Indian J. Pediat., 31: 8, 1964

SCLEREMA NEONATORUM* A Sludy of 17 Cases SIDDIIARTIt K.. K.HETARPAL a n d

V. V. SUBRAltMANYAM

New Delhi

Sclerema neonatorum as defined by HUGHES and HAMMOND 4 " i s an u n c o m m o n disease characterised by a diffuse rapidly spreading non-oedematous tallow-like hardening of the subcutaneous tissue occurring in the first few weeks of life". The skin in the involved areas cannot be picked up and the subcutaneous tissues are b o u n d down to the subjacent muscle and bone. This usually starts in the lower limbs, either in the calves, thighs or the face and spreads all over the b o d y with the exception of the palms, soles and genitalia. Though the physical findings are marked, histologically the skin and subcutaneous tissues show no identifiable lesions *' 7, 10 Observations Seventcen cases were observed and treated since January, 1960. The clinical observations are mentioned in Table 1. Eleven were males and the rest females. No seasonal incidence was observed, six cases having been seen in the winter months and 11 in the summer months. The birth weight ranged between 1.05 kg. and 2.75 kg. with an average of 1.80 kg. All of them excepting on infant were less than 2.5 kg. in weight at birth. Eleven of *From the department of Pediatrics, All India Institute of Medical Sciences, New Delhi.

these were premature by gestation and one was postmature. No relation was observed between the parity of the mother and the sclerema. Five were first-born and tile rest were born to multiparous women. The mothers' health during pregnancy had been tmeventful except in two cases. In one case tile serological tests for syphilis were positive in tile antenatal period and she was treated accordingly. The baby did not show any evidmlce o f congenital syphilis. Tile second m o t h e r had toxaemia and the b a b y was b o r n prematurely after 29 weeks. The delivery was spontaneous in 15 cases and by caesarian section in two. The rectal temperatures at the time tile sclerema was noticed varied f r o m 97~ to 98~ in six cases, 96~ to 97~ in two cases and 94~ to 96~ in eight cases and below 94 ~ in one case. All the cases were investigated for evidence of infection. Blood culture and X-ray of the chest were done in all the C~,ses.

Sclerema was associated with res. piratory infection (including empyema) in fourcases. T h e p r e s e n c e o f c r e p i t a t i o n s on clinical examination or opacity in tile X-ray of tile chest was taken as an indication of respiratory infection. Eight cases were associated with jaundice, three being

KI:IETARPAL AND SUBRAHMANYAM--SCLI.iRMA NEONATORUM

moderately severe (12-14 nag. per cent. serum bilirubin) and one with severe jaundice (20-24 nag. per cent. serum bilirubin). Blood culture yielded Alcaligenes faecalis and Pseudolnonas aeruginosa in two instances each and Proteus mirabilis and Pseudomonaspyocyaneus in one case each. Culture of the pus from the pleural cavity yielded Proteus morgani and Pseudomonas aeruginosa ill one case. Skin biopsy done in three eases did not reveal any identifiable change. Two cases were preceeded by major operative procedures, one for unilateral cleft lip and the other for tracheo-oesophageal fistula. One case had multiple congenital anomalies. The day of development of sclerema varied from the 2nd to the 17th day with an average of 6.5 days. The extent of involvement varied. The site which was affected initially was the lower limbs, most often over the calves. The other common areas involved were the cheeks. The hardening of the cheeks gave the appearance of a grimace and there was difficulty in feeding these cases. Involvement of the subcutaneous tissue of the lower limbs caused restriction of movements at the joints of the lower limbs. Feeding and respiratory difficulty was noticed in 12 cases. The activity of the babies in general was poor, whether it was due to sclerema per se, associated infection or other diseases is difficult to say. Treatment carried out in all these cases was mainly supportive and included (i) Warming of the patient in blankets and keeping the environment warm. (2) Administration of oxygen in the presence of cyanosis. (3) Maintenance of nutrition of the baby. If the baby was not able to swallow properly gavage feeding was done. (4) Antibiotics were administered to combat the infection, in the doses 2

9

mentioned. Injection of crystalline penicillin 50,000 to 100,000 I.U. intramuscularly 6-12 hourly, injection of streptomycin 20 mg/kg/day intranauscularly and tetracycline 20 mg/kg/day in two or three divided doses. (5) In addition corticosteroids were used in 12 cases (cortisone 8-10 mg/kg/day or prednisone 2.2. mg/kg/day immediately after the sclerema was noticed. With all the above supportive measures, the results of the therapy were discouraging. Only three of the 17 cases survived. In addition one baby who had sclerema on the 4th day of birth survived this and was "cured" completely of sclerema, but again developed sclererna on the 17th day, one day prior to his death. This clearly shows the gravity of the disease process and also that it may recur. Discussion GERLOCZY 3 observed sclerema mostly among premature infants, whose birth weight ranged from 1.5 to 2 kg. hi the HUGHES and HAMMONDS~ review of 31 cases, the average birth weight was 2.8 kg ranging between 2.15 kg and 4 kg. LEYIN et al 5 in their study found that the weight of the babies ranged from 1.09 kg to 2.25 kg. This is in accordance with our observations that 16 out of the 17 cases were premature by weight (range was between 1.05 kg and 2.75 kg with an average weight of 1.80 kg). This, however, clearly shows that sclerema is much more common in premature infants, as 94.1 per cent. of our cases were premature. Sclerema has often been noted to be associated with some grave condition such as septicaemia, respiratory infection or diarrhoea, usually with dehydrationS'L None of our cases had diarrhoea and dehydration. However, in the same period we observed sclerema-like changes

VOL. 31 NO. 192

10 INDIAN JOURNAL OF PEDIATRICS

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12 INDIAN JOURNAL OF PEDIATRICS in hypermtlraemia in lwo babies having diarrhoea and dehydration with a serum sodium level of 160 mEq/L and 163 mEq/L respectively. Much has been said about the relationship between the temperature of the baby and the development of sclerema, the sudden drop of temperature below 95~ being favourable to its development. However, the occurrence of sclerema in utero and sometimes in babies having pyrexia does not substantiate this. Only nine of our 17 cases had low temperatures (below 96~ ; the temperature in others was above 96~ HUGHES and HAMMOND4 believe that shock produced by circulatory collapse caused vasoconstriction and thus reduction of the peripheral temperature leading to solidification, "chilling" of the fat together with the thickening of the collagen fibers. High melting point of the foelal fat because of the low oleic acid mid high palmitic acid content has been postulated to predispose to solidification in the presence of a low body temperature I. The histopathological changes observed by various workers has varied from normal to crystallization of subcutaneous fat and cystqike spaces of subcutaneous fat ~-. In the eleven cases autopsied in HUGHES' a n d HAMMOND'S 4 series, the histopathological findings showed thickening o f the connective tissue bands in the subscutaneous tissue. However, we did not observe any identifiable changes in skin and subcutaneous tissue in the three cases whose skin biopsy was done. Sclerema neonatorum should be distinguished from scleredema, subcutaneous fat necrosis and scleremalike changes observed in cases with hypernatremiaS'L In sclerema n e o n a t o r u m there is widespread involvement of the skin, which is cold to touch and cannot be separated from underlying structuresS,9. The characteristic pitting seen in sclere.

VOL, 31 NO. 192 dema i~ absent tl. It of'ten occurs in prematures and the baby is usually 'ill' at the time of onset of sclerema. In subcutaneous fat necrosis there are scattered multiple lesions which can be freely moved over the underlying subcutaneous tissue, and the baby is usually healthy. In hypernatremiathe skin isnot bound to the underlying strctures and serum sodium is high. Treatment is nonspecific. The value of corticosteroids in the treatment of sclerema neonatorum has been studied by LEVIN et aF'. Of the 25 cases studied, 11 were treated with and 14 without corticosteroids. Only one of the 11 cases receiving corticosteroids survived (although remission occurred in two cases) and of the latter series three survived. Thus the steroids seem to have no important role in the treatment o f sclerema neonatorum. In our series 12 were treated with corticosteroids in addition to other therapeutic measures. There cases survived and another baby lived after the first attack and was " c u r e d " but succumbed to the second atack of sclerema one week later. None of the five cases who did not receive corticosteroids survived.

Summary 1. Seventeen cases of sclerema neonatorumwere observed during a period o f 3 years and 9 months. Of these 17, sixteen were premature by weight. 2. Septicaemia was present in six, respiratory infection in four, and previous operative interference in two. 3. Only three cases survived ; another patient survived the first attack but succumbed to the second.

References 1. CttANNON, H. J. and HARRISON,G. A.--The chemical nature of the subcutaneous fat in the normal and sclerematous infant. Biochem.J., 20 : 84, 1926. (Quoted by Silvermang).

Kill: FARPAL AND SUBRAIIMANYAM--SCLEREMA NEONATORUM 2.

3.

4. 5.

6.

ILORY, C. M , - - F a t necrosis of the newborn. Report of a case with necrosis of the subcutaneous and visceral fat. Arch. Path., 45 : 278, 1948. GERt, OCZY, F . - - C l i n i c a l a n d pathological role of d, l-alpha tocopherol in premature infants. ,4mr. Paediat., 173 : 171, 1949. (Quoted by SilvermanE) HUGHES, W. E. and HAMMONI), M. I...~ Sclerema neonalorum. J. Pediat., 32 : 676, 1948. LEVJN,S. E., BAKSr, C. M., and [SSEROW, L. -Sclerema neonatorum treated with corticosteroids. Brit. reed. J., 2 : 1933, 1961. MICHAEL,A. B., BRESCIA,M. A. TARTAGLIONE E. F. and QUEENS, N. Y.--Sclerema neonatorum. J. Pedlar., 45 : 720, 1954.

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POIFER, E. L.--Sclerema ueouatorum. Pathology of the fetus and newborn. The Year Book Publishers lne. Chicago, 1957. 8. SCHAFrER, A. J.--Sclercma neonatorum. Diseases of newborn. W. B. Saunders Contpany, Philadelphia, 1960. 9. SILVERMANN,W. A.--Sclerema n e o n a t o r u m in DUNHAM'S Premature infant. Paul B. Hoeber Inc., New York, 3rd Ed., 1961. 10. STOWENS, D . - - S c l e r e m a neonatorum. Pediatric Pathology. The Williams and and Wilkins Company, Baltimore, 1959. 11. WRIOnT, C. S.--Sclerema neonatorum in Text book of Pediatrics. Ed., Nelson, W.E. 7th Ed. 14/. B. Saunders Company, Philadelphia, 1959. 7.