Acrodermatitis enteropathica–like eruption and failure to thrive as presenting signs of cystic fibrosis

Zedek, Daniel C.; Morrell, Dean S.; Graham, M. J.; Goodman, David A.; Groben, Pamela A.

doi:10.1016/j.jaad.2006.04.038

Cited by 30 publications

(16 citation statements)

References 31 publications

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“…2,[4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19] The dermatitis typically presents as erythematous, scaling papules that later progress to extensive coalescing, desquamating plaques. The eruption is usually first noted in the perineum anywhere from several days to 15 months after birth, with subsequent spread to the perioral region, extremities, and trunk.…”

Section: Commentmentioning

confidence: 99%

“…When it occurs, low zinc levels in CF may be attributed to decreased release and absorption of zinc from dietary protein secondary to pancreatic insufficiency. 17 In addition, steatorrhea may impair reabsorption of endogenously secreted zinc, possibly via the formation of insoluble fat-zinc complexes. 22 In support of this hypothesis, Krebs et al 22 found that high losses of endogenous zinc associated with fat malabsorption were present in the feces of infants with CF.…”

Section: Commentmentioning

confidence: 99%

“…23 These observations may partially explain why oral zinc supplementation alone failed to improve the cutaneous lesions in several cases of CF before the diagnosis was made, despite the presence of zinc deficiency. 5,8,17 Deficiencies in EFAs have also been documented in patients with CF. 24 The exact cause of these abnormalities has been the subject of debate, with some studies attributing them to malabsorption 24 and others accrediting them to defects in fatty acid metabolism.…”

Section: Commentmentioning

confidence: 99%

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Cystic Fibrosis Presenting With Dermatitis

Wenk¹,

Higgins²,

Greer³

2010

Arch Dermatol

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Background Patients with cystic fibrosis classically present with evidence of pulmonary disease, exocrine pancreatic insufficiency, and high sweat chloride concentrations. Dermatitis as an initial manifestation of the disease is uncommon and has been attributed to multiple nutritional deficiencies. Observation We describe the case of a 3-month-old female infant with cystic fibrosis presenting with dermatitis in the setting of protein-energy malnutrition. A review of the laboratory study results in this case and others showed that a deficiency in zinc, essential fatty acids, and protein likely contributes to the development of the rash seen in cystic fibrosis. Conclusions Given the frequent delay in diagnosis, as well as the increased morbidity and mortality associated with protein-energy malnutrition in these patients, it is important to consider cystic fibrosis as a possible diagnosis in any infant presenting with a rash and other signs of malnutrition. The relative contribution of specific nutritional deficiencies and the degree to which they influence and interact with each other in producing the dermatitis remain unclear, although they may all affect a common underlying metabolic pathway

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Section: Commentmentioning

confidence: 99%

Section: Commentmentioning

confidence: 99%

Section: Commentmentioning

confidence: 99%

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Cystic Fibrosis Presenting With Dermatitis

Wenk¹,

Higgins²,

Greer³

2010

Arch Dermatol

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“…Up to 10% of patients with CF presented with hypoproteinemia, delayed development, anemia, and edema due to the kwashiorkor caused by pancreatic insufficiency. Cutaneous symptoms as a presenting sign in CF are rare [1,5,6,12,15]. The skin eruption is usually seen between the 3rd to 7th months of age and is seen about a month before edema and severe malnutrition.…”

Section: Discussionmentioning

confidence: 99%

A 4-month-old boy with acrodermatitis enteropathica-like symptoms

et al. 2008

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“…2 They are more prone to illnesses caused by zinc deficiencies, such as acrodermatitis, which improve with supplementation. [3][4][5] Deficiency of micronutrients, including zinc, is associated with an increased risk of infection. However, data on zinc supplementation among children with cystic fibrosis, although limited, suggest that the results of supplementation are variable in terms of improvement in pulmonary function test or reduction in the need for antibiotics.…”

Section: Introductionmentioning

confidence: 99%

Zinc Supplementation for One Year Among Children with Cystic Fibrosis Does Not Decrease Pulmonary Infection

Sharma¹,

Lodha²,

Shastri³

et al. 2015

Respir Care

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BACKGROUND: Children with cystic fibrosis may have a deficiency of micronutrients, including zinc, which may affect their susceptibility to infections. There is a paucity of data on zinc supplementation among children with cystic fibrosis. We hypothesized that a pharmacologic dose of zinc administered daily for 12 months would reduce the need for antibiotics by 50%. METHODS: This double-blind randomized placebo-controlled trial was conducted among children with cystic fibrosis to assess the effect of zinc supplementation on the need for antibiotics and pulmonary function tests. The children, age 5-15 y, of either sex, received either 30-mg zinc tablets or similar looking placebo tablets daily in addition to standard care. They were followed up every month for a period of 12 months and whenever they had pulmonary exacerbations. Their serum zinc was estimated at baseline and at 12 months of enrollment. During each visit, the children underwent a pulmonary function test and sputum culture. RESULTS: Of a total of 43 children screened, 40 were enrolled, and of them, 37 completed the study. The median (interquartile range) number of days of the administration of antibiotics over 12 months of follow-up among the children receiving zinc was 42 (14 -97) d. In the placebo group, it was 38 (15-70) d (P ‫؍‬ .79). There were no significant differences in the percent-of-predicted FEV 1 or change in FEV 1 values at 12 months (P ‫؍‬ .44). The number of children in whose respiratory specimens Pseudomonas was isolated was similar for the 2 groups at different time intervals. The adverse events reported were similar in the 2 groups. CONCLUSION: We did not find any significant difference in the need for antibiotics, pulmonary function tests, hospitalization, colonization with Pseudomonas, or the need for antibiotics for children with cystic fibrosis receiving zinc supplementation of 30 mg/d.

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Acrodermatitis enteropathica–like eruption and failure to thrive as presenting signs of cystic fibrosis

Cited by 30 publications

References 31 publications

Cystic Fibrosis Presenting With Dermatitis

Cystic Fibrosis Presenting With Dermatitis

A 4-month-old boy with acrodermatitis enteropathica-like symptoms

Zinc Supplementation for One Year Among Children with Cystic Fibrosis Does Not Decrease Pulmonary Infection

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