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Rare disease leaves burn-like injuries

Published: Tuesday, Sept. 20 2011 11:55 p.m. MDT

Tina Puckett sits with Dr. John Zone during an examination of her father, Lester Crow, at University Hospital.

Laura Seitz, Deseret News

PERRY, (Box Elder County) — Lester Crow has the look of a man who survived a fire as he sits in an easy chair in his daughter's living room. His left leg, swathed in gauze and what looks like netting, is elevated. The skin on his cheeks and eyelids is slightly too pink, like newborn shiny skin. And his arms and the part of his chest you can see above the V-neck of his loose-fitting cotton shirt are mottled with swirls of purplish-pink and white.

Crow, 58, spent many weeks in a burn unit this summer in Arkansas before relocating to Utah so that his grown daughter Tina Crow Puckett and her son Stone, 14, can care for him.

But no fire seared the man. Rather, he is the victim of an attack by his own immune system, due to an extremely rare condition called pemphigus vulgarus, which often starts as blisters in the mouth but can, as it has in Crow's case, cover much of the body.

The skin's job is to keep infections out and fluids in. The breech created by PV's blisters and skin breaks lets fluids seep out and can lead to life-threatening infections, says Dr. John Zone, chairman of dermatology at the University of Utah School of Medicine, who treats Crow and others who have the potentially disfiguring and even deadly disease.

Not too long ago, he says, 90 percent of those with pemphigus vulgaris died in the first year. But modern medicines and techniques now spare the majority.

First, though, it wreaks havoc on families.

The condition makes Crow nearly literally 1 in a million, says Molly Stuart, chief executive officer of the International Pemphigus Pemphigoid Foundation, based in Sacramento, Calif. It's a disease believed to affect just a few hundred at any given time in the United States, a tiny number compared to more common, better-known autoimmune diseases like diabetes. Over the course of a decade or so, a few thousand have some form of pemphigus, but the number currently suffering is small. It is a true orphan in terms of its appeal to drug manufacturers as something to study and for which to work on a cure.

What is not uncommon, says Stuart, is the disruption to the family's life: Many families with a severe, unexpected, acute condition go through variations of what Crow's family has endured, including crippling expense, a sometimes very long search for diagnosis and effective treatment, time off work not only for the patient, but for those who love and physically care for him. Even the stares from strangers who wonder what happened or are fearful it's contagious cross the boundaries of a single diagnosis, a sort of universal experience that's painful and sometimes absurd, as Puckett describes it.

Lester's story

Tina Puckett was living in Perry with her son, Stone, when she learned her father might literally be dying in that burn unit in Arkansas. He'd had heart surgery in early spring and he told her that he'd had a couple of small ulcers in his mouth, then a little blister had formed on his leg near the site where they'd taken a vein graft to do bypass surgery. From there, the sores spread until he had blisters on his ears and cheeks and eyelids, his arms, his legs, his chest and back. Suddenly, he was fighting to survive, the man himself an open wound.

She scrambled to get there, where she hovered for weeks as his condition yo-yoed. What had seemed like a small complication to a spring surgery had become a summer-consuming — and possibly dad-destroying — nightmare.

When he seemed somewhat stable, she arranged to bring him to Utah so she could take care of him. How long, was the question. Her son needed her, too. She had been using time off from her job through the Family Medical Leave Act, but it wouldn't last forever. She also had to find medical care for him here. She found Zone. When she brought Crow home, Zone had him hospitalized for a few days while they stabilized his wounds.

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