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A case of pellagra in a child two years old
A CASE O F P E L L A G R A I N A C H I L D T W O YEAI~S OLD EUGENE T. 1VICENERY,M.D. CHICAGO, ILL.
G R A in children in the Central W e s t is a v e r y r a r e condition, p Ea nLdL Abecause of the unusual oecnrrence of this deficiency disease, the following case is reported. CASE REPot'r: G. E., a 1Viexican boy, aged two years, was first seen on l~ay 3, on account of a skin condition. The mother was living and well. The father had been treated in a sanltarinm, but no positive diagnosis of tuberculosis had been made. There were two children in the family, the patient and a glrl one year old. There was no history of any miscarriages or syphilis in the immediate family. The birth history was uneventful. The child had been on the breast until eight months of age, when he was placed on whole milk, eight ounces with five teaspoonfuls of sugar, four feedings a day. Gradually other foods were added to his diet, such as cereal, soups, beans, rice, apples, bananas, and prunes, but there was no history of any fresh frults~ vegetables, or meat having been given. Cod liver oil had been taken very irregularly. The mother considered the child's appetite good, b u t she had to make things very sweet before he wou]d take any food. Envi.ron.~ner family lived in a box ear. The small home was very clean, and the children were given good care. D evelop~nent.--The first tooth erupted at four months. He sat erect at ten months, but at present he does not walk and talks very little. The mother first noticed the skin condition when the child was weaned from the breast, sixteen months ago, the child then being only eight months of age. Exa~ninatioa showed a very thin, undernourished Mexican boy, sitting erect with his back to the light, eyes closed, with marked photophobia and blepharospasm. There was a complete alopee~a of the scalp. The teeth were in excellent condition, without caries or discoloration. The gums were very soft but did not bleed. The heart and lungs were negative. The abdomen was slightly distended, but the liver and spleen were not palpable. The extremities were thin and undeveloped. The most striking thing about the patient was the symmetrical skin lesion. S]c/~.--The three typical stages of the cutaneous manifestations of pellagra; namely, hyperemia, desquamation, and fissul'es~ were all present and were distributed over the body in the following manner: The face was covered with a masklike lesion, with skin contractures under the eyes so marked as to create a Ibi]atera] ectropion. About the neck the lesion extended in a collar fashion, as described by Cassal. The anterior chest was free from any involvement. Below the umbilicus, it extended downward and outward to both anterior superior spines of the pelvis, then down the mesial portion of the thigh, involving the scrotum, anal region~ and the buttocks. The feet were covered entirely, the lesion extending up to and around the ankle, with a slight extension up the inner side of the leg to the knee. The hands, especially, the dorsum, were involved up to the wrist in a gauntletdike arrangement. (Figs. 1, 2, 3.) Na~t~.re of the Ski~ Les~o~s.--In some areas the skin was involved in a hyperemic stage with slight desquamation; in other parts~ about the scrotum and anus~ there From The Children's IViemorial Hospital. Service Dr. Joseph Brennemann. 478
~ C ENERY:
PELL&G~A
479
were small vesicles and fissures which had become secondarily infected from scratching. The color varied from a rose to a vivid red and dark brown. About the lower eyelids there was an increased amount of pigment with atrophic thinning of the epidermis and a certain amount of fibrosis of the subcutaneous connective tissue, which brought about the bilateral eetropion of the lower lids. Laboratory Datam.--Examination of the blood showed, hemoglobin (Sahli) 80 per cent, W.B.C. 10,600, t~:B.C. 4,300~000, Differential--Poly. 53 per cent, Lymph. 42 per cent, Trans. 4 per cent. :~o abnormal cells present. The Pirquet test was negative. The Wassermann test was negative. The x-ray of the chest was considered normal. An examination of the stools for parasites was negative. Co~rse and Treatment.--The child was put on whole milk s fresh vegetables, orange juice, cod liver oil, and yeast. A t first he refused to take anything solid, and if the
9"ig. 1.--Front view showing symmetrical distribution of lesions. feedings were forced, the lesions about the mouth bled profusely so it was necessary to give food by gavage. His progress was very stow and he would take little or nothing but sweets at first. With a tremendous amount of patience~ he was finally coaxed to take small amounts of fresh vegetables and meat. The skin was treated with bland ointments. Under the r6gime, the cutaneous lesions began to improve. A f t e r being in the hospital for six weeks, the prognosis looked favorable. At t h i s time the following incident took place, which illustrates the harmful effects of sunlight on pellagrinous dermatitis. The child was placed in a solarium for a few hours; two days later t h e skin lesions became worse with a reappearance of vesicles, blebs, and fissures. The p a t i e n t ' s general condition also reacted, with loss of appetite and severe diaa.rhea, which lasted for several days. About a month after this relapse, it was thought that small doses of quartz light might desensitize the skin to such a reaction, so four doses of thirty seconds exposure were given over
480
T t / E JOUICbTAL OF PEDIATRICS
a period o f a week. T h e d a y following t h e last exposure, a n o t h e r reaction, s i m i l a r to t h e one caused b y t h e exposure to s u n l i g h t , took place. T h e e x a c e r b a t i o n of t h e s k i n lesion a n d t h e g a s t r o i n t e s t i n a l s y m p t o m s were m o r e prolonged, a n d his r e t u r n to t a k i n g o f f o o d o t h e r t h a n b y g a r a g e was a m a t t e r of t h r e e weeks. H i s m e n t a l r e a c t i o n d u r i n g all of t h i s period w a s v e r y u n h a p p y . He seldom p l a y e d w i t h his t o y s a n d w a s t h e p i c t u r e of a b j e c t misery. T h e p r o g r e s s of t h e case was u n e v e n t f u l a f t e r t h e exposure to s u n l i g h t a n d q u a r t z light. T h e s k i n i m p r o v e d a~ad t h e a p p e t i t e w a s b e t t e r t h a n i t h a d ever been.
~ig. 2 . - - S h o w i n g lesions on a b d o m e n a n d legs.
Fig. 3.~]~ack view showing distribution a b o u t the a n u s . A complete recovery w a s looked for, w h e a u n e x p e c t e d l y a n d w i t h o u t w a r n i n g the child w a s f o u n d d e a d i n bed, t h r e e m o n t h s a f t e r admission. hreerops~ w a s p e r f o r m e d a f e w h o u r s a f t e r d e a t h b y Dr. g. Bigler. A u t o p s y Report: The b o d y is t h a t of a m a r k e d l y d e h y d r a t e d a n d u n d e r n o u r i s h e d M e x i c a n m a l e child a b o u t two y e a r s os age. Over t h e whole occipital p o r t i o n of t h e h e a d t h e r e is a d a r k red~ s l i g h t l y m o i s t area~ in w h i c h t h e r e is no n o r m a l skin. T h e s k i n s u r r o u n d i n g t h i s a r e a is d a r k b l u e to p u r p l e i n color. T h e r e is no hair p r e s e n t on t h e scalp a l t h o u g h t h e r e is evidence in o~e a r e a of a d o w n y fuzz. S c a t t e r e d t h r o u g h o u t t h e scalp a r e d a r k b r o w n p i g m e n t e d areas, s o m e isolated~ other eonglomer~te. A b o u t b o t h eyes, i n c l u d i n g all s k i n f r o m t h e s u p e r i o r orbital r i d g e to
MC ENERY :
PELLAGRA
481
2 cm. below the eye, the skin is rather moist and colored yellow to red. The same skin lesion extends downward covering the whole nose, the complete upper lip, and about three-fourths of the lower lip. The skin over the entire body is pigmented i n n blotchy manner. In some places, the pigmented areas are from a light brown to dark brown in color, and in other places are more of a grayish-white. The pigmented area is very pronounced on the neck, abdomen, and back. The characteristic thing about the whole skin lesion is the symmetrical arrangement. Examination of the other viscera was negative for pathology, except f a t t y degeneration of the liver and cloudy swelling of the kidneys. The intestinal t r a c t was hyperemic and edematous. The thymus was of nqrmal weight and size. Microsvopio examination of skin sections by Dr. C. l~innerud: There is a moderate edema of the papillary eorium and epidermis, and a moderate dilatation and cellular infiltration. I t is surprising that there is not a greater tendency toward retention of the nuclei in the horny ]avers. Here also there are chromatophores in the eorium but they are situated immediately beneath the papillae. The nuclei of the connective tissue cells of the corium are much more numerous and distinct than normal. Many of the deeper vessels of the eorimn are with thickened walls. This thickening is apparently edematous and without increase in the number of cells.
Comment:
This patient
pellagra, brought meat.
presents
an extensive
and
severe
case of
on by a faulty diet deficient in fresh vegetables
The age at which this condition first manifested
With its o,eeurring in t h e C e n t r M W e s t , m a k e s it a r a r e childhood. 707 FULLERTON AVE.
and
itself, together condition of
G R A in children in the Central W e s t is a v e r y r a r e condition, p Ea nLdL Abecause of the unusual oecnrrence of this deficiency disease, the following case is reported. CASE REPot'r: G. E., a 1Viexican boy, aged two years, was first seen on l~ay 3, on account of a skin condition. The mother was living and well. The father had been treated in a sanltarinm, but no positive diagnosis of tuberculosis had been made. There were two children in the family, the patient and a glrl one year old. There was no history of any miscarriages or syphilis in the immediate family. The birth history was uneventful. The child had been on the breast until eight months of age, when he was placed on whole milk, eight ounces with five teaspoonfuls of sugar, four feedings a day. Gradually other foods were added to his diet, such as cereal, soups, beans, rice, apples, bananas, and prunes, but there was no history of any fresh frults~ vegetables, or meat having been given. Cod liver oil had been taken very irregularly. The mother considered the child's appetite good, b u t she had to make things very sweet before he wou]d take any food. Envi.ron.~ner family lived in a box ear. The small home was very clean, and the children were given good care. D evelop~nent.--The first tooth erupted at four months. He sat erect at ten months, but at present he does not walk and talks very little. The mother first noticed the skin condition when the child was weaned from the breast, sixteen months ago, the child then being only eight months of age. Exa~ninatioa showed a very thin, undernourished Mexican boy, sitting erect with his back to the light, eyes closed, with marked photophobia and blepharospasm. There was a complete alopee~a of the scalp. The teeth were in excellent condition, without caries or discoloration. The gums were very soft but did not bleed. The heart and lungs were negative. The abdomen was slightly distended, but the liver and spleen were not palpable. The extremities were thin and undeveloped. The most striking thing about the patient was the symmetrical skin lesion. S]c/~.--The three typical stages of the cutaneous manifestations of pellagra; namely, hyperemia, desquamation, and fissul'es~ were all present and were distributed over the body in the following manner: The face was covered with a masklike lesion, with skin contractures under the eyes so marked as to create a Ibi]atera] ectropion. About the neck the lesion extended in a collar fashion, as described by Cassal. The anterior chest was free from any involvement. Below the umbilicus, it extended downward and outward to both anterior superior spines of the pelvis, then down the mesial portion of the thigh, involving the scrotum, anal region~ and the buttocks. The feet were covered entirely, the lesion extending up to and around the ankle, with a slight extension up the inner side of the leg to the knee. The hands, especially, the dorsum, were involved up to the wrist in a gauntletdike arrangement. (Figs. 1, 2, 3.) Na~t~.re of the Ski~ Les~o~s.--In some areas the skin was involved in a hyperemic stage with slight desquamation; in other parts~ about the scrotum and anus~ there From The Children's IViemorial Hospital. Service Dr. Joseph Brennemann. 478
~ C ENERY:
PELL&G~A
479
were small vesicles and fissures which had become secondarily infected from scratching. The color varied from a rose to a vivid red and dark brown. About the lower eyelids there was an increased amount of pigment with atrophic thinning of the epidermis and a certain amount of fibrosis of the subcutaneous connective tissue, which brought about the bilateral eetropion of the lower lids. Laboratory Datam.--Examination of the blood showed, hemoglobin (Sahli) 80 per cent, W.B.C. 10,600, t~:B.C. 4,300~000, Differential--Poly. 53 per cent, Lymph. 42 per cent, Trans. 4 per cent. :~o abnormal cells present. The Pirquet test was negative. The Wassermann test was negative. The x-ray of the chest was considered normal. An examination of the stools for parasites was negative. Co~rse and Treatment.--The child was put on whole milk s fresh vegetables, orange juice, cod liver oil, and yeast. A t first he refused to take anything solid, and if the
9"ig. 1.--Front view showing symmetrical distribution of lesions. feedings were forced, the lesions about the mouth bled profusely so it was necessary to give food by gavage. His progress was very stow and he would take little or nothing but sweets at first. With a tremendous amount of patience~ he was finally coaxed to take small amounts of fresh vegetables and meat. The skin was treated with bland ointments. Under the r6gime, the cutaneous lesions began to improve. A f t e r being in the hospital for six weeks, the prognosis looked favorable. At t h i s time the following incident took place, which illustrates the harmful effects of sunlight on pellagrinous dermatitis. The child was placed in a solarium for a few hours; two days later t h e skin lesions became worse with a reappearance of vesicles, blebs, and fissures. The p a t i e n t ' s general condition also reacted, with loss of appetite and severe diaa.rhea, which lasted for several days. About a month after this relapse, it was thought that small doses of quartz light might desensitize the skin to such a reaction, so four doses of thirty seconds exposure were given over
480
T t / E JOUICbTAL OF PEDIATRICS
a period o f a week. T h e d a y following t h e last exposure, a n o t h e r reaction, s i m i l a r to t h e one caused b y t h e exposure to s u n l i g h t , took place. T h e e x a c e r b a t i o n of t h e s k i n lesion a n d t h e g a s t r o i n t e s t i n a l s y m p t o m s were m o r e prolonged, a n d his r e t u r n to t a k i n g o f f o o d o t h e r t h a n b y g a r a g e was a m a t t e r of t h r e e weeks. H i s m e n t a l r e a c t i o n d u r i n g all of t h i s period w a s v e r y u n h a p p y . He seldom p l a y e d w i t h his t o y s a n d w a s t h e p i c t u r e of a b j e c t misery. T h e p r o g r e s s of t h e case was u n e v e n t f u l a f t e r t h e exposure to s u n l i g h t a n d q u a r t z light. T h e s k i n i m p r o v e d a~ad t h e a p p e t i t e w a s b e t t e r t h a n i t h a d ever been.
~ig. 2 . - - S h o w i n g lesions on a b d o m e n a n d legs.
Fig. 3.~]~ack view showing distribution a b o u t the a n u s . A complete recovery w a s looked for, w h e a u n e x p e c t e d l y a n d w i t h o u t w a r n i n g the child w a s f o u n d d e a d i n bed, t h r e e m o n t h s a f t e r admission. hreerops~ w a s p e r f o r m e d a f e w h o u r s a f t e r d e a t h b y Dr. g. Bigler. A u t o p s y Report: The b o d y is t h a t of a m a r k e d l y d e h y d r a t e d a n d u n d e r n o u r i s h e d M e x i c a n m a l e child a b o u t two y e a r s os age. Over t h e whole occipital p o r t i o n of t h e h e a d t h e r e is a d a r k red~ s l i g h t l y m o i s t area~ in w h i c h t h e r e is no n o r m a l skin. T h e s k i n s u r r o u n d i n g t h i s a r e a is d a r k b l u e to p u r p l e i n color. T h e r e is no hair p r e s e n t on t h e scalp a l t h o u g h t h e r e is evidence in o~e a r e a of a d o w n y fuzz. S c a t t e r e d t h r o u g h o u t t h e scalp a r e d a r k b r o w n p i g m e n t e d areas, s o m e isolated~ other eonglomer~te. A b o u t b o t h eyes, i n c l u d i n g all s k i n f r o m t h e s u p e r i o r orbital r i d g e to
MC ENERY :
PELLAGRA
481
2 cm. below the eye, the skin is rather moist and colored yellow to red. The same skin lesion extends downward covering the whole nose, the complete upper lip, and about three-fourths of the lower lip. The skin over the entire body is pigmented i n n blotchy manner. In some places, the pigmented areas are from a light brown to dark brown in color, and in other places are more of a grayish-white. The pigmented area is very pronounced on the neck, abdomen, and back. The characteristic thing about the whole skin lesion is the symmetrical arrangement. Examination of the other viscera was negative for pathology, except f a t t y degeneration of the liver and cloudy swelling of the kidneys. The intestinal t r a c t was hyperemic and edematous. The thymus was of nqrmal weight and size. Microsvopio examination of skin sections by Dr. C. l~innerud: There is a moderate edema of the papillary eorium and epidermis, and a moderate dilatation and cellular infiltration. I t is surprising that there is not a greater tendency toward retention of the nuclei in the horny ]avers. Here also there are chromatophores in the eorium but they are situated immediately beneath the papillae. The nuclei of the connective tissue cells of the corium are much more numerous and distinct than normal. Many of the deeper vessels of the eorimn are with thickened walls. This thickening is apparently edematous and without increase in the number of cells.
Comment:
This patient
pellagra, brought meat.
presents
an extensive
and
severe
case of
on by a faulty diet deficient in fresh vegetables
The age at which this condition first manifested
With its o,eeurring in t h e C e n t r M W e s t , m a k e s it a r a r e childhood. 707 FULLERTON AVE.
and
itself, together condition of