SUMMERTIME is not so easy for people living with certain autoimmune diseases. The sun, heat and even air-conditioning can intensify symptoms and cause problems that linger for months, if not years.
For doctors who manage those diseases, primarily lupus, scleroderma and Raynaud’s phenomenon, the challenges of educating their patients about sun avoidance become greater, too.
“These are diseases where an ounce of prevention is worth a pound of cure,” said Dr. M. Kari Connolly, an associate professor of dermatology and medicine at the University of California, San Francisco, whose practice focuses on patients with autoimmune diseases. “A little bit of overexposure to the sun can present a whole lot of problems, and if we can get patients to be compliant with sun avoidance and protection, we can minimize the chances of their getting additional complications of their disease.”
Lupus is a chronic inflammatory disease that causes damage to tissue and prolonged episodes of pain. The Lupus Foundation of America says that 500,000 to 1.5 million Americans have received a diagnosis of the systemic form of the disease, known as systemic lupus erythematosus, which affects the skin, joints, tissue, blood and organs. About 25 percent of these patients will also develop skin lesions. Discoid lupus, which affects only the skin, is found in roughly the same number as the systemic kind; 10 percent of these cases, however, progress to the systemic form of the disease.
Lupus is the most common autoimmune disease in which patients are photosensitive, or reactive to both ultraviolet A and B rays from the sun. The foundation says that 75 percent of patients with systemic lupus and 90 percent of discoid lupus patients will suffer flare-ups of symptoms from even brief exposures to sun or heat.
“Photosensitivity can trigger the whole darn disease, including full systemic flare and joint pain and kidney failure,” Dr. Connolly said. “The younger patients sometimes say, ‘The heck with this, I’m tired of carrying sun block,’ and they’ll stay out there, and it’s not just that they are going to give themselves a bad rash. This is something to take seriously.”
The link between the sun and lupus flare-ups is thought to be a set of inflammatory protein molecules called cytokines, which are activated when ultraviolet light hits the skin. The skin inflammation that results can create a chain reaction of other symptoms.
Julianne Lewis, 35, of Santa Rosa, Calif., said she began to show symptoms that were referred to generically as undifferentiated connective tissue disease 13 years ago. When her second son was born four years ago, Ms. Lewis became so sick with stiff, swollen joints, aching skin and lumps on her feet that she couldn’t get out of bed. Lupus was confirmed, she said, and since then Ms. Lewis has undergone chemotherapy treatment and taken immunosuppressant drugs to prevent kidney failure. She also takes blood pressure medicine, an antimalarial drug and occasional steroids to keep her symptoms in check.
“I have to avoid the sun because I developed the butterfly rash,” Ms. Lewis said, referring to the telltale rash that afflicts 40 percent of lupus patients. “It develops across my nose and cheeks. I get it pretty severely, and mine will go on my chest and arms. I have scars on my arms where the rash blistered so badly my skin was burned.”
Ms. Lewis said she had suffered flulike aches and fatigue from even a short time in the sun. She has given up swimming outside with her younger child, she said. Reading a book on the grass is also out of the question. And while Ms. Lewis says she still plays softball occasionally, she wears a hat, long sleeves and wraps a handkerchief around her face.
“It freaks people out because I look so funny trying to cover up,” she said. “I have been slow to come around to the hat and sunscreen thing. And I’ve probably made myself sicker longer because I don’t want to accept it.”
Doctors say a UVA and UVB sunscreen is just one component of a multiprong approach to limit the extent of her symptoms. Other strategies include sun-protection clothing, applying a sun-protection coating to car windows and staying indoors from 10 a.m. to 2 p.m.
But for patients with scleroderma and its most common symptom, Raynaud’s phenomenon, the solution can sometimes be as painful as the problem.
Scleroderma is a chronic autoimmune disease of the connective tissue that causes the skin to become so thick and leathery from inflammation that hair stops growing and sweat glands strangulate and die. The disease often begins in the fingertips before it works up the arms and throughout the rest of the body. In 90 percent of patients, it is accompanied by Raynaud’s phenomenon, where blood vessels become thickened, too, shutting off the flow to the extremities.
The Scleroderma Foundation estimates that of the 300,000 Americans with the disease, nearly a third have the systemic, or diffused, form. The rest have a form limited to a certain place on the body.
That is how the disease began in Dolores McCausland of Cape May, N.J. Two years ago, Mrs. McCausland, 75, had a biopsy taken of a sore on her arm. Doctors confirmed scleroderma as well as pulmonary hypertension, a common result of the disease’s affect on the kidneys, she said. She takes a combination of medicines to treat her blood pressure as well as an antihistamine and gabapentin to treat the pain and itching. She is also treated with oral chemotherapy and an immunosuppressant.
Mrs. McCausland said that covering her arms while driving to avoid exposure to sunlight or using her backyard pool was nearly impossible because the sores on her arms make them sensitive to touching.
“If you saw my arms, you would say, ‘Oh, my God, there’s something wrong with her,’ ” Mrs. McCausland said. “You know when you put a blood pressure cuff on your arm? My arms look like they have been squeezed, and it’s forcing the muscle out to my elbow and shoulder. It’s very painful and itchy.”
Covering the entire body, even in the heat, is critical for scleroderma patients because they have greater susceptibility to skin cancer. And since the blood vessels can also become sclerotic, or thickened, full coverage helps protect people from the shock of entering an air-conditioned environment.
“The problem is that in the summer these patients go from dramatic warm to cold, and they can become very symptomatic from that,” said Dr. Chris T. Derk, an assistant professor of medicine in the rheumatology division at Thomas Jefferson University in Philadelphia. “It’s exceedingly rare, but we have people with Raynaud’s of the heart vessels, and they can go into spasms and give you a small heart attack. You have to cover the whole body because if they can’t rewarm the hand, they can break it.”
Mrs. McCausland said she coped by keeping her house temperature warm and wearing Isotoner gloves to the grocery store, even during the summer months.
“People think I’m some kind of nut,” she said. “I have never been sick a day in my life, and I had to come up with this. ”