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].

• Sepsis

  Cultures of blood and cerebrospinal fluid were obtained in all neonates with clinical suspicion of sepsis. [jhu.pure.elsevier.com]

  Optimal Therapeutic Approach for this Disease Finding the source of the sepsis or other insult is key, and then supportive care is indicated. [neurologyadvisor.com]

  This could be linked to the high rate of sepsis in these neonates, as neonatal sepsis is fatal when associated with the condition. 4,15,16 This makes prompt diagnosis and treatment of this disease, including underlying sepsis, imperative to save the lives [journals.lww.com]

  Suscepdefense for the infant against sepsis. [kundoc.com]

• Surgical Procedure

  Exclusion criteria included neonates with congenital anomalies or hydrops fetalis, those admitted to the hospital for a major surgical procedure or who had a clinically evident skin infection confirmed by surface culture, neonates with a generalized skin [journals.lww.com]

  Kellum et al (1968) argues against this theory: Many infants sustain major systemic stresses, surgical procedures and major infections without developing sclerema neonatorum. [researchgate.net]

  Immaturity of the neonatal lipoenzymes is further compromised by hypothermia, infection, shock, dehydration, and surgical and environmental stresses. [clinicalgate.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. [nature.com]

• Pediatric Disease

  Written with more than 425 chapters, the book will encompass virtually all pediatric subspecialties, covering every pediatric disease and organ system. [books.google.com]

• Dyspnea

  Neonatal scleredema 16. multiple organ dysfunction bradycardia, dyspnea, microcirculation disturbance, shock, DIC, acute renal failure, pulmonary hemorrhage 17. [slideshare.net]

• Vomiting

  Parents should be warned of the early signs of hypercalcemia such as lethargy, irritability, hypotonia, vomiting, polyuria, polydipsia, and dehydration. Analgesia is given if the lesion is painful. [dailyrounds.org]

  Vomiting after feeding and mild respiratory difficulty accompanied the skin symptoms. Waxy, indurated skin with erythema was observed on the whole body, sparing the face, both nipples, and the genital area (Fig. 1). [ncbi.nlm.nih.gov]

• 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]

  Sclerema neonatorum appears frequently in premature newborn, with associated diseases as sepsis, congenital heart disease, and respiratory distress. [accesspediatrics.mhmedical.com]

  The affected infants often have other associated diseases, e.g., 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 [medigoo.com]

• Erythema

  SYMPTOMS Erythema toxicum neonatorum (toxic erythema of the newborn, toxic erythema) Transient neonatal pustular melanosis (lentigenes neonatorum) Subcutaneous fat necrosis of the newborn. [yogavanahill.com]

  NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases Acrodermatitis Cradle cap Cryotherapy Cutaneous skin tags Dry skin Dry skin -- self-care Epidermolysis bullosa Erythema multiforme Erythema nodosum Erythema toxicum Granuloma annulare [icdlist.com]

  Erythema nodosum in children: a prospective study. [scielo.br]

  Code: P830 Description: Sclerema neonatorum Chapter: Certain conditions originating in the perinatal period Blocks Conditions involving the integument and temperature regulation of fetus and newborn Similars P83.0 - Sclerema neonatorum P83.1 - Neonatal erythema [icd-code.org]

• Skin Disease

  Chapter 17 Nevi and Skin Tumors 349 Chapter 18 Cutaneous Manifestations of Internal Diseases 381 Chapter 19 Treatment of Skin Diseases 397 [books.google.de]

  Skin Disease Dynamics Get All The Support And Guidance You Need To Be A Success At Having Better Skin. This Book Is One Of The Most Valuable Resources In The World When It Comes To Important Info On Dealing With Skin Disease. Get My Free Ebook [myfertilityguide.biz]

  The skin becomes so tense that the infant cannot move. Most infants with this skin disease do not survive unless the underlying internal abnormalities resolve. [perridermatology.com]

• Dermatitis

  Pityriasis rosea Pityriasis rubra pilaris Pyogenic granuloma Sebaceous cyst Seborrheic dermatitis Seborrheic keratosis Skin and hair changes during pregnancy Skin flaps and grafts -- self-care Skin graft Skin lesion biopsy Skin self-exam Stasis dermatitis [icdlist.com]

  About Me I have varied research interests ranging from eHealth, Health Information Exchange, Clinical Trials and Research, Contact Dermatitis, Bioinformatics, and Cosmetic Dermatology. [gulfdoctor.net]

  ) neonatal skin infection ( P39.4 ) staphylococcal scalded skin syndrome ( L00 ) Type 2 Excludes cradle cap ( L21.0 ) diaper [napkin] dermatitis ( L22 ) Other conditions of integument specific to newborn Clinical Information A severe, sometimes fatal [icd10data.com]

  neonatal skin infection ( P39.4 ) staphylococcal scalded skin syndrome ( L00 ) Excludes2 cradle cap ( L21.0 ) diaper [napkin] dermatitis ( L22 ) P83.0 Sclerema neonatorum P83.1 Neonatal erythema toxicum P83.2 Hydrops fetalis not due to hemolytic disease [icd10coded.com]

• Urticaria

  Umbilical polyp of newborn P83.8 Other specified conditions of integument specific to newborn P83.81 Umbilical granuloma P83.88 Other specified conditions of integument specific to newborn Inclusion term(s): Bronze baby syndrome Neonatal scleroderma Urticaria [icd10coded.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 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]

  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]

• Irritability

  Allergies, irritants, your genetic makeup, and certain diseases and immune system problems can cause rashes, hives, and other skin conditions. Many skin problems, such as acne, also affect your appearance. [icdlist.com]

  Parents should be warned of the early signs of hypercalcemia such as lethargy, irritability, hypotonia, vomiting, polyuria, polydipsia, and dehydration. Analgesia is given if the lesion is painful. [dailyrounds.org]

  Although most cases of SCFN resolve spontaneously, SCFN may be associated with symptomatic hypercalcemia presenting as lethargy, hypotonia, irritability, vomiting, polyuria, polydipsia, constipation, and dehydration. [ijpd.in]

  Examination revealed a sick baby who was irritable and lethargic with moderate dehydration and sclerema. Her weight was 2200 g, indicating a weight loss of 29%. She had a respiratory rate of 30/min and was acidotic. [sjkdt.org]

• Agitation

  In some cases with significant pain and agitation, prednisolone, and morphine were also used. [7] Woods and Cederholm [1] reported a sedative agent dexmedetomidine usage for pain and irritation control in SFN of newborn. [jcnonweb.com]

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].

• Polyps

  […] neonatorum P83.1 - Neonatal erythema toxicum P83.2 - Hydrops fetalis not due to haemolytic disease P83.3 - Other and unspecified oedema specific to fetus and newborn P83.4 - Breast engorgement of newborn P83.5 - Congenital hydrocele P83.6 - Umbilical polyp [icd-code.org]

  […] fetalis not due to hemolytic disease P83.3 Other and unspecified edema specific to newborn P83.30 Unspecified edema specific to newborn P83.39 Other edema specific to newborn P83.4 Breast engorgement of newborn P83.5 Congenital hydrocele P83.6 Umbilical polyp [icd10data.com]

  […] unspecified edema specific to newborn P83.30 Unspecified edema specific to newborn P83.39 Other edema specific to newborn P83.4 Breast engorgement of newborn Inclusion term(s): Noninfective mastitis of newborn P83.5 Congenital hydrocele P83.6 Umbilical polyp [icd10coded.com]

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. [mdedge.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]

Treatment Options Treatment is supportive. Monitor fluid status, electrolytes, evaluate for sources of sepsis or other stress. Survival is uncommon. Treatment with systemic steroids and therapeutic warming has not improved survival. [dermatologyadvisor.com]

Prognosis is poor. Minimal inflammation helps distinguish sclerema neonaturum from subcutaneous fat necrosis of the newborn. U.S. [definitions.net]

The prognosis is poor if it becomes generalized. [medical-dictionary.thefreedictionary.com]

[…] usually good; hypercalcemia associated with poor prognosis severity correlates with mortality; many infants die in a few days Occasional patients may show a mixed pattern with features of both syndromes. [meducator3.net]

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]

Certain etiologic considerations. J Invest Dermatol 12: 331, 1949 Google Scholar | Crossref | Medline Horsfield GI, Yardley HT : Sclerema neonatorum. [journals.sagepub.com]

This review attempts to put into perspective their similarities and differences in light of historical, biochemical, pathologic, and etiologic considerations. [meta.org]

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]

Reconstucting transmission with epidemiological analysis and whole-genome sequencing. BMJ (2015) 5:e007397. doi: 10.1136/bmjopen-2014-007397 PubMed Abstract | CrossRef Full Text | Google Scholar 10. [frontiersin.org]

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]

Etiologies and pathophysiology Symptoms Diagnosis Treatment 3. [slideshare.net]

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]

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. [neurologyadvisor.com]

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]

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]

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]

This could have been prevented if the child was brought to the hospital early, thereby preventing the pre-renal failure progressing to intrinsic renal failure. [sjkdt.org]

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.