV fluids. The baby has improved clinically and was weaned from CPAP to nasal prongs on day 3 and day 4. Suddenly, the baby has a generalized tonic–clonic seizure and myoclonic jerks involving an upper limb, immediately IV fosphenytoin, and phenobarbitone were given along with inotropic support for the correction of shock. IV antibiotics upgraded to injection meropenem and amikacin.

On examination, the body temperature is normal, pulse was 132/min, breath rate was 52/min, and cyanosis was observed. The skin was unable to pinch and found attached to the underlying subcutaneous tissue, muscle, and bone. The laboratory findings showed high C-reactive protein (78.6 mg/l), leukocytosis (22,990 cells/mm3), neutrophilia (89%), thrombocytopenia (20,000/mm3), and lymphocytopenia (8%). There was metabolic acidosis with a compensatory respiratory alkalosis (low HCO3, PCO2, and BEecf). The So2 was 66%, BEecf was -11 mmol/L, and the serum total bilirubin was high (10.5 mg/ml). The 2D echocardiogram report showed small atrial septal defect (ASD) (4 mm) with the left-to-right shunt [Figure 1], mild tricuspid regurgitation, and pulmonary arterial hypertension. Neurosonogram was normal. The blood culture report showed Staphylococcus aureus isolated after 48 h of incubation. Antibiotic susceptibility test revealed resistance to ceftazidime, cefuroxime, oxacillin, and sensitive to chloramphenicol, rifampin, and gentamycin [Figure 2].
Figure 1: Two-dimensional echocardiogram showing the atrial septal defect

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Figure 2: Blood culture and antibiotic susceptibility test report

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Based on the above findings, the baby was diagnosed as EOS with ASD and SN.

The baby was continued in NICU and supported with CPAP. The baby had seizures again, so given injection phenobarbitone and later changed to oral syrup phenobarbitone. In view of severe sepsis, injection meropenem, teicoplanin, and colistimethate sodium was given for 21 days. Phototherapy was given for 6 days for neonatal jaundice. The baby received IV fluids later tube feeds were initiated on day 7 and gradually increased to full feeds on day 12. IV immunoglobins was given as treatment in view of severe sclerema.

The baby was discharged on the 23rd day and advised to continue the medications. The suggestion was given for mandatory breastfeeding, and immunization was given per schedule. The baby was reviewed after 7 days and followed up.

  Discussion Top

EOS is generally caused by organisms colonizing the maternal genitourinary tract by contaminating vaginal canal, cervix, placenta, and amniotic fluid. Hence the chances of acquiring the pathogen are high in either in utero or intrapartum, and risk factors for EOS include both maternal and infant factors.[1] Maternal risks include the intake of contaminated foods before labor and procedures during pregnancy such as amniocentesis, and cervical cerclage may also increase the rates of intra-amniotic infection, leading to neonatal sepsis. Maternal risk factors during the labor include fever, prolonged rupture of membranes, and vaginal colonization with Group B streptococcus.[2] Neonatal risk factors mainly include those with defective immunoregulatory genes, premature neonates, and other neonatal risk factors are male sex and low birth weight. Prevention is mainly through obstetric use of intrapartum antibiotic prophylaxis and antibiotics for Group B streptococcal infection. Breastfeeding is one of the natural preventive measures.[4]

SN is a rare disease characterized by firm, indurated, waxy skin lesions on buttocks, thighs, cheeks, deltoid, and scapular region. In most of the cases, fat-free soles, palms, and genitalia are spared. It was usually seen in the first few days after birth may be immediately postpartum and predominantly found in preterm males.[5] The main theory of pathogenesis involves the solidification of highly saturated neonatal fat with the fall of body temperature. Risk factors for developing SN were high septicemia, respiratory distress, neonatal jaundice, hypoglycemia, metabolic acidosis, respiratory alkalosis, hyperkalemia, hypocalcemia, and elevated blood urea. The mortality rate for SN was very high, ranging from 67% to 88%. There was no specific treatment for SN, systemic steroids, and exchange transfusions, and IV immunoglobin was considered the best treatment for SN.[6]

In the literature, not much cases on SN and EOS were reported; in our case, the patient was a preterm baby, and prognosis was low at the time of presentation. The key part of our case was treatment and management of SN. As mortality rate due to SN was high among neonates. Sepsis induced the sclerema and vigilant management is needed as both conditions need to be managed at the same time. A case report by Nakalema et al. reported SN in a term infant with similar presentations to our case and outcome of the patient of death.[7] Another case by Buster et al. reported SN in a preterm baby with similar clinical presentation, and they used the IV immunoglobin, but still the baby was died due to severe outcomes of the disease.[8] Hence, the chances of survival were low in preterm babies with SN and EOS.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

  Conclusion Top

SN is a rare disease usually seen in preterm neonates associated with high septicemia, and mortality is very high due to no specific treatment. In many cases, exchange transfusions and IV Ig showed a better prognosis, but it is not affordable/available all the time. Hence, more research studies are required for the treatment of SN.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Simonsen KA, Anderson-Berry AL, Delair SF, Davies HD. Early-onset neonatal sepsis. Clin Microbiol Rev 2014;27:21-47.  Back to cited text no. 1
Park SH, Kim SC. Sclerema neonatorum in a full-term infant showing favorable prognosis. Ann Dermatol 2017;29:790-3.  Back to cited text no. 2
Dorjay K, Dolker S, Arif T, Adil M, Ganju S. Sclerema neonatorum. Indian J Paediatr Dermatol 2019;20:302-5  Back to cited text no. 3
Kuzniewicz MW, Puopolo KM, Fischer A, Walsh EM, Li S, Newman TB, et al. A Quantitative, risk-based approach to the management of neonatal early-onset sepsis. JAMA Pediatr 2017;171:365-71.  Back to cited text no. 4
Zeb A, Darmstadt G. Sclerema neonatorum: A review of nomenclature, clinical presentation, histological features, differential diagnoses and management. J Perinatol 2008;28:453-60  Back to cited text no. 5
Hill IM. Sclerema neonatorum. Medscape Drugs Dis 2018. Available from: https://emedicine.medscape.com/article/1112191-overview. [Last accessed on 2021 Jun 16].  Back to cited text no. 6
Nakalema G, Egesa WI, Kumbakulu PK, Nduwimana M, Anaya AD, Kizito MS, et al. Sclerema neonatorum in a term infant: A case report and literature review. Case Rep Pediatr 2020;2020:8837064. doi: 10.1155/2020/8837064.  Back to cited text no. 7
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;92:83-7.  Back to cited text no. 8


  [Figure 1], [Figure 2]


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