Nikki Thomason, founder of the Cheetah Club
How a handful of patients could hold the key to a cure for cancer
Patients with a rare skin disease called lymphomatoid papulosis, also known as "LyP," call themselves "the spotted ones." In layman's terms, LyP is a lymphatic system disorder that manifests itself in self-healing nodules and papules ("bumps" and "spots") that come and go spontaneously.
What's unique about LyP and what has been baffling doctors and patients for years is its extraordinary ability to inexplicably turn itself off, and then turn itself back on again. It looks like cancer and can act like cancer, but frequently and for no reason, it suddenly disappears of its own accord for weeks or months at a time before reactivating. The lesions vary in shape and size. They can be small, round, shiny and smooth, or large, crusty open wounds. Some leave scars that are flat, pinkish red, brown or purple; others leave keloid scars that are flesh-colored, red or pink and may be nodular or ridged; and some, if left alone, don't leave scars at all. The number of lesions can vary from a single flare up to eruptions numbering hundreds. They can come and go for decades.
The disease is so rare that the chances of developing it are practically one in a million -- the overall prevalence rate is estimated at 1.2 to 1.9 cases per 100,000 population -- but 10 to 20 percent of cases can turn out to be precursors for more serious conditions, including mycosis fungoides, Hodgkin's disease, cutaneous T-cell lymphoma and anaplastic large cell lymphoma.
Statistically, there should be only about 5,000 "spotted ones" worldwide. Yet so far, only a few hundred have found each other through The Cheetah Club, started by San Francisco Bay Area resident Nikki Thomason, who, after two years of breakouts, was finally diagnosed in March 1998 at the age of 40. Thomason says the name "Cheetah Club" came to her while watching the Discovery Channel. "The narrator was saying 'cheetah' was the Hindi word for the spotted one," she recalls. "I thought it was the perfect name."
The Cheetah Club runs an AOL chat room, which allows those list members who are on America Online to share experiences and spread awareness of the disease. And awareness is what LyP sufferers desperately need in the search for answers.
In fact, in some scientific circles, LyP still hasn't been fully embraced. The American Cancer Society does not classify LyP as a cancer, but it has been acknowledged as such in Europe. In 1997, the European Organization for Research and Treatment of Cancer (EORTC) formally recognized LyP as a lymphoma.
At this time, LyP is not thought to be hereditary, but there is no known cure. Most doctors begin by treating their patients with a topical steroid cream, such as Diprolene®. If the steroid creams to not prevent the development of new lesions (which is true in most cases), the next line of treatment is Psoralen® combined with exposure to ultraviolet light (PUVA) or low oral doses of methotrexate (MTX). MTX doses need to be kept very low (usually up to 15mg weekly) to avoid liver toxicities.
LyP has only been studied in-depth since 1968, so it's still relatively unknown. One of its champions, however, is Dr. Marshall Kadin -- a Harvard professor who's been the torchbearer for LyP for a decade. As well as juggling two jobs -- associate professor of pathology at Harvard Medical School and associate director of the skin cancer program at Boston's Beth Israel Deaconess Medical Center -- Kadin has also recently returned from a successful sabbatical at the Institute of Dermatology in Zurich, Switzerland, where he was conducting further LyP research. Early results are promising. "LyP is an important clue to the [workings] of lymphoma," he said. "The major breakthroughs in cancer will not come from altering chemotherapy regimens, but instead from understanding basic mechanisms of disease development."
Kadin accepts that the debate over the possible link between non-Hodgkin's and LyP is a controversial one, but he believes it deserves further investigation. "A genetic basis for LyP shouldn't be entirely excluded, either," he said. "No one understands it at the present." Since some LyP patients do die from related lymphomas, Kadin is determined to find the connection, if there is one.
Back on the West Coast, meanwhile, patients such as Thomason and her fellow Cheetah Club members are thrilled with the possibility that their rare disease could one day provide the key to a cure for cancer. "What if they do find a cure for cancer because of it?" says 23-year-old Julie Berruto, as she imagines the headline. "'LyP saves the world' -- wouldn't that be awesome?"