Acute onset of blisters in an infant with acrodermatitis enteropathica: A case report

Cleminson, Kristyna; Hull, Peter; Price, Emma L.; Green, Peter J.

doi:10.1177/2050313x20984119

Cited by 2 publications

(1 citation statement)

References 5 publications

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“…Skin is the third most zinc-abundant tissue in our body (skeletal muscle 60%, bones 30%, liver 5%, and skin 5%) [13]. It is involved in epidermal keratinocyte differentiation and growth as well as in antiin ammatory processes and wound healing [14] and is crucial for epidermal stem cells [15]. Knowing its roles, it's clear that a zinc de ciency can cause various clinical manifestations, which can involve every organ and system.…”

Section: Discussionmentioning

confidence: 99%

Acrodermatitis enteropathica during parenteral nutrition: a pediatric case report

Proli

Margiotta

Ferretti

et al. 2023

Preprint

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Background: Acrodermatitis enteropathica is a rare disorder characterized by the triad composed by dermatitis, alopecia and diarrhoea. Its acquired form can be caused by inadequate zinc intake, malabsorptive processes, excessive renal or intestinal loss. A rare cause of acquired zinc deficiency is iatrogenic nutritional deficiency due to parenteral nutrition. The diagnosis can be really difficult because the early clinical signs are non-specific and patient’s eventual comorbidities can often mask symptoms. Case presentation: A 5-years-old child affected by several comorbidities, consequent to C. Koserimeningo-encephalitis occurred in the neonatal period, was admitted to Pediatric ward for acute pancreatitis and he had been fed via total parenteral nutrition for one month. Symptoms started approximately 15 days after the start of a standardised standardized parenteral nutrition mixture. The child presented with diarrhoea, alopecia and erythematous bullous skin lesions, distributed predominantly in acral and periorificial sites and not responsive to topical treatments. Zinc serum dosage were very low (10 µg/dL, with normal values 68-107 µg/dL). Clinical improvement was very fast after oral zinc supplementation (5mg/daily), with a rapid regularisation in the intestinal habits and re‐epithelialization of the skin lesions. Conclusion: Trace elements are an essential component of parenteral nutrition. The supplementation of trace elements is a complex and important part of the parenteral nutrition prescription. Even few days of zinc shortage, especially in frail patients, may cause a severe dermatitis that can be easily prevented. Despite its rarity, acrodermatitis enteropathica should be strongly considered in the differential diagnosis of skin lesions for these patients.

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Section: Discussionmentioning

confidence: 99%

Acrodermatitis enteropathica during parenteral nutrition: a pediatric case report

Proli

Margiotta

Ferretti

et al. 2023

Preprint

View full text Add to dashboard Cite

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Acrodermatitis enteropathica – diagnostic challenges

Sztupecka-Rutkowska

Wojciechowska

Heropolitańska-Pliszka

et al. 2023

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Background: Acrodermatitis enteropathica is a rare genetic metabolic disorder which results in poor absorption of zinc ions in digestive tract. Zinc is an important microelement in human body, that plays a crucial role in many metabolic and biochemical pathways in organism. Classic clinical manifestation of zinc deficiency, seen in about 20% of cases, is the triad of symptoms – acral and periorificial dermatitis, diarrhea and alopecia. Results: Here, we report a case of 16 months-old girl with atypical course of acrodermatitis enteropathica. First skin lesions appeared after infancy and initially presented as blisters treated as erythema multiforme without success. In that time epidermolysis bullosa was also considered by doctors. One month later the patient developed more severe clinical picture containing erythematous plaques, paronychia, alopecia suggesting acrodermatitis enteropathica, however laboratory test showed zinc serum level within reference ranges. Since anti-inflammatory topical corticosteroids and antibiotics used for a few weeks were not effective, further diagnostic procedures were introduced. Direct immunofluorescence excluded autoimmune skin disorders, whereas genetic study showed pathogenic mutation characteristic of acrodermatitis enteropathica in SCL39A4 gene. Although serum zinc level was normal, we observed theatrical improvement of healing skin lesions only after initiation of oral supplementation of zinc. Conclusions: Blisters and erythematous plaques especially located in traumatized areas developing in children may suggest several severe disorders like epidermolysis bullosa, linear IgA bullous dermatosis, epidermolytic ichthyosis, erythema multiforme, therefore we recommend rapid diagnostics containing immunological, genetic and biochemical tests in such cases, because making a proper diagnosis allows rapid initiation of a proper treatment and can improve life quality of patients and prevent distant consequences like mental and physical retardation in cases of metabolic disorders.

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Acute onset of blisters in an infant with acrodermatitis enteropathica: A case report

Cited by 2 publications

References 5 publications

Acrodermatitis enteropathica during parenteral nutrition: a pediatric case report

Acrodermatitis enteropathica during parenteral nutrition: a pediatric case report

Acrodermatitis enteropathica – diagnostic challenges

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