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Observation |

Cystic Fibrosis Presenting With Dermatitis FREE

Kurt S. Wenk, MD; Kristen B. Higgins, MD; Kenneth E. Greer, MD
[+] Author Affiliations

Author Affiliations: Departments of Medicine (Dr Wenk) and Dermatology (Drs Higgins and Greer), University of Virginia Health System, Charlottesville. Dr Wenk is now with the Department of Dermatology, Medical Faculty Associates, George Washington University, Washington, DC. Dr Higgins is in private practice in Charlotte, North Carolina.


Arch Dermatol. 2010;146(2):171-174. doi:10.1001/archdermatol.2009.361.
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Published online

ABSTRACT

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.

Figures in this Article

Cystic fibrosis is an autosomal recessive disease resulting from mutation of the cystic fibrosis (CF) transmembrane conductance regulator gene (CFTR; OMIM 602421) on chromosome 7, leading to the production of a defective epithelial chloride channel.1 The condition classically manifests as a triad of clinical findings, including chronic pulmonary disease, exocrine pancreatic insufficiency, and abnormally high sweat electrolyte concentrations.2 Less frequently, patients present with protein-energy malnutrition (PEM) characterized by edema, anemia, and hypoproteinemia.3 PEM occurs in 5% to 13% of infants with CF and is associated with increased morbidity and mortality.3,4 Dermatitis as an initial presentation of CF is rare and has been attributed to deficiencies in zinc, protein, and essential fatty acids (EFAs).2,5,6 The rash typically occurs 1 month before the onset of edema and other clinical manifestations.2 Given the increased morbidity and mortality associated with this condition, prompt recognition and initiation of treatment is necessary. We describe the case of a 3-month-old girl with CF presenting with dermatitis and PEM.

REPORT OF A CASE

A 3-month-old female infant was referred for evaluation of a rash and failure to thrive. She was born at 395/7 weeks’ gestation (birth weight, 3289 g [50th percentile]; birth length, 50.8 cm [60th percentile]) to nonconsanguineous parents and was exclusively breast-fed until started on a regimen of iron drops and supplemental formula 1 week before presentation. The rash began several days after birth and initially started as a small erythematous macule over the right buttock that subsequently spread to involve the entire buttocks, genitals, and thighs. In the week before admission, the infant developed similar lesions on her trunk, on her proximal upper extremities, and underneath her chin. The lesions had been treated with a number of medications, including topical corticosteroids, antifungal cream, and pimecrolimus with no resolution. In addition, her parents reported a 4-day history of swelling first noted on her feet, which then affected her legs, arms, and face. She had a lifelong history of green, watery stools, as well as a history of gastroesophageal reflux disease since the age of 3 weeks that was treated with a histamine2 blocker. Her parents noted increased fussiness and hunger in the weeks immediately preceding presentation.

At the time of admission, her body weight, head circumference, and length were 4.11 kg (<3rd percentile), 37 cm (<3rd percentile), and 57 cm (20th percentile), respectively. Physical examination revealed coalescing erythematous plaques with overlying desquamation covering the buttocks and genitourinary region (Figure), with smaller erythematous plaques dispersed diffusely across her anterior torso, arms, hands, and chin. Her face and extremities were markedly edematous. Nails and mucous membranes were normal, and she had no evidence of hepatomegaly or alopecia. Abnormal laboratory values included the following: hemoglobin, 7.6 g/dL (reference range, 10.0-18.0 g/dL); hematocrit, 24.2% (reference range, 31.0%-55.0%); total protein, 3.0 g/dL (reference range, 6.0-8.3 g/dL); albumin, 1.2 g/dL (reference range, 3.5-5.7 g/dL); prealbumin, <7.0 mg/dL (reference range, 18-45 mg/dL); aspartate aminotransferase, 49 U/L (reference range, 13-39 U/L); and increased fecal fat. Her zinc status was not determined until several days after treatment initiation and was 43.6 μg/dL (reference range, 67.0-124.0 μg/dL). The white blood cell count (14 600/μL [reference range, 5000-19 500/μL]), alanine aminotransferase (43 U/L [reference range, 7-52 U/L]), alkaline phosphatase (355 U/L [reference range, 120-450 U/L]), and total bilirubin (0.4 mg/dL [reference range, 0.3-1.2 mg/dL]) were within normal limits. A sweat chloride test was unsuccessful, but a CF polymerase chain reaction test was positive for the homozygous ΔF508 mutation, confirming the diagnosis of CF. (To convert alanine aminotransferase, alkaline phosphatase, and aspartate aminotransferase to microkatals per liter, multiply by 0.0167; albumin, hemoglobin, and total protein to grams per liter, multiply by 10; total bilirubin to micromoles per liter, multiply by 17.104; hematocrit to a proportion of 1.0, multiply by 0.01; prealbumin to milligrams per liter, multiply by 10; white blood cell count to ×109 per liter, multiply by 0.001; and zinc to micromoles per liter, multiply by 0.153).

Place holder to copy figure label and caption
Figure.

Coalescing erythematous plaques with overlying desquamation involving the buttocks, genitourinary region, and proximal extremities.

Graphic Jump Location

The infant was initially treated with an elemental formula via nasogastric tube for her malnutrition but was switched to breast milk, supplemental enzymes, and vitamins after her diagnosis was made. On discharge from the hospital, she exhibited marked improvement with less edema, resolving lesions, and improved nutritional status.

COMMENT

Dermatitis as an initial manifestation of CF is rare and has been previously reported in 24 other known patients.2,419 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. Infants often have a history of several large, loose stools per day occurring at the onset or shortly after development of the lesions. Other findings of PEM, including failure to thrive and pitting edema of the extremities and face, are nearly universal by the time of presentation, whereas alopecia2,5,6,8,9,13,16 and hepatomegaly5,6,9,13,16 are less consistently observed. Nail and mucous membrane involvement has not been reported, and laboratory values almost always show evidence of anemia and hypoproteinemia.

The cutaneous findings in CF are thought to be caused by a combination of zinc, protein, and EFA deficiencies,2,5,6 although the exact pathogenesis of the dermatitis is unclear. It is well known that deficits in zinc levels can produce a dermatitis similar to that noted in our case. This is perhaps best seen in acrodermatitis enteropathica, an autosomal recessive disorder associated with decreased intestinal zinc absorption due to mutation of the SLC39A4 gene on chromosome 8q24.3.20 This gene is responsible for producing a transmembrane protein within intestinal enterocytes that facilitates zinc uptake.20 The observed eruption consists of erythematous dry, scaly plaques in a periorificial and acral pattern on the face, scalp, hands, feet, and anogenital regions. Several conditions leading to an acquired zinc deficiency have presented with similar cutaneous findings and include intestinal malabsorption syndromes, prematurity, defects in mammary zinc secretion, prolonged total parenteral nutrition administration, and anorexia.2,7

In cases in which zinc was measured, 12 of 16 infants with CF presenting with dermatitis had decreased levels,2,59,13,16,17 suggesting that deficiency in this nutrient likely contributes to the cutaneous findings. Mild to moderate zinc deficiency in infants with CF who do not have skin lesions has also been described.21 Accurate assessment of an individual's zinc status is complicated by the fact that blood levels decrease during states of inflammation and hypoalbuminemia, which are both often present in these infants. 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 al22 found that high losses of endogenous zinc associated with fat malabsorption were present in the feces of infants with CF. In children with CF and pancreatic insufficiency, they also found decreased fractional absorption of exogenous zinc that subsequently increased with pancreatic enzyme replacement therapy.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 malabsorption24 and others accrediting them to defects in fatty acid metabolism.25 Essential fatty acid deficiency associated with dermatitis has been observed in patients with CF2,6 and in other studies,26 producing lesions similar to those seen with zinc deficiency. Essential fatty acid status was determined in 2 of 24 reported cases of CF presenting with dermatitis, and the infants in both of those cases were found to have low EFA levels.2,6 However, given the high prevalence of fatty acid abnormalities in individuals with CF who do not present with lesions (≤85% in those with evidence of malabsorption24 and 47%27 to 67%28 in randomly screened patients with CF), it is likely that other factors also contribute to the dermatitis seen in these patients.

Most cases of CF complicated by malnutrition and dermatitis have had evidence of protein deficiency as manifested by hypoalbuminemia and hypoproteinemia. These findings have led to the comparison of this condition with kwashiorkor, an edematous state of malnutrition that is associated with deficiency in protein and other nutrients. The characteristic skin lesions associated with kwashiorkor can resemble those seen in infants with CF. However, individuals with kwashiorkor may present with additional findings, including softening and thinning of the nails, glossitis, cheilitis, and perlèche.5,29 Similar to CF-associated dermatitis, the dermatologic manifestations seen in kwashiorkor may reflect multiple nutritional deficiencies, including a lack of vitamins and minerals commonly associated with protein-rich foods. Hypozincemia, seen often in kwashiorkor,30 is thought to at least partially contribute to the cutaneous findings, and topical application of zinc has been shown to lead to more rapid healing of kwashiorkor skin lesions.31

In addition to skin changes, kwashiorkor has other clinical features that are similar to those seen in infants with CF and PEM, including edema, anemia, alopecia, elevated liver enzyme levels, and failure to thrive. Indeed, many researchers consider these infants to have a diagnosis of kwashiorkor secondary to malabsorption.9,16 The edema seen in both conditions may have a common underlying cause involving free radical–mediated damage to cell membranes leading to an increased leak of electrolytes and altered fluid homeostasis.32 Anemia is also found in both states and likely arises because of decreased globin synthesis secondary to inadequate protein.15 Hepatic steatosis (fatty liver) may result from a number of malnutrition-related deficits. When reported, hepatic enzyme levels were elevated in nearly all cases of CF dermatitis, and fatty liver was diagnosed in 5 of 6 liver biopsy or autopsy findings.5,7,9,12 One infant had a particularly complicated course that included significant hepatitis and signs of hepatic failure.19

Although it is evident that deficiencies in zinc, EFAs, and protein are associated with the development of dermatitis in infants with CF and in other children and adults as discussed in the preceding paragraphs, the exact pathophysiological mechanism that generates the dermatitis remains to be elucidated. Many studies have investigated the role of prostaglandins in CF, hypothesizing that altered levels of these substances may be the common underlying cause of the skin findings and other clinical manifestations of the disease.33 The metabolism of EFAs such as linoleic acid has been shown to generate bioactive products that may be important in protecting the epidermis from hyperproliferative and inflammatory processes.34 Deficiencies in these EFAs and their metabolites may alter epidermal homeostasis, resulting in the production of proinflammatory leukotrienes, decreased prostaglandin levels, and reduced activity of specific monohydroxy fatty acids that may function to inhibit epidermal hyperproliferation.34 By serving as a cofactor for the Δ6-desaturase enzyme involved in EFA metabolism,35 zinc may also affect this pathway and influence the production of eicosanoids and other biologically active metabolites. Numerous studies have supported the importance of zinc in the metabolism of EFAs, as reviewed by Cunnane et al.35,36 Finally, decreased protein (more specifically, deficiencies in certain amino acids) may alter EFA metabolism.37 PEM is frequently associated with EFA deficiency, and it has been suggested that many of the clinical symptoms—including skin changes—seen in these malnourished individuals may be at least partially attributed to EFA deficiency.38

In conclusion, the dermatitis seen in infants with CF is likely multifactorial, with deficiencies in zinc, EFAs, and protein contributing to its development. However, the relative contribution of each nutrient and the degree to which each influences and interacts with the others in producing the dermatitis are unclear. They all may affect a common underlying pathway such as EFA metabolism, altering the production of eicosanoids and other metabolites. In addition, free radical–mediated damage secondary to suboptimal nutritional status and other oxidative stresses may contribute to the dermatitis and other symptoms seen in these infants.9,32 The same metabolic pathways may be affected in other conditions, such as acrodermatitis enteropathica and kwashiorkor, that often have similar dermatologic findings and overlapping nutritional deficiencies. Given the frequent delay in diagnosis, as well as the increased morbidity and mortality associated with PEM in these patients, it is important to consider CF as a possible diagnosis in any infant presenting with dermatitis and other signs of malnutrition. The differential diagnosis should also include biotin deficiency, immunodeficiency syndromes, and aminoaciduria disorders.

ARTICLE INFORMATION

Correspondence: Kurt S. Wenk, MD, Department of Dermatology, Medical Faculty Associates, George Washington University, 2150 Pennsylvania Ave NW, Washington, DC 20037 (kswenk@gmail.com).

Accepted for Publication: September 1, 2009.

Author Contributions: Dr Wenk had full access to all the data in the study and takes responsibility for the integrity and accuracy of the case study. Study concept and design: Wenk and Higgins. Acquisition of data: Wenk, Higgins, and Greer. Analysis and interpretation of data: Wenk. Drafting of the manuscript: Wenk. Critical revision of the manuscript: Wenk, Higgins, and Greer. Administrative, technical, and material support: Wenk. Study supervision: Wenk, Higgins, and Greer.

Financial Disclosure: None reported.

REFERENCES

Riordan  JRRommens  JMKerem  B  et al.  Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA [published correction appears in Science. 1989;245(4925):1437]. Science 1989;245 (4922) 1066- 1073
PubMed Link to Article
Darmstadt  GLMcGuire  JZiboh  VA Malnutrition-associated rash of cystic fibrosis. Pediatr Dermatol 2000;17 (5) 337- 347
PubMed Link to Article
Nielsen  OHLarsen  BF The incidence of anemia, hypoproteinemia, and edema in infants as presenting symptoms of cystic fibrosis: a retrospective survey of the frequency of this symptom complex in 130 patients with cystic fibrosis. J Pediatr Gastroenterol Nutr 1982;1 (3) 355- 359
PubMed Link to Article
Abman  SHAccurso  FJBowman  CM Persistent morbidity and mortality of protein calorie malnutrition in young infants with CF. J Pediatr Gastroenterol Nutr 1986;5 (3) 393- 396
PubMed Link to Article
Darmstadt  GLSchmidt  CPWechsler  DSTunnessen  WWRosenstein  BJ Dermatitis as a presenting sign of cystic fibrosis. Arch Dermatol 1992;128 (10) 1358- 1364
PubMed Link to Article
Hansen  RCLemen  RRevsin  B Cystic fibrosis manifesting with acrodermatitis enteropathica–like eruption: association with essential fatty acid and zinc deficiencies. Arch Dermatol 1983;119 (1) 51- 55
PubMed Link to Article
Rosenblum  JLSchweitzer  JKissane  JMCooper  TW Failure to thrive presenting with an unusual skin rash. J Pediatr 1985;107 (1) 149- 153
PubMed Link to Article
Schmidt  CPTunnessen  W Cystic fibrosis presenting with periorificial dermatitis. J Am Acad Dermatol 1991;25 (5, pt 2) 896- 897
PubMed Link to Article
Phillips  RJCrock  CMDillon  MJClayton  PTCurran  AHarper  JI Cystic fibrosis presenting as kwashiorkor with florid skin rash. Arch Dis Child 1993;69 (4) 446- 448
PubMed Link to Article
Ghali  FESteinberg  JBTunnessen  WW  Jr Picture of the month: acrodermatitis enteropathica–like rash in cystic fibrosis. Arch Pediatr Adolesc Med 1996;150 (1) 99- 100
PubMed Link to Article
Mazzocchi  CMichel  JLChalencon  VTeyssier  GRayet  ICambazard  F Zinc deficiency in mucoviscidosis [in French]. Arch Pediatr 2000;7 (10) 1081- 1084
PubMed Link to Article
Farber  SVawter  GF The Children's Hospital Medical Center Boston, Mass. J Pediatr 1961;59 (3) 451- 459
Link to Article
Crone  JHuber  WDEichler  IGranditsch  G Acrodermatitis enteropathica–like eruption as the presenting sign of cystic fibrosis: case report and review of the literature. Eur J Pediatr 2002;161 (9) 475- 478
PubMed Link to Article
Patrizi  ABianchi  FNeri  ISpecchia  F Acrodermatitis enteropathica–like eruption: a sign of malabsorption in cystic fibrosis. Pediatr Dermatol 2003;20 (2) 187- 188
PubMed Link to Article
Muñiz  AEBartle  SFoster  R Edema, anemia, hypoproteinemia, and acrodermatitis enteropathica: an uncommon initial presentation of cystic fibrosis. Pediatr Emerg Care 2004;20 (2) 112- 114
PubMed Link to Article
Mei-Zahav  MSolomon  MKawamura  ACoates  ADurie  P Cystic fibrosis presenting as kwashiorkor in a Sri Lankan infant. Arch Dis Child 2003;88 (8) 724- 725
PubMed Link to Article
Zedek  DMorrell  DSGraham  MGoodman  DGroben  P Acrodermatitis enteropathica–like eruption and failure to thrive as presenting signs of cystic fibrosis. J Am Acad Dermatol 2008;58 (2) ((suppl)) S5- S8
PubMed Link to Article
O’Regan  GMCanny  GIrvine  AD “Peeling paint” dermatitis as a presenting sign of cystic fibrosis. J Cyst Fibros 2006;5 (4) 257- 259
PubMed Link to Article
Lovett  AKokta  VMaari  C Diffuse dermatitis: an unexpected initial presentation of cystic fibrosis. J Am Acad Dermatol 2008;58 (2) ((suppl)) S1- S4
PubMed Link to Article
Wang  KZhou  BKuo  YMZemansky  JGitschier  J A novel member of a zinc transporter family is defective in acrodermatitis enteropathica. Am J Hum Genet 2002;71 (1) 66- 73
PubMed Link to Article
Krebs  NFSontag  MAccurso  FJHambidge  KM Low plasma zinc concentrations in young infants with cystic fibrosis. J Pediatr 1998;133 (6) 761- 764
PubMed Link to Article
Krebs  NFWestcott  JEArnold  TD  et al.  Abnormalities in zinc homeostasis in young infants with cystic fibrosis. Pediatr Res 2000;48 (2) 256- 261
PubMed Link to Article
Easley  DKrebs  NJefferson  M  et al.  Effect of pancreatic enzymes on zinc absorption in cystic fibrosis. J Pediatr Gastroenterol Nutr 1998;26 (2) 136- 139
PubMed Link to Article
Farrell  PMMischler  EHEngle  MJBrown  DJLau  SM Fatty acid abnormalities in cystic fibrosis. Pediatr Res 1985;19 (1) 104- 109
PubMed Link to Article
Freedman  SDBlanco  PGZaman  MM  et al.  Association of cystic fibrosis with abnormalities in fatty acid metabolism. N Engl J Med 2004;350 (6) 560- 569
PubMed Link to Article
Hansen  AEWiese  HFBoelsche  ANHaggard  MEAdam  DJDDavis  H Role of linoleic acid in infant nutrition: clinical and chemical study of 428 infants fed on milk mixtures varying in kind and amount of fat. Pediatrics 1963;31 (1) 171- 192
Lepage  GLevy  ERonco  NSmith  LGaléano  NRoy  CC Direct transesterification of plasma fatty acids for the diagnosis of essential fatty acid deficiency in cystic fibrosis. J Lipid Res 1989;30 (10) 1483- 1490
PubMed
Mischler  EHParrell  SWFarrell  PMRaynor  WJLemen  RJ Correction of linoleic acid deficiency in cystic fibrosis. Pediatr Res 1986;20 (1) 36- 41
PubMed Link to Article
Oumeish  OYOumeish  I Nutritional skin problems in children. Clin Dermatol 2003;21 (4) 260- 263
PubMed Link to Article
Liu  THoward  RMMancini  AJ  et al.  Kwashiorkor in the United States: fad diets, perceived and true milk allergy, and nutritional ignorance. Arch Dermatol 2001;137 (5) 630- 636
PubMed
Golden  MHNGolden  BEJackson  AA Skin breakdown in kwashiorkor responds to zinc. Lancet 1980;1 (8180) 1256
PubMed Link to Article
Golden  MHN The development of concepts of malnutrition. J Nutr 2002;132 (7) 2117S- 2122S
PubMed
Lemen  RJGates  AJMathe  AAWaring  WWHyman  ALKadowitz  PD Relationships among digital clubbing, disease severity and serum prostaglandins F2a and E concentrations in cystic fibrosis patients. Am Rev Respir Dis 1978;117 (4) 639- 646
PubMed
Ziboh  VACho  YMani  IXi  S Biological significance of essential fatty acids/prostanoids/lipoxygenase-derived monohydroxy fatty acids in the skin. Arch Pharm Res 2002;25 (6) 747- 758
PubMed Link to Article
Cunnane  SCHuang  YSHorrobin  DFDavignon  J Role of zinc in linoleic acid desaturation and prostaglandin synthesis. Prog Lipid Res 1981;20157- 160
PubMed Link to Article
Cunnane  SC Role of zinc in lipid and fatty acid metabolism and in membranes. Prog Food Nutr Sci 1988;12 (2) 151- 188
PubMed
Narce  MPoisson  JPBelleville  JChanussot  B Time-course effects of protein malnutrition on hepatic fatty acids Δ6 and Δ5 desaturation in the growing rat. Br J Nutr 1988;60 (2) 389- 402
PubMed Link to Article
Smit  ENMuskiet  FAJBoersma  R The possible role of essential fatty acids in the pathophysiology of malnutrition: a review. Prostaglandins Leukot Essent Fatty Acids 2004;71 (4) 241- 250
PubMed Link to Article

Figures

Place holder to copy figure label and caption
Figure.

Coalescing erythematous plaques with overlying desquamation involving the buttocks, genitourinary region, and proximal extremities.

Graphic Jump Location

Tables

References

Riordan  JRRommens  JMKerem  B  et al.  Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA [published correction appears in Science. 1989;245(4925):1437]. Science 1989;245 (4922) 1066- 1073
PubMed Link to Article
Darmstadt  GLMcGuire  JZiboh  VA Malnutrition-associated rash of cystic fibrosis. Pediatr Dermatol 2000;17 (5) 337- 347
PubMed Link to Article
Nielsen  OHLarsen  BF The incidence of anemia, hypoproteinemia, and edema in infants as presenting symptoms of cystic fibrosis: a retrospective survey of the frequency of this symptom complex in 130 patients with cystic fibrosis. J Pediatr Gastroenterol Nutr 1982;1 (3) 355- 359
PubMed Link to Article
Abman  SHAccurso  FJBowman  CM Persistent morbidity and mortality of protein calorie malnutrition in young infants with CF. J Pediatr Gastroenterol Nutr 1986;5 (3) 393- 396
PubMed Link to Article
Darmstadt  GLSchmidt  CPWechsler  DSTunnessen  WWRosenstein  BJ Dermatitis as a presenting sign of cystic fibrosis. Arch Dermatol 1992;128 (10) 1358- 1364
PubMed Link to Article
Hansen  RCLemen  RRevsin  B Cystic fibrosis manifesting with acrodermatitis enteropathica–like eruption: association with essential fatty acid and zinc deficiencies. Arch Dermatol 1983;119 (1) 51- 55
PubMed Link to Article
Rosenblum  JLSchweitzer  JKissane  JMCooper  TW Failure to thrive presenting with an unusual skin rash. J Pediatr 1985;107 (1) 149- 153
PubMed Link to Article
Schmidt  CPTunnessen  W Cystic fibrosis presenting with periorificial dermatitis. J Am Acad Dermatol 1991;25 (5, pt 2) 896- 897
PubMed Link to Article
Phillips  RJCrock  CMDillon  MJClayton  PTCurran  AHarper  JI Cystic fibrosis presenting as kwashiorkor with florid skin rash. Arch Dis Child 1993;69 (4) 446- 448
PubMed Link to Article
Ghali  FESteinberg  JBTunnessen  WW  Jr Picture of the month: acrodermatitis enteropathica–like rash in cystic fibrosis. Arch Pediatr Adolesc Med 1996;150 (1) 99- 100
PubMed Link to Article
Mazzocchi  CMichel  JLChalencon  VTeyssier  GRayet  ICambazard  F Zinc deficiency in mucoviscidosis [in French]. Arch Pediatr 2000;7 (10) 1081- 1084
PubMed Link to Article
Farber  SVawter  GF The Children's Hospital Medical Center Boston, Mass. J Pediatr 1961;59 (3) 451- 459
Link to Article
Crone  JHuber  WDEichler  IGranditsch  G Acrodermatitis enteropathica–like eruption as the presenting sign of cystic fibrosis: case report and review of the literature. Eur J Pediatr 2002;161 (9) 475- 478
PubMed Link to Article
Patrizi  ABianchi  FNeri  ISpecchia  F Acrodermatitis enteropathica–like eruption: a sign of malabsorption in cystic fibrosis. Pediatr Dermatol 2003;20 (2) 187- 188
PubMed Link to Article
Muñiz  AEBartle  SFoster  R Edema, anemia, hypoproteinemia, and acrodermatitis enteropathica: an uncommon initial presentation of cystic fibrosis. Pediatr Emerg Care 2004;20 (2) 112- 114
PubMed Link to Article
Mei-Zahav  MSolomon  MKawamura  ACoates  ADurie  P Cystic fibrosis presenting as kwashiorkor in a Sri Lankan infant. Arch Dis Child 2003;88 (8) 724- 725
PubMed Link to Article
Zedek  DMorrell  DSGraham  MGoodman  DGroben  P Acrodermatitis enteropathica–like eruption and failure to thrive as presenting signs of cystic fibrosis. J Am Acad Dermatol 2008;58 (2) ((suppl)) S5- S8
PubMed Link to Article
O’Regan  GMCanny  GIrvine  AD “Peeling paint” dermatitis as a presenting sign of cystic fibrosis. J Cyst Fibros 2006;5 (4) 257- 259
PubMed Link to Article
Lovett  AKokta  VMaari  C Diffuse dermatitis: an unexpected initial presentation of cystic fibrosis. J Am Acad Dermatol 2008;58 (2) ((suppl)) S1- S4
PubMed Link to Article
Wang  KZhou  BKuo  YMZemansky  JGitschier  J A novel member of a zinc transporter family is defective in acrodermatitis enteropathica. Am J Hum Genet 2002;71 (1) 66- 73
PubMed Link to Article
Krebs  NFSontag  MAccurso  FJHambidge  KM Low plasma zinc concentrations in young infants with cystic fibrosis. J Pediatr 1998;133 (6) 761- 764
PubMed Link to Article
Krebs  NFWestcott  JEArnold  TD  et al.  Abnormalities in zinc homeostasis in young infants with cystic fibrosis. Pediatr Res 2000;48 (2) 256- 261
PubMed Link to Article
Easley  DKrebs  NJefferson  M  et al.  Effect of pancreatic enzymes on zinc absorption in cystic fibrosis. J Pediatr Gastroenterol Nutr 1998;26 (2) 136- 139
PubMed Link to Article
Farrell  PMMischler  EHEngle  MJBrown  DJLau  SM Fatty acid abnormalities in cystic fibrosis. Pediatr Res 1985;19 (1) 104- 109
PubMed Link to Article
Freedman  SDBlanco  PGZaman  MM  et al.  Association of cystic fibrosis with abnormalities in fatty acid metabolism. N Engl J Med 2004;350 (6) 560- 569
PubMed Link to Article
Hansen  AEWiese  HFBoelsche  ANHaggard  MEAdam  DJDDavis  H Role of linoleic acid in infant nutrition: clinical and chemical study of 428 infants fed on milk mixtures varying in kind and amount of fat. Pediatrics 1963;31 (1) 171- 192
Lepage  GLevy  ERonco  NSmith  LGaléano  NRoy  CC Direct transesterification of plasma fatty acids for the diagnosis of essential fatty acid deficiency in cystic fibrosis. J Lipid Res 1989;30 (10) 1483- 1490
PubMed
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