Dec,, 19371 ERYTHRODERMA DESQUAMATIVA : LINDBERG 741

A CASE OF ERYTHRODERMA DES-

QUAMATIVA (LEINER-MOUSSOUS) IN

AN INDIAN CHILD

By K. LINDBERG Medical Officer,- Barsi Light Railway: Company

Cases of erythroderma desquamativa in

nurslings are not often met with, and as they present etiological and therapeutical problems of great interest, the report of a recently observed case which showed unusual features seems fully justified. Moreover, as far as I know, no case of this disease has up till now been recorded from India.

History of the case

A male infant was born as the fifth child to a

Mohammedan brick-layer in the railway colony at

Kurduvadi on 8th March, 1937. Its weight on 11th March was 6 lb. 10| oz. and the child seemed healthy and well developed. The parents and the other children were in good health (one child had however died from extensive burns at the age of 2J years) and there was nothing in their present or past history that had any bearing on the case under report. The child was breast fed from its birth and although the parents had been in the habit of giving opium to their first children this was not done, according to their statements, in the case of this child. The baby was seen again by a sub- assistant surgeon on infant welfare duty on 11th April and it was reported to be quite well and weighing 9 lb. 6 oz.; an increase of 2 lb. 11* oz. in four weeks.

On 1st May, after having been advised to bring the child for vaccination, the father came to the dispensary without the baby and stated that the child was not in a fit condition to be vaccinated owing to a skin disease 'bran' (bhusa) having formed on its body. The father was then instructed to bring the child for

examination. A big, fat and plump baby, weighing 9 lb. 10 oz.

was then produced, the skin had a markedly reddish hue and was tense and firm as if soaked with fluid but without pitting; its surface was dry and covered

nearly everywhere by epidermic flakes, which were

mostly large and partly detached. This condition, the erythema, the desquamation and the tenseness of the

integuments, was generalized on the head, trunk and

limbs, but the hands, both dorsal and palmar aspects, were entirely unaffected and the soles of the feet showed only slight erythema and desquamation. There was no raised zone of demarcation between the healthy and affected parts. The desquamated flakes were

particularly large on the trunk, the scalp and the

proximal parts or the limbs; the erythema_ appeared most intense on the same regions and the infiltration of the integument seemed most marked on the face, especially around the orbits and the mouth. The eye- lids could not be completely closed and the child could not suck, not being able to move the lips freely. The penis and scrotum were also cedematous. There was

no loss of hair on the scalp. The rectal temperature was 100.2?F. The lungs were

clear but the child was said to have been coughing a little for the last five or six days. There was some

dyspnoea. The heart sounds were normal. The spleen was not palpable. The liver was enlarged and appa- rently tender, and reached two finger-breadths below the costal margin in the nipple line. The conjunctiva! were of a dirty greyish-yellow colour and showed a

markedly dry aspect; there was also a lack of lustre of the corneae.

According to the father the child had been quite well up to five or six days previously when redness and desquamation started on the scalp; it rapidly spread down the face and trunk, with the exception of the

hands and the soles of the feet, within the next three days. The child was said to have had some disturbance of the bowels during the five or six previous days but the parents were not explicit on this point. The father had procured castor oil from the village and admin- istered it to his child one or two days before it was seen and stated that the bowels had been satisfactory afterwards. He said that no other treatment had been

given. There had not been any vomiting; the child had had four diarrhceic stools since the previous evening. The mother's milk was discontinued; a proprietary

butter-milk preparation (Eledon 16 gm. to water 90 c.c.) was supplied and the mother was instructed to give as many teaspoonfuls as the child would take every second hour, haliverol one drop daily and calcium lactate 0.10 gm. thrice daily were prescribed; 05 per cent

carbolic acid in olive oil was supplied for application twice daily, and the parents were asked not to use any water on the skin of their child.

The following day the general condition of the child was stationary; the rectal temperature remained at

100.3?F.; the erythema and tenseness were less marked and desquamated flakes removed through the oil appli- cation revealed almost healthy looking epidermis. The butter-milk mixture was rejected by the child and only water and a small amount of breast milk taken.

In the morning of 3rd May the temperature rose to 104.2?F.; there had been four loose stools, but no

vomiting; dyspnoea was marked. A blood examination showed: haemoglobin 75 per cent (Tallqvist scale); total white cells 12.000. Differential white cell count:

Neutrophils .. 77 per cent

Juveniles .. 10.5 ,,

Stab forms .. 40 ?

Segmented forms .. 26.5 ?

Lymphocytes .. 15.5 ?

Mononuclears .. 7.5 ?

Two c.cm. of the mother's whole blood as advised by Petenyi (1925) were then injected intramuscularly. In the evening the temperature rose to 105?F., and respirations were 80 per minute. Early the following morning (4th May), the child was

found semi-conscious with eyeballs turned upwards and showing deep icterus of the sclerae. The lungs were

clear; heart sounds weak and slow. The lower margin of the liver was now felt at the level of the umbilicus in the nipple line. The spleen was not enlarged. There had been two diarrha;ic stools and no vomiting. Urine was passed spontaneously during the examina-

tion and was received directly in a glass. It was clear, deep orange yellow with a greenish foam, of acid reaction. The following tests were carried out:

Gmeliri + +?. Benedict?green precipitate. Uro- bilinogen?negative. Indican?negative. Albumin? trace. Sediment: Epithelial cells?(some of 'renal'

type); a few granular casts, erythrocytes very scanty. The stool specimen was loose, greenish, faecal and

showed very numerous neutral fat globules, neutral fat flakes and a moderate amount of fatty acid crystals. There were no leucocytes. The child died early in the afternoon of the same

day. A post-mortem examination was refused by the parents.

Discussion

There seems to be no doubt that the case

was one of erythema desquamativa, Leiner- Moussous. The age of the baby (two months), the non-occurrence of vesicles or bullae and the

presence' of constitutional symptoms differen- tiated it from the dermatitis exfoliativa neonatorum of Ritter von Rittersheim. The absence of marked temperature up to the day before the fatal termination together with the

742 THE INDIAN MEDICAL GAZETTE [Dec., 1937

comparatively slight systemic disturbance during the first three days of observation and the mode of extension of the cutaneous condition seemed to exclude erysipelas. As already mentioned there was no history of any toxic remedy having been given to the child previous to its illness.

It is proposed to examine briefly whether the scanty and incomplete data furnished by the

present case lend any support to one or other of the two main theories put forward to explain cases of eiythroderma desquamativa.

I. Avitaminosis theory.?Cases of ery- throderma desquamativa accompanied by keratomalacia and xerosis have been reported especially in Germany during the years that followed the war, but such coexistence does not seem to have been observed with any frequency in countries where vitamin-A deficiency is common. Although xerosis was evident in the present case when the baby was seen at the

height of the illness, the child was apparently in perfect health and development up to the time of the acute onset of the illness and, con- sidering the rare occurrence of xerosis in babies at Kurduvadi, it is believed that deficiency of vitamin A was not likely to be at the origin of the illness in this case but was probably a result of the hepatic involvement, the liver being known as the storage organ of this vitamin. I have elsewhere drawn attention to the relationship between vitamin-A deficiency and disease of the liver (Lindberg, 1935). E. Moro, the authority on eczema

seborrhoicum, is said to consider erythema desquamativa to be due to a vitamin-H defi-

ciency, apparently from a mainly anatomo-

pathological reasoning as no doubt there is a

disturbed secretion of the sebaceous glands in

both the conditions. The available information on this vitamin is however still too scanty to

allow a discussion of this theory. II. Theory of intestinal toxcemia.?As the

disease is observed practically only during the first three months of life it is evident that the

great majority of the cases have been reported in breast-fed babies. Erythema desquamativa has however in fairly numerous instances been seen in artificially-fed babies as well, the percentage of cases in such babies varying from 5 (Leiner) to 16 per cent in the series of various authors. This fact would indicate that the noxious agent is not necessarily of a purely endogenous nature but may primarily originate from the outside world.

It is not difficult to conceive why cases of

erythroderma desquamativa are limited to the first few months of life. The delicate structure of the epidermis, its incomplete cornification and the abundance of capillaries in the papillary layer make the skin of young nurslings exceed- ingly sensitive and explain the rapid extension of the disease; further, the tendency to

seborrhcea commonly present during the first weeks of life and the incomplete development of

the sweat glands would favour any desquamative process. The water balance disturbance evid- enced by the infiltration of the integument is a

sign that has been frequently noted as well as a tendency to anaemia. Noxious agents are of course particularly

likely to gain entry through the mucous

membrane of the intestines owing to its great vulnerability during the first months of life. Hill (1934) says

'

dyspepsia ' and diarrhoea are

said to be constant features in cases of

erythroderma desquamativa and the develop- ment of alimentary toxaemia (the

1 exsiccation toxicosis of nurslings' of American writers) during or after its course has been noted by several authors. The occurrence of dehydration can be understood both as a result of the diarrhoea and the hydrolability already referred to, fluid being drained from parenchymatous organs and retained in the corium.

The clinical picture of alimentary toxaemia was nearly complete in the present case, with

diarrhoea, disturbed consciousness, dyspnoea and enlargement of the liver. As far as I can find from the literature at my disposal the occurrence of jaundice has however not been noted in other cases. For some reason there was during the third day of observation a sudden increase, quantitative or qualitative, of the toxic agents or a sudden breakdown of what remained of the defensive mechanism of the body with conse- quent fever, further involvement of the liver as translated bv further increase in size, jaundice, choluria and presumably guanidinaemia followed by rapid coma and death.

Clinical descriptions of cases of erythroderma desquamativa show somewhat variable features and one is tempted to believe that the noxae

causing this syndrome may be of different natures. Skin reactions are rarely specific and owing to constitutional peculiarities, the nature of which can seldom be determined, the skin may react in a certain and similar way to stimuli which may be physically or chemically different.

References

Hill, L. W. (1934). Erythroderma Desquamativa. Journ. Pediat., Vol. IV, p. 436. (Abstract?Jouri i. Amer. Med. Assoc., 1935, Vol. CIV, p. 237.) Lindberg, K. (1935). A Case of Keratomalacia with

Enlargement of the Liver. Journ. Christian Med. Assoc., India, Vol. X, p. 25.

Petenyi, G. (1925). liber Leinersche Dermatitis. Therapia (Budapest), Vol. II, p. 425. (Abstract?Ztbl. Haul. Geschlk., 1926, Vol. XIX, p. 643.)

[Note.?Erythroderma desquamativa is not a term

used by; British dermatologists, as far as we are aware, and the condition would appear to fall into the group of allergic dermatitis of infants. In a young breast-fed child the exciting agent is probably a foreign substance in the mother's milk. Death occasionally occurs in children with allergic dermatitis, without any cause being obvious, but it probably always means that the child is suffering from some serious disease of the intestinal

organs, especially the liver, which has not been diagnosed, and the present case suggests this explanation of the child's death.?Editor, I. M. (7.1