J.Crew's Jenna Lyons Emerges a Swan After Childhood Disorder

Fashionista was born with potentially disfiguring incontinentia pigmenti.

ByABC News
January 22, 2013, 4:46 PM

Jan. 23, 2013— -- Like many growing girls, Jenna Lyons was a self-perceived ugly duckling, but lately -- especially after J.Crew's colorful fashion line took center stage on the Obama girls at the inauguration this week -- she has emerged as a beautiful swan.

J.Crew's 44-year-old creative director said at Glamour's Women of the Year awards in November, "I know what it's like not to feel beautiful. ... I remember that feeling."

Stick-thin, with bold over-sized glasses and fashionably mismatched clothing, she has her own back story. Lyons was born with Bloch-Sulzberger syndrome or incontinentia pigmenti (IP), a genetic disorder that can cause skin scarring, loss of hair and malformed teeth. She has said she wears dentures.

In its most severe form, the genetic disorder can affect the central nervous system, causing seizures, learning disabilities and mental retardation, according to the Incontinentia Pigmenti International Foundation (IPIF), a nonprofit that supports research into the disorder.

Fortunately for Lyons, she has the milder form, which only discolors skin on the trunk. In some women it is so mild, it is never even diagnosed.

But IP can be deadly serious. Because it is an X-linked genetic mutation, it nearly always kills boys in utero. Males do not have a second X chromosome to overcompensate for the mutation, which attacks their immune systems.

In girls, there can be potential vision problems, including crossed eyes, cataracts or blindness.

Emily Batista, an 18-year-old who made a film about IP, "My Skin," at Reel Works Teen Filmmaking while in high school in Brooklyn, N.Y., said the psychological effects are just as traumatizing.

She has dark patches all over her body and missing teeth.

"IP was a struggle for me when I was younger," she said. "I got bullied a lot about my appearance. I never knew if I had a place in the world or if I was just that kid that would get picked on forever, but with a few good friends and as time went on I learned that we all mature and people really don't care about my appearance."

Like Lyons, Batista had dental surgery, but said, "the pain will be worth it in the end when I have the smile I dreamed of having as a kid."

Susanne Emmerich, founder and executive director of the IP foundation, told ABCNews.com that she hoped Lyons would also come out as a spokeswoman to bring awareness to the disorder.

Even if a milder case of IP is not life-threatening, women can be carriers and have a daughter with the devastating health consequences.

"I hear from a lot of young people who say they don't know anyone else with IP and they feel so isolated," said Emmerich, who wrote an email to Lyons asking her to get involved in the nonprofit foundation. "They need someone to identify with -- someone who deals with Michelle Obama, you couldn't get higher than that."

Lyons has addressed overcoming her earlier awkwardness in an interview with the New York Times last week. She said, "I am so not an insider, and that is O.K. I might feel like one in very particular moments. But I don't feel the same pressure I did when I was young to be part of the club. I'm not one of the cool kids, and that is totally fine."

Emmerich, a 75-year-old former fundraiser, said she founded IPIF in 1994 when her nephew's daughter was born with the disorder, but had been misdiagnosed.

"This was before there were genetic tests," she said. "She had little blisters that looked like herpes, which she didn't have. She was treated with medication for six months."

Babies with IP are sometimes born with a detached retina, which is correctable with laser surgery.

"She didn't have that, but if she had, it would have been a disaster," Emmerich said. "There can also be neurological implications."

Her grandniece, Gemma Stern, now 18 and a student at Tufts University, said she was "thrilled" when she learned that she had the disorder in common with fashion icon Lyons.

"It felt great to hear Jenna speak about her struggles growing up with IP because I can completely relate," she said.

Stern was self-conscious about a large bald spot on the top of her head, so when she was 10, she got a hair transplant. She has also undergone extensive dental work to correct her deformed teeth.

"Like Jenna, I have scars and discolored skin and have always been embarrassed when wearing shorts," she said. "Luckily, with age these scars have somewhat faded."

Like others, Stern was teased, but now in college, she said, "people are shocked when I tell them I have IP. ... I have gone to many doctors where I had to explain the disease to them."