When an 84-year-old man in Hong Kong was admitted to a hospital for a condition related to an enlarged prostate, doctors noticed something else about him—he was oddly gray, according to a case report in the New England Journal of Medicine.
His skin, particularly his face, had an ashen appearance. His fingernails and the whites of his eyes had become silvery. When doctors took a skin biopsy, they could see tiny, dark granules sitting in the fibers of his skin, in his blood vessels, in the membranes of his sweat glands, and in his hair follicles.
A blood test made clear what the problem was: the concentration of silver in his serum was 423 nmol/L, over 40 times the reference level for a normal result, which is less than 10 nmol/L. The man was diagnosed with a rare case of generalized argyria, a buildup of silver in the body’s tissue that causes a blueish-gray discoloration—which is generally permanent.
When someone consumes silver particles, the metal moves from the gut into the bloodstream in its ionic form. It’s then deposited throughout the body in various tissues, including the skin, muscles, heart, lungs, liver, spleen, and kidneys. There’s some evidence that it accumulates in at least parts of the brain as well.
Discoloration becomes apparent in tissues exposed to sunlight—hence the patient’s notably gray face. Silver ions in the skin undergo photoreduction from ultraviolet light exposure, forming atomic silver that can be oxidized to compounds such as silver sulfide and silver selenide, creating a bluish-gray tinge. Silver can also stimulate the production of the pigment melanin, causing darkening. Once discoloration develops, it’s considered irreversible. Chelation therapy—generally used to remove metals from the body—is ineffective against argyria. That said, some case studies have suggested that laser therapy may help.
If Margaritaville were a real place, it should definitely keep a few dermatologists on hand.
In a case of an oft-overlooked food preparation risk, a 40-year-old man showed up to an allergy clinic in Texas with a severe, burning rash on both his hands that had two days earlier. A couple of days later, it blistered. And a few weeks after that, the skin darkened and scaled. After several months, the skin on his hands finally returned to normal.
The culprit: lime juice and sunlight.
It turns out that just before developing the nasty skin eruption, the man had manually squeezed a dozen limes, then headed to an outdoor soccer game without applying sunscreen. His doctors diagnosed the man’s rash as a classic case of phytophotodermatitis, according to a case report published Wednesday in the New England Journal of Medicine.
The condition is caused by toxic substances found in plants (phyto) that react with UV light (photo) to cause a burning, blistering, scaling, pigmented skin condition (dermatitis).
Specifically, the toxic chemicals are furocoumarins, which are found in some weeds and also a range of plants used in food. Those include celery, carrot, parsley, fennel, parsnip, lime, bitter orange, lemon, grapefruit, and sweet orange. Furocoumarins include chemicals with linear structures, psoralens, and angular structures, called angelicins, though not all of them are toxic.
Hair is resistant to digestion and isn’t easily moved through the digestive system. As such, it often gets lodged in folds of the gastric lining, denatures, and then traps food and gunk to form a mass. Over time, it will continue to collect material, growing into a thick, matted wad.
Of all the bezoars, trichobezoars are the most common. But none of them are particularly easy to spot. On CT scans, bezoars can be indistinguishable from food in the stomach unless there’s an oral contrast material. To look for a possible bezoar in the teen, her doctors ordered an esophagogastroduodenoscopy, in which a scope is put down into the stomach through the mouth. With that, they got a clear shot of the problem: a trichobezoar. (The image is here, but a warning: it’s graphic).
Tangled tail
But this trichobezoar was particularly rare; hair from the mottled mat had dangled down from the stomach and into the small bowel, which is an extremely uncommon condition called Rapunzel syndrome, named after the fairy-tale character who lets down her long hair. It carries a host of complications beyond acute abdominal pain, including perforation of the stomach and intestines, and acute pancreatitis. The only resolution is surgical removal. In the teen’s case, the trichobezoar came out during surgery using a gastrostomy tube. Surgeons recovered a hairball about 2.5 inches wide, along with the dangling hair that reached into the small intestine.
For any patient with a trichobezoar, the most important next step is to address any psychiatric disorders that might underlie hair-eating behavior. Hair eating is often linked to a condition called trichotillomania, a repetitive behavior disorder marked by hair pulling. Sometimes, the disorder can be diagnosed by signs of hair loss—bald patches, irritated scalp areas, or hair at different growth stages. But, for the most part, it’s an extremely difficult condition to diagnose as patients have substantial shame and embarrassment about the condition and will often go to great lengths to hide it.
Another possibility is that the teen had pica, a disorder marked by persistent eating of nonfood, nonnutritive substances. Intriguingly, the teen noted that she had pica as a toddler. But doctors were skeptical that pica could explain her condition given that hair was the only nonfood material in the bezoar.
The teen’s doctors would have liked to get to the bottom of her condition and referred her to a psychiatrist after she successfully recovered from surgery. But unfortunately, she did not return for follow-up care and told her doctors she would instead see a hypnotherapist that her friends recommended.
The human body is full of marvels, some even bordering on miraculous. That includes the limited ability for nerves to regenerate after injuries, allowing people to regain some function and feeling. But that wonder can turn, well, unnerving when those regenerated wires end up in a jumble.
Such is the case for a rare neurological condition called gustatory hyperhidrosis, also known as Frey’s syndrome. In this disorder, nerves regenerate after damage to either of the large saliva glands that sit on either side of the face, just in front of the ears, called the parotid glands. But that nerve regrowth goes awry due to a quirk of anatomy that allows the nerves that control saliva production for eating to get tangled with those that control sweating for temperature control.
In this week’s issue of the New England Journal of Medicine, doctors in Taiwan report an unusual presentation of the disorder in a 76-year-old woman. She told doctors that, for two years, every time she ate, her face would begin profusely sweating. In the clinic, the doctors observed the phenomenon themselves. They watched as she took a bite of pork jerky and began chewing.
Enlarge/ Panel A, 10 seconds after beginning chewing; Panel B, 30 seconds after; Panel C, 50 seconds after; and Panel D, 75 seconds after.
At the start, her face was dry and had a normal tone. But, within 30 seconds, her left cheek began to glisten with sweat and turn red from flushing. By 50 seconds, large beads of sweat coated her cheek. At 75 seconds, droplets ran down her cheek and onto her neck.
Anatomy quirk
Seven years before that doctor’s appointment, the woman had undergone surgery to remove the parotid gland on that side of her face due to the growth of a benign tumor. Gustatory hyperhidrosis is a common complication after such a removal, called a parotidectomy. Some published studies estimate that up to 96 percent of parotidectomy patients will go on to develop the disorder. But, if it does develop, it usually does so within about six to 18 months after the surgery—the time it can take for nerves to regrow. But, in the woman’s case, it appeared to develop after five years since she reported that it started only two years prior to her appointment. It’s unclear why there was such a delay.
Doctors hypothesize that gustatory hyperhidrosis develops after salivary gland injuries or surgeries because of the way nerve fibers are bundled in that part of the head. The nerves that control the salivary glands are part of the parasympathetic nervous system (PSNS). This division of the nervous system is sometimes described as controlling the relatively calm “rest and digest” bodily functions, which are controlled unconsciously as part of the autonomic nervous system that controls things like heart rate.
The PSNS is in contrast to the other part of the autonomic nervous system, called the sympathetic nervous system (SNS). The SNS controls the unconscious “fight or flight” stress responses, which include sweat glands.
Tangled fibers
While PSNS fibers that control the saliva glands and SNS fibers that control sweat glands are from different divisions, they come together on the side of the face. Specifically, they meet up in a tributary nerve called the auriculotemporal nerve. And, they don’t just feed into the same physical conduit, they also overlap in their chemical regulation. Often SNS and PSNS fibers are activated by different signaling molecules (aka neurotransmitters). But it just so happens that the nerve fibers that control sweat glands are activated by the same neurotransmitter that activates the fibers in the PSNS, including those regulating saliva glands. They’re both regulated by a neurotransmitter called acetylcholine.
When PSNS and SNS nerve fibers are damaged near the parotid salivary gland from injury or surgery, the nerves can regenerate. But, given their physical and chemical overlaps, doctors think that in gustatory hyperhidrosis, PSNS nerve fibers end up growing back abnormally, along the paths of SNS fibers. This ends up connecting the PSNS fibers to sweat glands in the skin. So, upon signals of eating, the crossed nerve fibers lead not to salivation but heat responses, including sweat production and blood vessel dilation, which explains the facial flushing.
Living with it
Thankfully, there are various treatments for people with gustatory hyperhidrosis. They include surgical reconstruction or injections of Botox (botulinum neurotoxin), which can shut down the activity of the sweat glands. Similarly, there are topical anticholinergics, which block and inhibit the activity of acetylcholine, the neurotransmitter that activates the nerve fibers activating the sweat glands. There are also topical antiperspirants that can help.
After the doctors in Taiwan diagnosed their patient with gustatory hyperhidrosis, they discussed these options with her. But she reportedly “opted to live with the symptoms.”
In what may be the biological equivalent to getting struck by lightning, a very unlucky man in the Philadelphia area took a very rare bee sting directly to the eyeball—and things went badly from there.
As one might expect, the 55-year-old went to the emergency department, where doctors tried to extract the injurious insect’s stinger from the man’s right eye. But it soon became apparent that they didn’t get it all.
Two days after the bee attack, the man went to the Wills Eye Hospital with worsening vision and pain in the pierced eye. At that point, the vision in his right eye had deteriorated to only being able to count fingers. The eye was swollen, inflamed, and bloodshot. Blood was visibly pooling at the bottom of his iris. And right at the border between the man’s cornea and the white of his eye, ophthalmologists spotted the problem: a teeny spear-like fragment of the bee’s stinger still stuck in place.
(Images of the eye and stinger fragment are here for those who aren’t squeamish. The white arrow in Panel A shows the location of the stinger fragment while the asterisk marks the pooled blood.)
Get thee to an ophthalmologist
In a report published recently in the New England Journal of Medicine, treating ophthalmology experts Talia Shoshany and Zeba Syed made a critical recommendation: If you happen to be among the ill-fated few who are stung in the eye by a bee, you should make sure to see an eye doctor specifically.
“I am not surprised that the ER missed a small fragment,” Shoshany told Ars over email. “They pulled out the majority of the stinger, but the small fragment was only able to be visualized at a slit lamp,” she said, referring to a microscope with a bright light used in eye exams. In this case, they visualized the stinger at 10X or 16X magnification with the additional help of a fluorescent dye. Moreover, after spotting it, the stinger fragment “needed to be pulled out with ophthalmic-specific micro-forceps.”
After finally getting the entirety of the wee dagger out, Shoshany and Syed prescribed a topical antibacterial and prednisolone eye drops (a steroid for inflammation). At a five-month follow-up, the patient had recovered and the vision in his right eye had improved to 20/25.
For those now in fear of eye stings, Soshany has some comforting words: “Ocular bee stings are very rare.” She noted this was the first one she had seen in her career. Although there are documented cases in the scientific literature, the incidence rate is unknown. The odds of getting struck by lightning, meanwhile, are 1 in 15,300, according to the National Weather Service.
But one troubling aspect of this case is that it’s unclear why the man was stung to begin with. According to Shoshany, the man worked on a property with a beehive, but he didn’t work with the insects himself. “He reports he was just walking by and several bees flew up to him; one stung him in the eye,” she said. It’s unclear what provoked them.
Most of the time, when kids accidentally gulp down a non-edible object, it travels toward the stomach. In the best-case scenarios for these unfortunate events, it’s a small, benign object that safely sees itself out in a day or two. But in the worst-case scenarios, it can go down an entirely different path.
That was the case for a poor teen in California, who somehow swallowed a quarter. The quarter didn’t head down the esophagus and toward the stomach, but veered into the airway, sliding passed the vocal cords like they were a vending-machine coin slot.
Enlarge/ Radiographs of the chest (Panel A, postero- anterior view) and neck (Panel B, lateral view). Removal with optical forceps (Panel C and Video 1), and reinspection of ulceration (Panel D, asterisks)
In a clinical report published recently in the New England Journal of Medicine, doctors who treated the 14-year-old boy reported how they found—and later retrieved—the quarter from its unusual and dangerous resting place. Once it passed the vocal cords and the glottis, the coin got lodged in the subglottis, a small region between the vocal cords and the trachea.
Luckily, when the boy arrived at the emergency department, his main symptoms were hoarseness and difficulty swallowing. He was surprisingly breathing comfortably and without drooling, they noted. But imaging quickly revealed the danger his airway was in when the vertical coin lit up his scans.
“Airway foreign bodies—especially those in the trachea and larynx—necessitate immediate removal to reduce the risk of respiratory compromise,” they wrote in the NEJM report.
The teen was given general anesthetic while doctors used long, optical forceps, guided by a camera, to pluck the coin from its snug spot. After grabbing the coin, they re-inspected the boy’s airway noting ulcerations on each side matching the coin’s ribbed edge.
After the coin’s retrieval, the boy’s symptoms improved and he was discharged home, the doctors reported.
It’s hard to imagine a more common stressor for new parents than the recurring riddle: Why is the baby crying? Did she just rub her eyes—tired? Is he licking his lips—hungry? The list of possible culprits and vague signs, made hazier by brutal sleep deprivation, can sometimes feel endless. But for one family in New England, the list seemed to be swiftly coming to an end as their baby continued to slip away from them.
According to a detailed case report published today in the New England Journal of Medicine, it all started when the parents of an otherwise healthy 8-week-old boy noticed that he started crying more and was more irritable. This was about a week before he would end up in the pediatric intensive care unit (PICU) of the Massachusetts General Hospital.
His grandmother, who primarily cared for him, noticed that he seemed to cry more vigorously when the right side of his abdomen was touched. The family took him to his pediatrician, who could find nothing wrong upon examination. Perhaps it was just gas, the pediatrician concluded—a common conclusion.
Rapid decline
But when the baby got home from the doctor’s office, he had another crying session that lasted hours, which only stopped when he fell asleep. When he woke, he cried for eight hours straight. He became weaker; he had trouble nursing. That night, he was inconsolable. He had frantic arm and leg movements and could not sleep. He could no longer nurse, and his mother expressed milk directly into his mouth. They called the pediatrician back, who directed them to take him to the emergency room
There, he continued to cry, weakly and inconsolably. Doctors ordered a series of tests—and most were normal. His blood tests looked good. He tested negative for common respiratory infections. His urinalysis looked fine, and he passed his kidney function test. X-rays of his chest and abdomen looked normal, ultrasound of his abdomen also found nothing. Doctors noted he had high blood pressure, a fast heart rate, and that he hadn’t pooped in two days. Throughout all of the testing, he didn’t “attain a calm awake state,” the doctors noted. They admitted him to the hospital.
Four hours after he first arrived at the emergency department, he began to show signs of lethargy. Meanwhile, magnetic resonance imaging of his head found nothing. A lumbar puncture showed possible signs of meningitis—high red-cell count and protein levels—and doctors began courses of antibiotics in case that was the cause.
Six hours after his arrival, he began losing the ability to breathe. His oxygen saturation had fallen from an initial 97 percent to an alarming 85 percent. He was put on oxygen and transferred to the PICU. There, doctors noted he was difficult to arise, his head bobbed, his eyelids drooped, and he struggled to take in air. His cry was weak, and he made gurgling and grunting noises. He barely moved his limbs and couldn’t lift them against gravity. His muscles went floppy. Doctors decided to intubate him and start mechanical ventilation.
