When a 58-year-old man asked his dermatologist about "strange grooves" on his scalp, he immediately recognized them as cutis verticis gyrate (CVG)—a rare, progressive, and usually benign condition. But when his doctor dug deeper into the cause of the condition, he uncovered a more serious disorder, Lisa Sanders writes for New York Times Magazine.

Expedite patient diagnosis

'It didn't hurt; it was just weird'

During an annual skin check at the University of Utah, the patient informed his physician, dermatologist Jason Mathis, that the skin at the top of his head in the back seemingly sunk into the bone in a few spots. And while the condition wasn't painful, the patient and his wife found it odd.

When Mathis examined the man's scalp, he noticed "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," Sanders writes. Specifically, Mathis could see the man had folds of what seemed to be excess scalp bulging upward, like there was too much skin in a confined area. According to Mathis, these lumps were hard and could not be smoothed out. Plus, the skin around them seemed to be thicker than it was on the rest of his scalp.

Immediately, Mathis recognized the condition as CVG, Sanders writes. He added that the condition, which generally presents first during puberty or early adulthood, is rare, and progressive, but generally benign.

"But," the man's wife interrupted, "he didn't have it when we got married in 1982. It started maybe 15 or 20 years ago."

Finding the root cause of the condition

Mathis agreed the condition was unusual in how it presented, and so, after he diagnosed the man with CVG, Mathis visited a website called VisualDX—a diagnostic resource that provides thousands of images and cases contributed by doctors from around the world, Sanders writes.

The report on CVG described three forms of the disorder, two of which Mathis was familiar with. But Mathis discovered a third form, known as secondary CVG, that can come on at any age and is associated with a variety of disorders, including eczema, folliculitis, thyroid diseases, and cancer, Sanders writes. And in certain cases, the condition could also be caused by a hormone abnormality called acromegaly, a disorder that "is caused by an abnormal growth on the pituitary gland of the brain, a tumor that makes excess growth hormone," Sanders writes.

As a result of acromegaly, the body produces an excess of growth hormone which enlarges the soft tissues of the face, including the ears, the nose, and the tongue, along with the soft tissues of the hands and feet. If left untreated, Sanders writes, this kind of tumor results in premature death.

As Mathis reviewed the list, he couldn't directly tie any of the conditions to the patient's CVG, but he did recall that his patient was a large man, standing over six feet tall and weighing over 250 pounds with prominent ears and a large nose. Even the man's tongue was large, Sanders writes.

After further questioning the patient confirmed that his hands, feet, and nose had grown larger in recent years. Mathis ordered a blood test that indicated the patient had unusually high levels of a growth factor often stimulated by this type of tumor—and a subsequent CT scan confirmed a growth roughly the size of a lima bean on his pituitary.

Mathis referred the man to a neurosurgeon, who was able to remove the tumor.

'A small price to pay'

Roughly two years after the surgery, the patient reported that his fingers and nose have gotten smaller, although the ridges on his scalp remain. The patient also said he has started to experience hair loss, potentially because his hair needed the excess growth hormone to stay, Sanders writes. But the man's wife told Sanders, "she considers that a small price to pay." (Sanders, New York Times Magazine, 12/29/2021)