Diagnosis of a painful toe: tophi or too few tofu?

November 2004

“Doc, my foot hurts so dang-ed much.” He is nearly crying.

The mammoth 16-year-old hobbles into the office with his mother for the umpteenth time this year. I believe he has missed an average of about 65 days of school and visited the office about 35 times annually in the last two years. He weighs almost twice as much as I do (no small feat, or feet), and he towers over me. Yet he always enters the examination room with his mother, a congenial lady, who has been divorced for a decade. He insists that she initially explain his latest malady as well.

Frequently dressed in the latest “grunge” black T-shirt representing some outlandish punk rock band or skull and crossbones, he is a sophomore in high school who struggles daily with schoolwork. Yet he persists. He is always obsequious and respectful in mannerisms, for he says that his “momma would knock him upside the head” otherwise. His ritual: he plops up onto the examination table and further embellishes upon his mother’s simpler history: “Doc, I got this-here problem…”

Usually, it is a vague upper respiratory infection or simple “bellyache.” And usually, of course, simple reassurances are dispensed along with some pleasantries and “how is the rest of your life?” interrogations. He gratefully accepts my “intricate” four-part diagnostic examination and detailed prognosis. (“You’ll outlive me by decades.”)

In addition, I must often adjust one of his multitude of psychiatric medications for mood disorder or attention deficit disorder, which have been prescribed by the psychiatrist du jour at the local mental health center. These medications commonly precipitate adverse reactions or interact negatively with each other. At one point, he was even so snockered from his medications that I was forced to help him off the exam table. I quickly weaned him down from his 600 mg of bupropion and 6 mg of risperidone.

A few times his mother has called the office in desperation, saying, “He is so depressed!” or “He is acting so strangely!” or “Could you just write for a refill” of his X, Y or Z psychoactive medication. I would quickly obtain an appointment for him to see me and occasionally rapidly turn him to his psychiatrist. Interestingly, he denies having ever used illicit drugs or alcohol — “What, Doc, are you crazy? That stuff messes with your mind!” Oh yes, I have checked his urine a few times before.

Now one must realize that this young man is a recipient of our capitated Medicaid program, for which we are paid enough to cover 1.5 office visits annually. Still, he needs some male consolation, advice and, of course, the invariable excuse to miss school for a day or two.

His father abandoned him when he was 5 years of age. This gentle giant is truly a lost soul who needs a surrogate father figure. Like many of my patients, he has been dealt an unfortunate hand of cards that would make any poker player cry.

“What’s up, doc?”

So today is a new day for my young man. He is too quiet, responding minimally to my questions. “Where does it hurt? You cannot walk on it? It hurts to put your shoe on?” I peruse the radiographs of his foot. As expected: flat normal. I reread the emergency department report: afebrile, foot pain, histrionic, tearful, asking for school excuse, laundry list of psychoactive medications, normal foot radiograph, normal complete blood count and erythrocyte sedimentation rate. He is signed out — foot pain; etiology: psychosomatic.

Initially, I see no redness, swelling or ecchymoses of the foot. So I gently palpate his toes one by one. Nothing, until I touch the great toe. Tears erupt with the slightest pressure. No way! I distract him with my usual “clever” maneuvers — “How’s your girlfriend? How’s school?” and so forth. Not much change in the exam. He still yelps. No effusion and only minimal erythema is present on a second thorough review of his first metatarsal phalangeal joint.

So I tell the mother and young man that I need to obtain some blood work. I initiate therapy with naproxen and ask them to check back with me in the morning. No, I do not get off that easily. “What could it be, Doc?” Trapped.

“Well, I believe it is something I have never seen before in 22 years of pediatric practice. Gout — but I don’t know for sure. Other things may cause this problem.” (Lord, I do not know what they are?)

“I have an older uncle with gout. What is gout anyway, Doc?” Retrieving information that is over 25 years old within my own central nervous system, which processes with something akin to a Pentium-1 computer loaded with a miserable two megabytes of RAM, it is not a facile discussion. But I perform my best simple explanation of gout and its causes. He does not imbibe (I asked again), but he does consume a hefty amount of meat and cheeses. Not much tofu or other legumes in his diet. Perhaps ripe for tophi?

The plot thickens

Mother calls me back the next morning, and as anticipated, the serum uric acid is mildly elevated. “Yes, he is still in pain. That naproxen and my leftover Vicodin is not helping him much. Oh, and by the way, how many days of school should I get an excuse for?” Of course. Now while I am evaluating his joint condition, I also note that in his Chemistry 27, his bilirubin is 2.0 mg/mL and his aspartate transaminase is 112 IU/mL. I am getting deeper and deeper into an enigma.

I immediately attempt to contact several rheumatologists without success; most do not accept Medicaid or patients younger than 18 years. Others have a two-month wait. But I have done my research, and many textbooks say that if it waddles like a duck, limps like a duck and quacks like a duck, then it must be gout. In contrast, other textbooks say that a joint aspiration must be performed to confirm the diagnosis. I even contact a few rheumatologists who say that it cannot be gout — he is simply too young. “But he even has a family history of an uncle with gout,” I implore. “Then what else localizes in the first great toe with excruciating pain? The only three things that I know which commonly cause acute excruciating joint pain specifically here (and elsewhere) in teenagers are a septic joint, rheumatic fever and gout. His otherwise normal examination, normal electrocardiogram and blood work rule out the first two diagnoses.” Gout just does not really occur at this age, I was cordially informed.

Within the next 48 hours, I proceed to evaluate his abnormal liver function tests. His ultrasound and computed tomography scan of his abdomen reveal steatosis (fatty liver). Now I really need a rheumatologist. And a dietitian. (For the patient!) His mother says his pain is still persistent and he is miserable. I remember that, along with dietary manipulations, the two primary long-term treatments for gout include allopurinol and probenecid, neither of which would likely help his acute pain. His 24-hour urine uric acid is also significantly elevated at 680 mg/L/24 hour (normal ,300 mg/L/24 hour), but not above the 800 mg/day threshold that would preclude the use of probenecid due to risk for urolithiasis. However, my personal digital assistant ePocrates warns me that both drugs have been rarely associated with severe hepatotoxicity.

Yet for attacks of acute gout, colchicine may be beneficial, and it lacks potential for liver toxicity. In fact, oral colchicine in some textbooks is considered the surrogate litmus test for acute gouty arthritis — if the joint pain subsides rapidly after several doses in the first 48 hours, then the diagnosis of gout is almost assured, perhaps even without joint aspiration.

With no pain relief in sight, no rheumatologist for a month, a need for many hydrocodone tablets for weeks with his polypharmaceutical central nervous system, and a limping “duck,” each resoundingly screamed at me for a brief trial of colchicine. “So, I not-so-boldly went where no pediatrician had apparently gone before,” to paraphrase a Star Trek captain’s log. He received 4 tablets of colchicine over 24 hours.

Only time will tell

Within 24 hours of initiating colchicine therapy, his pain was ameliorated dramatically. Within three days, his pain abated entirely. Serendipitous? I contemplate that we may need to evaluate him for certain hereditary disorders of purine metabolism, such as hypoxanthine guanine phosphoribosyltransferase deficiency.

His follow-up visit to the rheumatologist next month indeed confirmed that “you do not have gout.” When questioned further by the patient, the doctor explained, “We have no reason as to why your great toe was so painful. Perhaps you stubbed it hard unwittingly. You do have an old fracture in your other metatarsal.”

As Jimmy Buffet sings his personal mantra: “Only time will tell. Only time will tell.” But I can assure you also that no immature amphibian would joyously anticipate the benefits of a needling a future painful webbed toe. For now, a diet high in tofu may indeed help both his steatosis and his tophi.

For more information:

• Wortmann RL. Treatment of acute gouty arthritis: one physician’s approach and where this management stands relative to developments in the field. Curr Rheumatol Rep. 2004;6(3):235-239.
• Terkeltaub RA. Clinical practice. Gout. N Engl J Med. 2003;349:1647-1655.

• Stan L. Block, MD, has a pediatric practice in Bardstown, Ky., and is a member of the Infectious Diseases in Children Editorial Advisory Board.