BACKGROUND/AIMS

Clinical trials investigating skin findings of cystic fibrosis are limited. The aim of the present study was to evaluate the dermatological findings of patients with cystic fibrosis.

MATERIAL and METHODS

Twenty-six patients diagnosed with cystic fibrosis by the Department of Pediatric Chest Diseases were included in the study. In the pediatric chest diseases outpatient clinic, full blood, vitamin D, vitamin E, albumin, and IgE tests were recorded. In addition to the routine dermatological examination required from the pediatric chest diseases polyclinic, a diagnosis of aquagenic wrinkling was made. The cases were contacted with water at at 39°-40° C in 27°C room temperature conditions, and the water-related wrinkle was assessed after 3 and 5 min of contact.

RESULTS

There were 12 male and 14 female cases. The average age of the patients was 78.80 (8-192) months, and the mean disease duration was 52.53 (2-180) months. In the dermatological examination of the cases, xerosis cutis (18 cases, 69.2%) was most frequently found. Aquagenic palmar wrinkles were observed in 20 (76.9%) cases, and palmar wrinkles were >30% at 5 min. In 5 (25%) cases, delta F508 mutation was present. In 7 (26.9%) cases, pili annulati was observed by light microcopy.

CONCLUSION

The most common skin finding in cystic fibrosis cases is xerosis. The percentage of aquagenic wrinkling is 76.9%. To our knowledge, the association of cystic fibrosis with pili annulation is a previously unreported finding and was reported for the first time in our study.

Keywords: Cystic fibrosis, dermatologic findings, pili annulati, aquagenic palmar wrinkle