SCLEREMA NEONATORUM

Synonyms.—Scleroderma neonatorum; Sclerema of the newborn; Underwood‘s
disease; Fr., Sclerémè des nouveau-nés; Algidité progressive; L‘endurcissement ath-
repsique (Parrot); Ger., Das Sklerem der Neugeborenen; Das Fettsklerem.

Symptoms.—This rare disease of infancy, first described by Un­
derwood,⁴ shows itself usually at or shortly after birth, and, as a rule,
first manifests itself upon the lower extremities, and more or less rapidly
invades other parts, in most cases the general involvement ensuing
within three or four days. In some instances jaundice has been asso­
ciated. The skin is at first generally whitish and waxy in appearance;

¹ Brocq, Annales, 1898, No. 2.

² Darier and Gaston, ibid., 1897, p. 451.

³ Allen, Jour. Cutan. Dis., Jan., 1899, p. 40.

⁴ Underwood, Diseases of Children, 1874, p. 76.

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HYPERTROPHIES

it later becomes faintly livid or mottled, and is hard, stiff, leathery
and tense, and the surface cold. It does not pit upon pressure. Fusion
or agglutination with the subjacent parts is noted, and in consequence
of this or as a result of induration of the integument, or of both, the
infant is as if frozen or hewn from marble; it is unable to move or suckle,
respires feebly, and usually, already weakened by intestinal disorders,
pneumonic or circulatory disturbance, the pulse falls, the temperature
drops to several degrees below normal, and it perishes in a few days or
one or two weeks.¹ In those cases in which it appears to be congenital
or develops immediately at birth, death usually results in one or two
days. In extremely exceptional instances the disease, after involving
a small portion of the surface, retrogresses, the involved tissues soften,
regain their elasticity, and recovery ensues; or it may remain stationary,
for a time at least, and resemble somewhat scleroderma in the adult.²

Etiology.—Fortunately the malady is extremely rare, appearing
within the first ten days of life;³ and the cases recorded have for the
most part been in emaciated or atrophic infants in maternity wards
or foundling asylums. Both Underwood and Parrot consider it an
institution disease, often in overcrowded rooms, and associated with
bad hygiene and improper feeding. Some cases seem to be congenital;
others develop in a day or two after birth without recognizable cause;
in others it appears several days after birth, apparently as a result of con­
stitutional diseases which rapidly depress or drain the vitality and bring
on collapse, such as diarrhea, lung affections, cardiac weakness, etc.
According to J. L. Smith,⁴ a considerable proportion of infants with this
disease are prematurely born.

Pathology.—Although the disease had been previously de­
scribed by Underwood and a few others more than a hundred years
ago, it was not until Parrot's⁵ observations that the confusion between
this affection and œdema neonatorum was dissipated, although there
is yet a not uncommon belief that these two affections are allied, and
that they may be also closely related respectively to the sclerous and
edematous types of scleroderma, a view which has, I believe, much in
its favor. Langer⁶ looks upon the malady as due to solidification of
the fat, resulting from the temperature depression, the fat of the new-
born containing relatively so much palmatin and stearin, which readily

¹ In a case reported by L. W. Meyers, Jour. Cutan. Dis., 1909, p. 87, there was
slight elevation of temperature; disease began on third day on the buttocks and thighs
and then spread, child dying on the twenty-fifth day.

² Barr, Brit. Med. Jour., May 4, 1889; Bunch, Brit. Jour Derm., 1898, p. 145
(case demonstration); Pringle, ibid., 1899, p. 290 (case demonstration); and W. Brown­
ing, Jour. Cutan. Dis., 1900, p. 563, report interesting cases of somewhat limited and
peculiar character.

³ Money, Lancet, 1888, vol. ii, p. 811, records 2 cases, sisters, developing one or
two months after birth, associated with paralysis, death ensuing two or several months
later; another sister had previously died from the same disease, also developing late and
lasting a few months before death ensued. The only other child—a boy of two and
one-half years—had so far remained free. I am not sure that these cases belong to this
disease; they are apparently connecting cases between this affection and some cases
of scleroderma, as observed in the adult.

⁴ J. L. Smith, Diseases of Children.

⁵  Parrot, Clinique des Nouveau-nés, L'Athrepsie, Paris, 1877.

⁶ Langer, Wien. med. Presse, 1881, pp. 1375 and 1412.

ŒDEMA NEONATORUM

591

solidifies when the body-heat drops below normal—a view which scarcely
accords with the anatomic findings of Parrot and Ballantyne,¹ who
found practically a “dried-out" skin, some thickening of the layers and
diminution of the fat, and no true sclerosis and no serous effusion, the
drying out being due to the diarrhea. Wiederhofer² and Soltmann³ also
practically accept the belief that the draining of the tissues by serum loss
is of pathologic import. Northrup, quoted by Smith, and others found
histologically nothing especially abnormal.

Prognosis and Treatment.—Apparently only cases in which
the sclerema is not general or complete recover, and these are rare,
a fatal end being the almost invariable result. Treatment consists
in measures to increase the body-heat and the administration of proper
alimentation and stimulants, by tubes passed through nose or mouth
to the pharynx and stomach, or by the rectum, or both.

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