Indian Journal of Paediatric Dermatology

REVIEW ARTICLE
Year
: 2019  |  Volume : 20  |  Issue : 4  |  Page : 302--305

Sclerema neonatorum


Konchok Dorjay1, Stanzin Dolker2, Tasleem Arif3, Mohammad Adil3, Sheetal Ganju4,  
1 Department of Dermatology, Dr Ram Manohar Lohia Hospital and Post Graduate Institute of Medical Education and Research, New Delhi, India
2 Department of Paediatric, North DMC Medical College and Hindu Rao Hospital, New Delhi, India
3 Department of Dermatology, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India
4 Department of Neonatology, LNJP Hospital, New Delhi, India

Correspondence Address:
Dr Konchok Dorjay
Dr Ram Manohar Lohia Hospital and Post Graduate Institute of Medical Education and Research, New Delhi - 110 001
India

Abstract

Sclerema neonatorum (SN) is a rare clinical condition usually seen during the 1st week of life. It presents as hardening of the skin and has a high mortality rate. It is usually associated with congenital anomalies, hypothermia, respiratory illnesses, and sepsis. Defective lipolytic enzymes, a high melting point, and resultant low solidification point of saturated fatty acids of subcutaneous fat, edema of connective tissue septae, and signs of underlying systemic diseases are the various theories proposed for the development of SN. The skin biopsy shows the thickening of trabeculae, sparse inflammatory infiltrates of lymphocytes, histiocytes and multinucleate giant cells, and X-ray diffraction of a biopsy sample of SN shows crystals in affected sites. The systemic corticosteroids have been used by various authors with variable response. Use of exchange transfusion has recently shown good results in improving the outcome of SN.



How to cite this article:
Dorjay K, Dolker S, Arif T, Adil M, Ganju S. Sclerema neonatorum.Indian J Paediatr Dermatol 2019;20:302-305


How to cite this URL:
Dorjay K, Dolker S, Arif T, Adil M, Ganju S. Sclerema neonatorum. Indian J Paediatr Dermatol [serial online] 2019 [cited 2019 Nov 17 ];20:302-305
Available from: http://www.ijpd.in/text.asp?2019/20/4/302/268388


Full Text



 Introduction



Sclerema neonatorum (SN) is clinically characterized by induration of the skin and subcutaneous tissue, which often starts in the buttocks and thighs and then spreads to other parts of the body. Gray defined it as a condition invariably occurring in the 1st weeks of life, occasionally present at birth, and characterized by the gradual development of sharply defined indurated areas in the subcutaneous tissues.[1] It commonly affects thighs, buttocks, scapular region, deltoid region, and the cheeks. The genitalia, palms, and soles are spared.[2] The skin of the child is cold and stiff. Hence, there is difficulty in sucking, respiration, and body movements. SN is slightly more common in the male with a sex ratio of 1.5:1.[3] SN is now a rare disease, precipitated by prematurity, asphyxia, and hypothermia.[4],[5] The summary of published studies on SN is shown in [Table 1]. For this review, the information was collected by PubMed, Google Scholar, and Scopus search including original articles, review articles, and case reports. The keywords sclerma neonatorum (“SN”) were used for the search.{Table 1}

 History



In 1718, Uzembezius used the term “Acute sclerema” for SN.[9],[15] Underwood presented a detailed description in 1784 as “Skinbound.”[9],[15],[16] Andry in 1785 assumed that the “Endurcissement du tissue cellulaire” and “Skinbound” were the same diseases.[16],[17] However, Chaussier in 1815 used the term “Sclereme” for “Endurcissement du tissue cellulaire.” In 1877, “Endurcissement du tissue cellulaire” was replaced by a new term, “oedema neonatorum.” Later, skinbound and “oedema neonatorum” were collectively given the term SN.[16],[17] Bernheimer-Karrer called the condition “Benign Sclerema” in 1922.[15] SN and subcutaneous fat necrosis of the newborn were described as the same disease by Gray in 1926.

 Pathogenesis



SN mostly affects preterm neonates in the 1st week of life. It is associated with many underlying conditions. They are gastroenteritis, pneumonia, congenital heart defects, hypothermia, septicemia, respiratory distress syndrome, septic shock, metabolic acidosis, severe malnutrition, transient hyperammonemia of the newborn, intestinal obstruction and therapeutic hypothermia.[5],[18],[19],[20],[21] In a study, Pelet found the low levels of C3 and factor B in sclerema with septicemia in newborn infants.[22] However, after transfusion, these levels became normal. Hence, they postulated defective activation of complement pathways for pathogenesis of SN. The pathogenesis of SN is explained by various theories. Earlier SN was thought to be a sign of grave underlying disorders requiring specific treatment for the same and was regarded as a special form of edema affecting the connective tissue septae causing thickening of the skin.[9],[11],[17],[23] Now, recently two more theories have been proposed.

High melting and a low solidification point of saturated fat theory

The fatty acids in the neonate are predominantly composed of saturated fatty acids, thus having a high melting point and a low solidification point. Hence, they have a tendency to harden at low body temperature.[24] Hughes and Hammond support this idea. They proposed that SN occurs due to circulatory collapse secondary to shock causing low peripheral body temperature leading to hardening of the skin.[14] Elliott argues against this theory.[17]

Defective fat metabolism theory

The second theory ascribes SN to be due to defective fat metabolism. A defect in adipose lipolytic enzymes or in one of the lipid transport mechanisms are believed to be the main underlying factors responsible for SN. Hence, there is hardening of the skin due to defective immobilization of fatty acids from the subcutaneous adipose tissue.[23]

 Investigations



The chemistry of the subcutaneous fat of the sclerematous infant was extensively studied by various authors.[24],[25],[26] They found that the oleic acid content was decreased. The crystals seen in the affected fat were shown to be the properties of triglycerides. The X-ray diffraction of the biopsy sample of SN shows that the crystals in affected sites are larger than the normal subcutaneous fat.[27] The epidermis and dermis are normal with sparse inflammatory infiltrate within the fat tissue. The fat spaces are diminished and trabeculae are broadened without fat necrosis.[23],[28] Dasgupta et al. showed subcutaneous fibrosis with lobulation.[6] Many authors reported that if the sclerematous lesion breaks down to form a sterile abscess, such crystals can be aspirated.[25],[29] There is reduced iodine value and a raised melting point of the fat. Based on this finding, Channon and Harrison found increased ratio of saturated to the unsaturated fatty acids.[24] Sweeney et al. found that triglyceride constituted 98%–99% of subcutaneous fat in 6– 7-week old normal infants.[30] In a study, Horsfield and Yardley found that there is a significant decrease in oleic acid contents in subcutaneous fat as well as in dermis and epidermis in SN. A complete sepsis workup should be initiated in all infants with sclerema neonatorum.[27]

 Clinical Features



Physical findings of SN appear suddenly, on the thighs and buttocks and spread rapidly, often affecting all parts of the body except the palms, soles, and genitalia. The onset of SN is usually at birth or during the 1st week of life. However, Spohn et al. recently reported a delayed onset of SN in the Neonatal Intensive Care Unit.[31] The involved skin is pale, waxy, and firm to palpation [Figure 1]. The skin cannot be pitted or pinched up [Figure 1] because it is bound to underlying subcutaneous tissue, muscle, and bone. The affected infant often displays flexion contractures at the elbow, knee, and hip joints; temperature instability; restricted respiration; difficulty in feeding; and decreased spontaneous movement. Newborns may also present with mask-like facies or “pseudotrismus,” an inability to completely open the mouth, secondary to the thickening of the skin over the face, arms, and hands.[4] Pulse, temperature, and respiratory rate are decreased, and there is difficulty in respiration and movement. No seasonal variation or maternal parity was associated with SN.[10] Underwood listed the following pathognomonic symptoms of SN [Table 2].[32]{Figure 1}{Table 2}

Subcutaneous fat necrosis of the newborn (SCFN) and scleredema are the two other related diseases which have to be differentiated from SN. SCFN affects postmature neonates during the 4 weeks of life as compared to SN, which is usually seen in premature neonates. Clinically, it presents as circumscribed areas of hardness attached to the skin, move freely over muscles and bone, and generally do not spread. It usually appears in healthy neonates. The histologic findings of sclerema neonatorum are subtle, The most consistent findings are edema, a thickening of the subcutaneous fibrous septa, and a radial array of fine, needlelike clefts in the fat cells, representing former sites of fat crystals. The lack of a granulomatous inflammation and the absence of fat necrosis help distinguish sclerema neonatorum from subcutaneous fat necrosis of the newborn.

 Treatment



Careful handling of the newborn, proper temperature control, rational use of antibiotics, and fluid and electrolyte correction has improved the prognosis of neonatal septicemia. But still, septicemia is an important cause of neonatal mortality. Recently, steroid therapy and exchange transfusion (ET) have significantly improved the outcome of SN.

Many authors have emphasized the importance of treating the underlying disease.[9],[11],[14] The outcome of SN might be influenced by use of antibiotics. In a study by Bower et al., nine out of 14 full-term infants with sclerema who received antibiotics without the addition of steroids survived.[13] There are other reports, in which improvement of SN with antibiotics has been reported.[12]

Steroids have been used in SN, but with variable result. Cortisone was used in SN by Kendig and Toone with good recovery. Kendall and Ledis gave A.C.T.H. with same dramatic outcome.[32]

Beneficial effect of steroids in SN was proposed by Marks as adjunctive therapy.[33] Levin et al. reported improved outcome in a comparative study in 25 patients with or without hydrocortisone. Shrestha et al. also managed SN with parenteral steroids.[34] Out of 11 children treated with hydrocortisone, remission was seen only in two cases, as compared to controls in which three survived out of 14 patients.[12] Therefore, there is yet no certain evidence to use corticosteroids in SN. Hence, further studies are required. Hughes and Hammond did a review of the literature and collected 16 cases of SN. They also reported three cases. Out of 19 patients, only five survived.[14]

Recently, the use of ET has shown promising results in improving outcome of SN. Xanthou et al. performed ET in two critically ill patients with sclerema. They found good improvement and the sclerema disappeared within 3–4 days.[35] In another study, Sadana et al. found greater survival of babies with premature birth (28- to 32-week gestation) than those born >32 weeks of gestation after ET.[36] The mechanism by which ET works in SN might be due to immunological components, including enhanced humoral and cellular immunity that are provided through ET to the immunologically immature neonates, or by improving oxygen transportation by improving the circulation and shifting the oxygen dissociation curve to the right, correcting hypovolemia, improving hemostatic mechanisms, and removing bacteria or bacterial toxins.[35],[37],[38],[39] Hence, there is increased survival in patients with SN and sepsis when repeat ET is done early during the disease. Park et al. recently found a favorable prognosis of SN in a full-term infant.[40]

Buster et al. recently reported a short-term clinical improvement with use of intravenous immunoglobulin in a patient of SN.[41] It probably helps by increasing both humoral and cellular immunity in the patient.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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