[Patients living with congenital ichthyosis available for interviews]
CHICAGO — For as long as Laura Hogan, 69, can remember, living with the rare skin condition lamellar ichthyosis has meant more than managing painful blisters and scaly skin. Throughout her life, she also has dealt with food allergies, chronic bronchitis, countless eyelid surgeries and overheating in the summer.
A new Northwestern University study suggests her experience with allergies may be more common than previously known among people living with congenital ichthyosis, a group of rare inherited scaling skin disorders that weaken the skin’s protective barrier from birth.
In the new study, published last month in the Journal of Allergy and Clinical Immunology, Northwestern scientists surveyed 115 children and adults with congenital ichthyosis and found significantly higher rates of food allergies, asthma and hay fever than in the general U.S. population. The study authors note that the survey size was large for a disorder estimated to affect one in 300,000 births.
Beyond identifying an overlooked burden for patients with a rare disease, the findings may also advance scientistic understanding of how the skin’s protective barrier plays a role in allergic disease.
“Clinicians think about ichthyosis as being only a skin disease, but it’s not,” said study senior author Dr. Amy Paller , who has spent 40 years studying ichthyosis and is chair of dermatology at Northwestern University Feinberg School of Medicine.
“Having asthma, food allergies and seasonal allergies can all impact quality of life and sleep. We need to approach allergic disorder with treatments that go beyond the moisturizers that are the mainstay of treatment for ichthyosis — and be more holistic in our care of ichthyosis patients,” added Paller, who also is a pediatric dermatologist at Ann & Robert H. Lurie Children’s Hospital of Chicago.
Findings
The survey included respondents with multiple forms of congenital ichthyosis. In it:
Researchers already knew that allergies were common in Netherton syndrome, a rare form of congenital ichthyosis, and in ichthyosis vulgaris, a common noncongenital form that predisposes towards eczema. However, this is the first study to show elevated rates of allergic disorders across all congenital ichthyoses that were studied. Patients with some of the most severe forms of congenital ichthyosis also had the highest rates of allergic disease, a pattern the authors said was consistent with those disorders’ greater skin-barrier damage. The findings build on research in eczema that suggests environmental allergens enter the body more easily through a damaged skin barrier.
‘God made me this way’
Hogan, of the Chicago area, always carries a card with information about ichthyosis. The card dispels what Hogan says is one of the biggest misconceptions about her condition — that it is contagious.
“When they see me, some people will get up and move away,” Hogan said. “I would rather have them ask, so I can give them the card and tell them, ‘You know how God made you this way? God made me this way.’”
Hogan has been a patient of Paller for about 40 years and often participates in her research projects. Hogan hopes those projects, including the latest survey findings, help educate the public and clinicians about ichthyosis. Because it’s a rare condition, “a lot of doctors don’t know it and don’t understand it,” she said.
‘His skin condition doesn’t define him’
The mother of a 13-year-old with epidermolytic ichthyosis, who’s also a patient of Paller, stressed that she didn’t want “his skin condition to define him.”
Jennifer Roach of Ottawa, Illinois, said her son, Mason, is a happy teenager with understanding friends at a small country school. Still, he deals with a lot of pain because “his skin is very fragile,” she said. “Sometimes his whole foot can be covered in blisters. It can be very painful.”
Roach said the new survey findings resonated with her because Mason has also been diagnosed with eosinophilic esophagitis, a rare allergic condition that makes eating challenging. “I always have hope with the ongoing research from Dr. Paller that someday it’s going to lead to a cure for ichthyosis,” she said.
The study is titled, “Congenital ichthyosis is associated with increased risk of allergic disorders.”
Journal of Allergy and Clinical Immunology
Congenital ichthyosis is associated with increased risk of allergic disorders