The Acrodermatitis Enteropathica-like Syndrome

Brazin, Stewart A.

doi:10.1001/archderm.1979.04010050031012

Cited by 16 publications

(6 citation statements)

References 24 publications

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“…Malabsorption can occur secondary to high‐fiber diet or to malabsorption syndromes, such as cystic fibrosis 5 . Acquired zinc deficiency has also been associated with chronic renal failure, malignancy, drugs, ethanol, pregnancy, 6,7 malnutrition 8 and total parenteral nutrition 9,10 . Occasionally, zinc deficiency can be seen in infants fed maternal milk; these patients suffer from low zinc levels in the maternal milk and they improve clinically when weaned off breast milk.…”

Section: Discussionmentioning

confidence: 99%

“…A summary of the literature on AE is given in Table 1, including type of lesions, age at onset or presentation, presence of infection, whether the AE was considered innate or acquired, whether vesicles or bullae were described and, when reported, any histopathological findings 5–8,10–13,15–38 …”

Section: Clinical and Histopathological Findings As Reported In Litermentioning

confidence: 99%

“…The reported clinical features of AE have been described mainly through isolated case reports and range clinically from erythematous, scaly patches to psoriasiform plaques and erosive to vesiculobullous lesions 3,14 . Corresponding to the broad spectrum of clinical lesions of AE, histopathological descriptions in the literature are equally varied and include hyperkeratosis, 10,11,14 parakeratosis, 8,10,11,39 acantholysis, 10,11,12 epidermal pallor 7,40 and dilated and tortuous capillaries 8,14,41 …”

Section: Clinical and Histopathological Findings As Reported In Litermentioning

confidence: 99%

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Bullous lesions in acrodermatitis enteropathica delaying diagnosis of zinc deficiency: a report of two cases and review of the literature

et al. 2008

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Acrodermatitis enteropathica (AE) is a rare disorder associated with poor absorption of zinc. A variety of clinical and histological findings have been reported in the literature, described mainly in isolated case reports. Because of the varied nature of these cases, the histological features of AE are described often as non-specific. We describe lesions of AE in two patients who presented with vesiculobullous and erosive skin lesions, both showing intra-epidermal, inflammatory vesiculation with surrounding eosinophilic epidermis and necrotic keratinocytes. The lack of clinical suspicion of AE led to their misdiagnosis. We present these two patients to further characterize the bullous variant of AE, and we review the previously reported clinical and histopathological findings.

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Section: Discussionmentioning

confidence: 99%

Section: Clinical and Histopathological Findings As Reported In Litermentioning

confidence: 99%

Section: Clinical and Histopathological Findings As Reported In Litermentioning

confidence: 99%

See 1 more Smart Citation

Bullous lesions in acrodermatitis enteropathica delaying diagnosis of zinc deficiency: a report of two cases and review of the literature

et al. 2008

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“…In the eariy stages of acrodermatitis enteropathica there are vesicobullous lesions on the fingertips and around the nails. Paronychia and nail dystrophy develop in almost all cases (36), often with Beau's lines marking acute episodes of zinc deficiency (37). Nail shedding may occur during acute inflammatory episodes affecting the nail unit.…”

Section: Thumb Sucking and Paronychiamentioning

confidence: 99%

Diseases of the Nails in Children

Barth

Dawber

1987

Pediatric Dermatology

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The nails have numerous functions ranging from cosmetic adornment to tools. Their most important function is to splint the soft pulp of the terminal phalanx to aid fine grasp and manipulation, which may be impaired by a deformed or damaged nail. This review of nail diseases begins with an account of the embryology, anatomy, and physiology of the nail. The presentation of the sections of diseases is somewhat arbitrary as there is at present no satisfactory classification of nail disease.

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“…The results of the light microscopic studies of AE induced by dietary zinc defects have been variable. The epidermal changes were described as eczcmatoid, degenerative, acantholytic, dyskeratotic or edematous (11)(12)(13)(14)(15). The first to describe the pallor of upper epidermal cells was Sjolin (16).…”

Section: Discussionmentioning

confidence: 99%

Histopathological study of transient acrodermatitis enteropathica due to decreased zinc in breast milk

Kanerva

et al. 1989

J Cutan Pathol

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Two children, one born prematurely and the other born at full term developed acrodermatitis enteropathica due to marginal or low levels of zinc in their mothers' breast milk. Skin from both patients was studied with light and electron microscopy. The most characteristic light microscopic features were parakeratosis, absence of the granular layer, and pallor of the upper epidermal cells. Normal flattening of the upper Malphighian cells did not occur. Electron microscopic examination revealed that keratohyalin was decreased to absent, and the upper malpighian cells were edematous with vacuoles and large numbers of ribosomes, but small numbers of tonofilaments. Large amounts of keratinosome-derived-lamellae were found in the intercellular spaces in the keratinization area. The keratinosome-derived-lamellae were focally intermingled with opaque lipid plaques or myelin figures, probably derived from keratinosomes. The electron microscopic findings show abnormal keratinization and suggest that it is related to a disturbance of keratinosome metabolism due to zinc deficiency.

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The Acrodermatitis Enteropathica-like Syndrome

Cited by 16 publications

References 24 publications

Bullous lesions in acrodermatitis enteropathica delaying diagnosis of zinc deficiency: a report of two cases and review of the literature

Bullous lesions in acrodermatitis enteropathica delaying diagnosis of zinc deficiency: a report of two cases and review of the literature

Diseases of the Nails in Children

Histopathological study of transient acrodermatitis enteropathica due to decreased zinc in breast milk

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