What Was Causing the Strange Grooves in the Man’s Scalp?
A dermatologist looks into a rare and generally benign condition and finds a more serious disorder.,
A dermatologist looks into a rare and generally benign condition and finds a more serious disorder.
“Is there anything else?” asked Dr. Jason Mathis. His patient, a 58-year-old man, had come with his wife to see the young dermatologist at the University of Utah for his annual skin check. Mathis had looked over the handful of moles and skin tags and found nothing alarming. What else was bothering the man?
There was one thing, the patient said after a pause. At the top of his head in the back, the skin seemed to sink into the bone in a few spots. It didn’t hurt; it was just weird. He asked another dermatologist about it a few years earlier. That doctor looked at his head, then checked in a textbook and said it was something or another — he couldn’t remember what the dermatologist called it. But he told the patient not to worry about it, and as far as that doctor was concerned, that was the end of it.
Mathis looked at the scalp, still covered by mostly dark hair. It looked normal enough, but a light touch revealed several irregularities. He carefully moved the hair away to reveal four linear grooves distributed around the crown and back of the man’s head. Each was an inch and a half to two inches long. The patient described it as hair growing into the skull, but Mathis could see that it was actually folds of what looked like excess scalp bulging upward into little mounds, as if there were too much skin in too small an area. The lumps were firm and couldn’t be smoothed out. The skin there seemed thicker than on the rest of the scalp.
Mathis recognized it immediately. It was a condition known as cutis verticis gyrata (C.V.G.), from the Latin meaning skin whorled at the scalp. It was first described in the mid-19th century by a French physician who called it cutis sulcata — furrowed skin. It’s rare, and progressive and usually benign, Mathis explained. It’s seen much more frequently in men than women, though it may be underdiagnosed in women because they wear their hair longer.
And that first dermatologist was right, he added. Most of the time it comes on at puberty or early adulthood, and while it’s strange, it’s nothing to worry about. “But,” the man’s wife interjected, “he didn’t have it when we got married in 1982. It started maybe 15 or 20 years ago.”
The man had discovered it one day in the shower. He was washing his hair and felt a strange dent on his scalp, then another, and another. He wasn’t sure how long they’d been there, but he never noticed them before. “Feel my head,” he asked his wife later that morning. She noted the grooves and furrows. “What is that?” she asked. No idea.
He started looking at other men’s heads. He asked his barbers. They’d never seen anything like it, either. When the dermatologist told him not to worry about it, he tried not to. But it was weird, right?
It was weird. Mathis sat down at his computer. He turned to a website he often visited called VisualDX. It was a diagnostic resource, providing thousands of images and cases contributed by doctors from around the world. You could find just about everything here. He often used it to show patients what they had and to reassure them that they weren’t alone. But every now and then he also used it as a reference to learn more about a disease. The brief report on C.V.G. described three forms of the disorder. Primary essential C.V.G. appears with no other symptoms or medical problems. There was also primary nonessential C.V.G.; this was usually associated with intellectual disability, and sometimes psychiatric or ophthalmological problems. Each was seen mostly in men and usually started in adolescence or early adulthood. These were the forms Mathis was familiar with. But this patient had none of the associated comorbidities and had developed the condition later in life.
And then there was secondary C.V.G., when the strange ridges and grooves were associated with a wide range of disorders, from eczema and folliculitis to thyroid diseases and cancer. This version of C.V.G. could come on at any age. The dermatologist reviewed the list. The patient had some folliculitis, but it was not on his scalp, so that wasn’t it. And no eczema. C.V.G. could be seen in diseases of the thyroid — when there was too much of the hormone made or too little. Thyroid hormone helps control how fast the biological engine of the body runs. Too much hormone, and it runs too fast, causing symptoms including a racing heart, insomnia and often weight loss. Too little of the hormone causes the opposite — fatigue, a slow heart, weight gain. Each condition can cause myxedema, the deposition of a mucuslike substance below the surface of the skin that makes the top layers, the epidermis, seem thicker. The man hadn’t complained of any of these thyroid symptoms, and the doctor found nothing besides the scalp abnormalities on examination. Still, he could send off a blood test look for this common problem as the potential source of this weird finding.
Credit…Photo illustration by Ina Jang
One Giant Step
The literature mentioned that another hormone abnormality can cause C.V.G.: acromegaly. This disorder is caused by an abnormal growth on the pituitary gland of the brain, a tumor that makes excess growth hormone. As a medical student, Mathis had been taught to recognize the disorder by looking for features of the most famous patient in his lifetime with acromegaly, Andr? Ren? Roussimoff, best known by the name he adopted first in his wrestling career and later as an actor: Andr? the Giant. And he was a giant: 7-foot-4 and weighing over 500 pounds. His face, well known to so many of us thanks to his role as Fezzik, the kind giant in Rob Reiner’s classic movie “The Princess Bride,” serves as a template for the effects of acromegaly. The excess growth hormone causes enlargement of the soft tissues of the face, including the ears, the nose and the tongue, as well as the soft tissues of the hands and feet. Untreated, this kind of tumor causes premature death.
Mathis looked at the patient — not just his skin but the whole person. He was a big man, over six feet tall and weighing more than 250 pounds. He had prominent ears and a large nose. Even his tongue was large. Acromegaly was certainly possible. He asked the patient if he had noticed any changes in the size of his feet or hands over the past few years. He certainly had, the man answered promptly. A few years ago, he and his wife replaced the wedding rings they bought each other when they married and didn’t have much money, and he needed a bigger ring. And his feet were growing, too. He used to wear a size 12 but now needed size 14. He figured it was because he was getting fatter. He had put on 75 pounds since their wedding day.
The dermatologist explained his thinking. He had never made a diagnosis of acromegaly and quickly looked up how to do it. The first step was to check the level of one of the growth factors usually stimulated by this kind of brain tumor. If that was high, the patient would need imaging of his brain to look for a tiny tumor on the pituitary gland. The blood test took days to come back. It was abnormal. And the CT scan revealed a growth about the size of a lima bean on his pituitary. Mathis referred the patient to a neurosurgeon, who removed the tumor. He didn’t see the patient again until the following year. By then, the patient reported, his fingers and nose had gotten smaller, and his gratitude to the young doctor had grown. Mathis was thrilled with his diagnosis. “I’m curious,” he told me recently, and that has been one of his most reliable and valuable tools as a doctor. And he’s not ashamed to have to look things up.
Two years have passed since the patient had his operation, and he and his wife have watched with interest as the man’s former face emerged. The lumpy scalp is unchanged, but his wife was particularly happy to welcome her husband’s old nose back. He is starting to lose his hair — maybe it needed the excess growth hormone to stay — but, his wife told me, she considers that a small price to pay.
Lisa Sanders, M.D., is a contributing writer for the magazine. Her latest book is “Diagnosis: Solving the Most Baffling Medical Mysteries.” If you have a solved case to share, write her at Lisa.Sandersmd@gmail.com.