Acrodermatitis enteropática: caso clínico y revisión de la literatura

Valdés, Roberto; Mauret, Michelle; Castro, Álex

doi:10.4067/s0034-98872013001100017

Cited by 6 publications

(3 citation statements)

References 10 publications

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“…The differential diagnoses to consider are psoriasis, atopic dermatitis, seborrheic dermatitis, contact dermatitis, skin infections with candida, Langerhans cell histiocytosis, and cystic fibrosis. 12 Once the disease has been diagnosed, the treatment consists of oral zinc supplementation given daily, which leads to a dramatic disappearance of the symptoms within a few days. With treatment, the survival rate is 100%.…”

Section: Discussionmentioning

confidence: 99%

Acrodermatitis Enteropathica

Nistor

Ciontu²,

Frăsinariu³

et al. 2016

Medicine

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Acrodermatitis enteropathica is a rare genetic autosomal recessive disorder, characterized by periorificial dermatitis, alopecia, and diarrhea. It is caused by mutations in the gene that encodes a membrane protein that binds zinc. We report a 14-month-old boy, admitted for erythematous, scaly and pustular lesions, initially located in the inguinal and perianal regions and on thighs, and very few erythematous lesions on the face. Due to the numerous bacterial skin superinfections with Staphylococcus aureus, including abscesses that required surgical incision, the clinical picture was modified, leading to a delayed establishment of the diagnosis. Later, the symptoms became suggestive for this disease, the diagnostic having been confirmed by low plasma zinc values. Under zinc therapy, skin lesions improved significantly in a few days, with favorable outcome. Two months later, the skin lesions almost disappeared.Abscesses due to bacterial skin superinfections may lead to initially misdiagnosed acrodermatitis enteropathica.

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Section: Discussionmentioning

confidence: 99%

Acrodermatitis Enteropathica

Nistor

Ciontu²,

Frăsinariu³

et al. 2016

Medicine

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“…psoriasis, atopic dermatitis, seborrheic dermatitis, Langerhan cell histiocytosis, and other nutritional dermatoses (such as biotin and essential fatty acid deficiency, pellagra, cystic fibrosis, and organic acidurias). 4 In both hereditary and acquired cases, immediate administration of supplemental zinc treatment is essential, and rapid and dramatic improvement is expected. For cases of hereditary deficiency, zinc replacement treatment is initiated at 3 mg/kg/d of elemental zinc and continued indefinitely with monitoring of serum zinc levels and zinc-dependent enzymes (alkaline phosphatase) every 3 to 6 months.…”

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confidence: 99%

“…Acquired deficiency is also associated with prematurity, low birth weight, exclusive parenteral nutrition, and malabsorption states . The clinical differential diagnoses may include psoriasis, atopic dermatitis, seborrheic dermatitis, Langerhan cell histiocytosis, and other nutritional dermatoses (such as biotin and essential fatty acid deficiency, pellagra, cystic fibrosis, and organic acidurias) …”

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confidence: 99%

Acquired Acrodermatitis Enteropathica

Guliani¹,

Bishnoi

2019

JAMA Dermatol

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A 6-month-old exclusively breastfed boy was brought to the dermatology department for evaluation of red, oozy plaques over his face, genitals, and extremities of 4 months duration. He was small for gestational age at birth, and was born of a nonconsanguineous marriage. At presentation, his length was 58 cm and weight was 4.5 kg (both below the third percentile for age). Cutaneous examination revealed sharply demarcated, fissured, erythematous plaques on the perioral, perianal, and inguinal skin, some with associated honey-colored crusting (Figure). In addition, paronychia and diffuse alopecia and crusting of the parieto-occipital scalp was noted.Based on these findings, serologic studies were obtained and the results were notable for a serum zinc level of 3.12 μmol/L (normal range, 10.71-17.59 μmol/L), a 24-hour urinary zinc level of 41.4 μg (normal range, 150-1200 μg/24 hr), and an alkaline phosphatase level of 878.3 nkat/L (normal range, 883.3-2133.3 nkat/L). Elemental zinc treatment was initiated at a dose of 3 mg/kg/d, which resulted in rapid clinical resolution over 2 weeks. The dose was then reduced to 1 mg/kg/d with planned discontinuation when the infant was no longer breastfeeding.Zinc deficiency can either be acquired or inherited, as in the case of acrodermatitis enteropathica. Both forms classically present with periorificial and acral dermatitis, diarrhea, and alopecia, though the complete triad is only noted in approximately 20% of cases. 1 Hereditary disease results from a deficiency of the enterocyte zinc transporter and should be suspected in formula-fed infants presenting with features associated with the disease in the first few days of life or breastfed infants after they are weaned from maternal milk (zinc is more bioavailable in breast milk than in other sources). 2 Acquired cases result from dietary deficiency. In infancy, this is often self-limited and frequently related to insufficient maternal zinc levels. As such, affected infants are typically breastfed at the time of presentation. 2 Acquired deficiency is also associated with prematurity, low birth weight, exclusive parenteral nutrition, and malabsorption states. 3 The clinical differential diagnoses may include

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Acrodermatitis enteropática en un lactante, un diagnóstico a considerar

Andrade¹,

Eslava²

2019

Piel

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Acrodermatitis enteropática: caso clínico y revisión de la literatura

Cited by 6 publications

References 10 publications

Acrodermatitis Enteropathica

Acrodermatitis Enteropathica

Acquired Acrodermatitis Enteropathica

Acrodermatitis enteropática en un lactante, un diagnóstico a considerar

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