Aquagenic Wrinkling Among Skin-Related Signs of Cystic Fibrosis

TOPLINE:

Cutaneous manifestations of cystic fibrosis (CF) include palmar wrinkling, nutrient deficiency dermatitis, vascular disorders, and reactions to CF treatments.

METHODOLOGY:

• Patients with CF, caused by a mutation in the CF Transmembrane Conductance Regulator (CFTR) gene, can develop diverse dermatologic manifestations.
• Researchers reviewed the literature and provided their own clinical experience regarding dermatologic manifestations of CF.
• They also reviewed the cutaneous side effects of CFTR modulators and antibiotics used to treat CF.

TAKEAWAY:

• Aquagenic wrinkling of the palm is common in individuals with CF, affecting up to 80% of patients (and 25% of CF gene carriers), and can be an early manifestation of CF. Treatments include topical medications (such as aluminum chloride, corticosteroids, and salicylic acid), botulinum toxin injections, and recently, CFTR-modulating treatments.
• CF nutrient deficiency dermatitis, often in a diaper distribution, usually appears in infancy and, before newborn screening was available, was sometimes the first sign of CF in some cases. It usually resolves with an adequate diet, pancreatic enzymes, and/or nutritional supplements. Zinc and essential fatty acid deficiencies can lead to acrodermatitis enteropathica–like symptoms and psoriasiform rashes, respectively.
• CF is also associated with vascular disorders, including cutaneous and, rarely, systemic vasculitis. Treatment includes topical and oral steroids and immune-modulating therapies.
• CFTR modulators, now the most common and highly effective treatment for CF, are associated with several skin reactions, which can be managed with treatments that include topical steroids and oral antihistamines. Frequent antibiotic treatment can also trigger skin reactions.

IN PRACTICE:

“Recognition and familiarity with dermatologic clinical manifestations of CF are important for multidisciplinary care” for patients with CF, the authors wrote, adding that “dermatology providers may play a significant role in the diagnosis and management of CF cutaneous comorbidities.”

SOURCE:

Aaron D. Smith, BS, from the University of Virginia (UVA) School of Medicine, Charlottesville, and coauthors were from the departments of dermatology and pulmonology/critical care medicine at UVA. The study was published online in the Journal of the American Academy of Dermatology.

LIMITATIONS:

The authors did not make a comment about the limitations of their review.

DISCLOSURES:

No funding was received for the review. The authors had no disclosures.

A version of this article first appeared on Medscape.com.