THE BLUE GIRLThe 17-year-old girl was eating lunch in her high-school cafeteria in the Netherlands when one of her friends gave her a funny look. ‘‘Your face is turning blue,’’ the friend told her. The others at the table stared. They saw it, too. The young woman hurried to the bathroom, where she saw a faint bluish tinge across her cheeks.
A TEMPORARY CURE
The patient’s neck.
The nurse at school told the young woman to go to the hospital. Instead she went home, where she examined her skin carefully. She could see the pale blue tint on her neck and chest, on her arm and the back of her hand. Despite the strange discoloration, she felt fine.
It was the first time she had worn a new pair of jeans. Could this be dye from her pants? She got into the shower. When she examined herself afterward, the blue coloring was gone.
Two weeks later, when the young woman was in gym class, one of the teachers called her over. Her face had a strange bluish tint — did she feel all right? Now she was worried. The girl hadn’t seen anything when she changed into her gym clothes, and yet the pale discoloration on her chest and arms was obvious. The teacher told her that she needed to go to the hospital. Her mother was at work, so a teacher took her to BovenIJ Hospital, the medical center closest to the girl’s school in northern Amsterdam.
UNSETTLING POSSIBILITIES In the emergency room, she lay on a stretcher surrounded by nurses and doctors checking her oxygen, her heart rate and her blood pressure. Tube after tube of blood was drawn.Test after test performed. She had an EKG, a chest X-ray, an ultrasound and a CT scan. The possibilities the doctors mentioned were terrifying.
CYANOSIS The most common and most dangerous cause of blue skin is cyanosis — the lack of oxygen-rich blood. (The word itself means blue tint, from the Greek root, cyan, a blue-green color.) This disorder can be caused by blood vessels that constrict so tightly — usually because of exposure to the cold — that the deoxygenated blood moves through the capillaries slowly, turning the skin a bluish color. People who stay in a cold swimming pool too long can develop a blue tinge on and around their lips.
Cyanosis can also occur when the body is not able to put enough oxygen into the circulating blood. This kind of central cyanosis is sometimes seen in smokers with severely damaged lungs who may at times have a blue tinge to their fingers or toes, particularly around the nail bed. The girl’s blue color would seem to suggest that she was cyanotic.
POSSIBLE DIAGNOSES 1. Heart defect: Some children are born with a hole in the wall that separates the blood on the right side of the heart, which is on its way to the lungs to get oxygen, from the blood on the left side, which has already been oxygenated and is about to be pumped into the body. The hole allows the deoxygenated blood, which should go to the lungs, to sneak back into circulation.
2. Lung defects: Some children are born with clusters of abnormal vessels that can tear and bleed easily. When these abnormal vessels occur in the lungs, blood can’t get the needed oxygen, and these children may turn blue and feel short of breath, especially with exertion.
3. Blood defects: Red blood cells carry oxygen from the lungs to all the tissues in the body. Leukemia causes the overproduction of white blood cells, often at the expense of these red blood cells. Could she have cancer?
SEEMINGLY NORMALThe tests the doctors did that day were normal. So were the tests done at the next visit and the next, and the next.The discoloration came and went over many weeks. No one understood how she could be cyanotic when all tests indicated she was healthy. One specialist finally decided that she needed to see someone outside of their small hospital and sent her to the internist at the Academic Medical Center on the other side of Amsterdam.
ANOTHER SET OF EYES Dr. Jacobien Hoogerwerf was in her fourth and final year of her internal-medicine residency. She read the patient’s file and then met her. The girl was robust — tall, with a normal weight and build — but right away Hoogerwerf could discern the pale blue tint of the skin on her neck and across her cheeks.The patient had a few other vague symptoms — she felt tired sometimes; out of breath sometimes; and occasionally she noticed a tingling sensation in her fingers and toes. The strange thing was that none of those symptoms were present when she was blue.
Otherwise, her history was unremarkable. She took birth-control pills and vitamin B12 for a deficiency her family doctor had detected. She had no other medical problems. She smoked half a pack a day of cigarettes and drank sometimes at parties.The girl’s physical examination was completely normal — except for her strange blue skin.
A FULLY EXPOSED HINTHoogerwerf noticed that the area beneath the young woman’s bra was not blue, though the skin around it was.The doctor took an alcohol wipe and scrubbed at a patch of blue on the girl’s neck. The neck remained a light blue, but the white square of cotton was now streaked with blue as well. That changed everything. Whatever she had, it was on the surface of her skin and not within the tissue. Hoogerwerf thought that the patient’s other symptoms — the shortness of breath, the fatigue, the tingling — were probably caused by anxiety over the possibility that she might have a terrible disease.
What could cause this young woman’s skin to change color? It wasn’t dye from her clothes — the patient explained that it happened even when she wasn’t wearing blue. The doctor arranged for the patient to see a dermatologist the next day. In the meantime she would try to figure out what was turning this girl blue, if it was not cyanosis.
At the end of the day, Hoogerwerf returned to the puzzle of her patient. She sat down at her computer, and instead of using the usual medical search engines, she turned to Google. The doctor entered ‘‘sweat,’’ ‘‘oxidation,’’ ‘‘blue’’ and ‘‘color’’ and hit enter. Two diseases appeared repeatedly: chromhidrosis and pseudochromhidrosis.
Chromhidrosis is a rare condition caused by an abnormal buildup of a pigment within certain sweat glands and characterized by the secretion of brown, black or occasionally orange or red sweat but only on the cheeks, underarms and around the nipples.
Pseudochromhidrosis, on the other hand, can cause discolored skin just about anywhere. In this disorder, sweat comes out of the glands clear but then interacts with something on the skin that produces the blue or sometimes black or red coloring.
The most common cause of this chemical reaction is a usually benign bacterium that lives on the skin called Corynebacterium. Excited, Hoogerwerf called the patient and instructed her to start using an antiseptic soap. If the discoloration was caused by bacteria, the soap should put an end to it.
When Hoogerwerf spoke to the patient a couple of weeks later, she hadn’t had a single episode of ‘‘the blues’’ since she started using the soap.There are symptoms that are so tightly married to a single diagnosis — chest pain means heart attack; blue skin means cyanosis — that when doctors see them they can think of little else. But when the symptom is present and the usual suspects have been ruled out, that tight connection can make it hard for doctors to imagine anything more, yet they must. In the words of the great diagnostician Sherlock Holmes, ‘‘When you have eliminated the impossible, whatever remains, however improbable, must be the truth.’’
A TEMPORARY CURE
The patient’s neck.
The nurse at school told the young woman to go to the hospital. Instead she went home, where she examined her skin carefully. She could see the pale blue tint on her neck and chest, on her arm and the back of her hand. Despite the strange discoloration, she felt fine.
It was the first time she had worn a new pair of jeans. Could this be dye from her pants? She got into the shower. When she examined herself afterward, the blue coloring was gone.
Two weeks later, when the young woman was in gym class, one of the teachers called her over. Her face had a strange bluish tint — did she feel all right? Now she was worried. The girl hadn’t seen anything when she changed into her gym clothes, and yet the pale discoloration on her chest and arms was obvious. The teacher told her that she needed to go to the hospital. Her mother was at work, so a teacher took her to BovenIJ Hospital, the medical center closest to the girl’s school in northern Amsterdam.
UNSETTLING POSSIBILITIES In the emergency room, she lay on a stretcher surrounded by nurses and doctors checking her oxygen, her heart rate and her blood pressure. Tube after tube of blood was drawn.Test after test performed. She had an EKG, a chest X-ray, an ultrasound and a CT scan. The possibilities the doctors mentioned were terrifying.
CYANOSIS The most common and most dangerous cause of blue skin is cyanosis — the lack of oxygen-rich blood. (The word itself means blue tint, from the Greek root, cyan, a blue-green color.) This disorder can be caused by blood vessels that constrict so tightly — usually because of exposure to the cold — that the deoxygenated blood moves through the capillaries slowly, turning the skin a bluish color. People who stay in a cold swimming pool too long can develop a blue tinge on and around their lips.
Cyanosis can also occur when the body is not able to put enough oxygen into the circulating blood. This kind of central cyanosis is sometimes seen in smokers with severely damaged lungs who may at times have a blue tinge to their fingers or toes, particularly around the nail bed. The girl’s blue color would seem to suggest that she was cyanotic.
POSSIBLE DIAGNOSES 1. Heart defect: Some children are born with a hole in the wall that separates the blood on the right side of the heart, which is on its way to the lungs to get oxygen, from the blood on the left side, which has already been oxygenated and is about to be pumped into the body. The hole allows the deoxygenated blood, which should go to the lungs, to sneak back into circulation.
2. Lung defects: Some children are born with clusters of abnormal vessels that can tear and bleed easily. When these abnormal vessels occur in the lungs, blood can’t get the needed oxygen, and these children may turn blue and feel short of breath, especially with exertion.
3. Blood defects: Red blood cells carry oxygen from the lungs to all the tissues in the body. Leukemia causes the overproduction of white blood cells, often at the expense of these red blood cells. Could she have cancer?
SEEMINGLY NORMALThe tests the doctors did that day were normal. So were the tests done at the next visit and the next, and the next.The discoloration came and went over many weeks. No one understood how she could be cyanotic when all tests indicated she was healthy. One specialist finally decided that she needed to see someone outside of their small hospital and sent her to the internist at the Academic Medical Center on the other side of Amsterdam.
ANOTHER SET OF EYES Dr. Jacobien Hoogerwerf was in her fourth and final year of her internal-medicine residency. She read the patient’s file and then met her. The girl was robust — tall, with a normal weight and build — but right away Hoogerwerf could discern the pale blue tint of the skin on her neck and across her cheeks.The patient had a few other vague symptoms — she felt tired sometimes; out of breath sometimes; and occasionally she noticed a tingling sensation in her fingers and toes. The strange thing was that none of those symptoms were present when she was blue.
Otherwise, her history was unremarkable. She took birth-control pills and vitamin B12 for a deficiency her family doctor had detected. She had no other medical problems. She smoked half a pack a day of cigarettes and drank sometimes at parties.The girl’s physical examination was completely normal — except for her strange blue skin.
A FULLY EXPOSED HINTHoogerwerf noticed that the area beneath the young woman’s bra was not blue, though the skin around it was.The doctor took an alcohol wipe and scrubbed at a patch of blue on the girl’s neck. The neck remained a light blue, but the white square of cotton was now streaked with blue as well. That changed everything. Whatever she had, it was on the surface of her skin and not within the tissue. Hoogerwerf thought that the patient’s other symptoms — the shortness of breath, the fatigue, the tingling — were probably caused by anxiety over the possibility that she might have a terrible disease.
What could cause this young woman’s skin to change color? It wasn’t dye from her clothes — the patient explained that it happened even when she wasn’t wearing blue. The doctor arranged for the patient to see a dermatologist the next day. In the meantime she would try to figure out what was turning this girl blue, if it was not cyanosis.
At the end of the day, Hoogerwerf returned to the puzzle of her patient. She sat down at her computer, and instead of using the usual medical search engines, she turned to Google. The doctor entered ‘‘sweat,’’ ‘‘oxidation,’’ ‘‘blue’’ and ‘‘color’’ and hit enter. Two diseases appeared repeatedly: chromhidrosis and pseudochromhidrosis.
Chromhidrosis is a rare condition caused by an abnormal buildup of a pigment within certain sweat glands and characterized by the secretion of brown, black or occasionally orange or red sweat but only on the cheeks, underarms and around the nipples.
Pseudochromhidrosis, on the other hand, can cause discolored skin just about anywhere. In this disorder, sweat comes out of the glands clear but then interacts with something on the skin that produces the blue or sometimes black or red coloring.
The most common cause of this chemical reaction is a usually benign bacterium that lives on the skin called Corynebacterium. Excited, Hoogerwerf called the patient and instructed her to start using an antiseptic soap. If the discoloration was caused by bacteria, the soap should put an end to it.
When Hoogerwerf spoke to the patient a couple of weeks later, she hadn’t had a single episode of ‘‘the blues’’ since she started using the soap.There are symptoms that are so tightly married to a single diagnosis — chest pain means heart attack; blue skin means cyanosis — that when doctors see them they can think of little else. But when the symptom is present and the usual suspects have been ruled out, that tight connection can make it hard for doctors to imagine anything more, yet they must. In the words of the great diagnostician Sherlock Holmes, ‘‘When you have eliminated the impossible, whatever remains, however improbable, must be the truth.’’