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Cystic fibrosis presenting with periorificial dermatitis.
1Department of Dermatology, Johns Hopkins Hospital, Baltimore, Maryland.
Journal of the American Academy of Dermatology
|November 1, 1991
Summary
A baby boy’s worsening skin rash and failure to thrive were linked to cystic fibrosis. Prompt nutritional therapy improved his condition, suggesting malabsorption due to pancreatic dysfunction.
Area of Science:
- Pediatric Dermatology
- Gastroenterology
- Genetics
Background:
- Periorificial dermatitis can present in infants, sometimes associated with underlying metabolic or genetic conditions.
- Failure to thrive (FTT) in infants necessitates a thorough investigation into potential causes, including nutritional deficiencies and malabsorption.
- Cystic fibrosis (CF) is a genetic disorder that can manifest with gastrointestinal complications, including pancreatic exocrine dysfunction.
Observation:
- A 2-week-old infant presented with progressively worsening periorificial dermatitis and FTT.
- Laboratory findings revealed decreased serum zinc, proteins, and amino acids, alongside increased fecal fat.
- Markedly elevated sweat chloride levels were detected in the infant.
Findings:
- The infant's dermatitis showed a prompt positive response to nutritional therapy.
- The clinical presentation and laboratory results were consistent with malabsorption secondary to pancreatic exocrine dysfunction.
- Elevated sweat chloride levels are a hallmark diagnostic indicator for cystic fibrosis.
Implications:
- Periorificial dermatitis in infants, particularly when accompanied by FTT and malabsorption, should prompt consideration of cystic fibrosis.
- Early diagnosis and management of cystic fibrosis-related complications, such as pancreatic exocrine dysfunction, are crucial for improving infant outcomes.
- Nutritional therapy plays a vital role in managing malabsorption syndromes in infants, including those associated with cystic fibrosis.