Acrodermatitis enteropathica (danbolt-closs) in five siblings

Vedder, James S.

doi:10.1016/s0022-3476(56)80167-4

Cited by 25 publications

(8 citation statements)

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“…It appears as if the onset of puberty improves conditions in some patients [9,10,12], as is also illustrated by our case 2. Since the zinc supplement necessary to maintain patients symptom-free is at a level twice that of the daily requirements in healthy humans [20], small changes in the supply of zinc in the food as well as changes in the organism's need for zinc, for example during growth periods and pregnancy, and in infectious diseases, probably play a part in modifying the course of the disease in the individual patient.…”

Section: Commentsupporting

confidence: 82%

“…In case 2, her four pregnancies were accompanied by an exacerbation of her skin disease. Similar cases have been described by others [10,21,22]. In case 2 the two children were born without congenital malformations.…”

Section: Commentsupporting

confidence: 81%

“…Vedder et ai [10] described, in 1956, a family with nonconsanguineous parents, where 5 of 11 children had AE. The second child, a girl, first develop ed her skin disease at the age of 8 months while being weaned.…”

Section: Review O F Reported Casesmentioning

confidence: 99%

“…However, this has been described in children of mothers with AE. In I case a mother gave birth to a stillborn anencephalic child after two spontaneous abortions [20], and in another case an achondroplastic dwarf was born who died shortly after birth [10], Zinc deficiency in rats is accompanied by a high rate of abortion and congenital malformation [23] especially in the central nervous system [24].…”

Section: Commentmentioning

confidence: 99%

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Untreated Acrodermatitis Enteropathica in Adults

Ølholm-Larsen¹

1978

Dermatology

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In the literature six cases of acrodermatitis enteropathica (AE) are described in which the patients survived to adult age. In a few of these cases the symptoms disappeared at the onset of puberty, while in the rest the disease had a fluctuating course with long symptom-free intervals and an uncharacteristic symptomatology. Two cases are reported. In case 1 there remained only characteristic skin changes on the feet at adult age, in case 2 both skin and intestinal symptoms disappeared at adult age. For long periods this patient was invalidated through depressions, a symptom which is characteristic of children suffering from an active AE. The same patient developed parkinsonism at a relatively young age. In both cases a very low serum zinc level essentially supported the diagnosis. It is possible that AE in adults is underdiagnosed because of an uncharacteristic symptomatology.

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Section: Commentsupporting

confidence: 82%

Section: Commentsupporting

confidence: 81%

Section: Review O F Reported Casesmentioning

confidence: 99%

Section: Commentmentioning

confidence: 99%

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Untreated Acrodermatitis Enteropathica in Adults

Ølholm-Larsen¹

1978

Dermatology

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“…Because the symptoms in some females improved at menarche and exacerbated with pregnancy, Vedder9 suggested that a hormonal factor may influence the progress of the disease. He suggested that this diminished enzyme activity could result in the absorption of a toxic polypeptide that produces the manifestations of this disease.…”

mentioning

confidence: 99%

Acrodermatitis Enteropathica

Idriss

Kaloustian

1973

Clin Pediatr (Phila)

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ACRODERMATITIS enteropathica was first recognized as a separate entity by Dan-bolt and Close in 1942, although cases had been previously described in the literature under a variety of titles. The syndrome comprises cutaneous eruption (usually peripheral and hence the term &dquo;acro&dquo;), gastrointestinal symptoms, failure to thrive, and apathy. The disease begins between the age of one week and ten years, females being affected more than males. Most cases have a positive family history with consanguinity in a few of them.The inheritance appears to follow the autosomal recessive pattern.7, III In this paper, we are reporting three new patients with acrodermatitis enteropathica. With all three cases, the parents were first cousins, though the pairs of parents were not related to each other. Case ReportsCase 1. N. A. S., a five-month-old girl, was admitted to the American University Hospital of Beirut because of a skin eruption that started around the mouth and progressed to appear on the arms, the feet, the elbows, and the hands. These lesions did not respond to topical therapy with antibiotics and corticosteroids. Later, diarrhea and vomiting began. The infant had been breast fed until the age of two months and the lesions appeared after the change to cow's milk. The parents were first cousins.On physical examination, she was underdeveloped, sick looking, and irritable. Her elbows, feet, hands, and the skin about her body orifices were covered with erythematous, scaly, dry, and elevated skin lesions. She had lost her eyebrows and her hair over the occipitonuchal region.Laboratory data revealed a normal hemogram and urinalysis. Proteus organisms and yeast-like fungi were cultured from the skin. The stool culture grew Proteus organisms but no fungi. Hexa-chlorophene (3%) soap, was used for local cleansing of the skin lesions.Since the skin lesions were consistent with acrodermatitis enteropathica, di-iodohydroxyquinoline by mouth, I g daily, was started. One month after initiation of therapy, the skin lesions had cleared and the diarrhea had subsided. This remission lasted for five months, until di-iodohydroxyquinoline was discontinued; relapse then occurred. When the drug was started again, the patient improved. She has been lost to follow-up.Case 2. Sh. R., a nine-month-old boy, was breast fed till the age of two months, then changed to cow's milk. Soon afterwards, erythematous vesicular skin lesions appeared over the face and extremities. These lesions did not respond to therapy with antibiotics and corticosteroids, and bouts of gastroenteritis began. The parents were first cousins.Physical examination revealed an underdeveloped and irritable infant whose face, extremities, and perineum were covered with erythematous, vesicular, and crusty skin lesions.Laboratory data revealed a normal hemogram and urinalysis. The skin biopsy was consistent with acrodermatitis enteropathica. Immunoelectrophoresis revealed qualitative absence of IgM. Skin culture grew Pseudomonas pyocyaneus.Gentamycin parenterally was used with...

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