A twenty-six-year-old woman came to us because for the past ten years she had had the strangest eruption. Her skin would raise up in waves of rope-like lesions, which were intensely itchy and at times burning. Through the years thousands of these waves had passed over her skin, which left it darkly pigmented. Only her face was clear. High-dose cortisone had been the only way to suppress the waves and the itch.

Even more impressive than this patterned eruption were her massively enlarged breasts. As she sat, we saw them extending down to rest on her thighs. They appeared to be about 20 pounds each in size. She told us that they had never stopped growing.

We knew her skin disease was erythema annulare centrifugum, the result of an immune response. We had seen it in patients with internal cancer, and we had seen it as a result of fungal or yeast infections. But why in this patient? And why were her breasts so enormous?

We hospitalized her and later studied her for months. She had no endocrine disorders, her menses were normal, and no lymphoma or other tumor could be found. Tests showed she was not allergic to any fungi, but she did have a positive L.E. test for lupus erythematosus and a large number of eosinophils (32%) in her blood. The latter spoke to the presence of an allergic reaction, and the former to a sensitization to her own tissues, an autoimmune process.

It was very strange how resistant a twenty-three-year-old woman's acne was to treatment. For months we watched one treatment after another fail. Her face continued to exhibit a full complement of blackheads, redness, and pustules at every visit. Local treatments achieved little, other than camouflage. A full array of systemic antibiotics staggered from the fray, accomplishing little more than inducing a yeast vaginitis. Even the acclaimed Accutane® failed, despite its advertising hype, “you've never seen results like this.”

Was there a hormonal problem? No, her menses were normal and androgen, the prime mover of acne, was shown to be at low levels in her blood.

Was it genetic? No one in the family had had serious acne. Was it due to food imbalance? We had seen a case of terribly resistant acne in a Naval cadet who, disliking the Navy chow, ate nothing but Hershey bars. Could it be due to an intake of nuts, long known to us as a cause of postadolescent acne? No, her diet was balanced and a nut-free regimen had not had the slightest effect. Could it be due to a drug, such as dilantin? No, she was healthy and avoided all medications.

We explored her cosmetic history. No, she did not use occlusive greasy makeup which produces poral closure with subsequent acne. Nor did she use sunscreen lotions. Furthermore, she used no hair pomades. Her habits were obviously not the cause of her distress.

“I've had eight years of rings on my skin and I'm tired of them. The only good news is they only come in the winter,” complained a thirty-nine-year-old woman. At first her lesions had been limited to her forearms, but in the ensuing winters the ringed red cords had spread to involve her trunk, arms and legs. They would start out as bumps, then enlarge and clear in the center, leaving mildly itchy red rings that sort of resembled hives. Cortisone would clear them, but they always came back to stay until May.

We knew what she had, but we did not know why. Her diagnosis was erythema annulare centrifugum, which translates into red rings expanding centrifugally while clearing centrally. But what causes it? Again, like so many other skin diseases, it does not have a single cause, but is a reaction pattern to many different things. It can have many patterns, such as polycyclic, arcuate, annular, festooned, serpentine, figurate, geographic, or annular (ring-shaped). Although the shape of the lesions belies the diagnosis, it does not reveal the cause.

Her long-suffering winters led us to admit her to the hospital. We wanted to know for sure that her rings were not a sign of internal malignancy. Fortunately, extensive studies revealed no underlying cancer or any other disease. She had no intestinal parasites found on stool examination, and her tests for lupus erythematosus were negative.

“My daughter has got it again. She had to be hospitalized last time and no one knows what it is. Please help.” We didn't practice on Saturdays, but early this Saturday morning we met a seven-year-old girl for an emergency evaluation of a fiery red rash, which was symmetrical on her cheeks, armpits, groin, and buttocks. It had come out just four hours ago and her mother was frantic. This was the third time her daughter had been stricken, each time in the same exact areas.

We learned that the first attack had occurred two years ago following a cough and runny nose, and had necessitated three days of hospitalization. There was no fever. Treatment with aspirin and an antihistamine resulted in fading of the rash without any trace later that week. Extensive studies revealed no cause for the rash, so the noncommittal diagnosis of “toxic erythema” was made. The second attack came just ten months ago. Again, no cause could be found, but hospitalization and treatment with intramuscular cortisone was necessary.

We continued with the history. Her parents and a three-year-old brother had remained well on each occasion. Yet, on this Saturday morning the girl looked and felt sick. Her joints were tender and swollen. She had complained of malaise the previous night and had slept poorly. The only medication given had been a teaspoonful of Night Time Cold Formula®, which contained pain relievers, a cough suppressant, and a decongestant.

“Did these hives come from my truck accident or didn't they?” asked a twenty-eight-year-old man. Five weeks before the hives appeared he had almost been killed in a truck accident. Now, a year later, the hives continued. We did not know the answer to his question, but were eager to find out. His hives were the most unusual we had ever seen. They were small red mosquito bite-like swellings, but each was surrounded by a remarkable halo of white blanched skin.

We were familiar with cholinergic urticaria, small hives resulting from heat, exercise, or emotional stress. But, these hives have halos of redness, not blanching. They result from an abnormal sensitivity to the neurotransmitter, acetylcholine. Injection of this chemical into the skin exactly reproduces the small hives with red circles. When patients say their hives are the result of “nerves,” we are usually skeptical, except for this, the only authentic example. For most patients hives are not due to nerves, but result from an allergic response to any one of a thousand allergens. Our patient did not have cholinergic urticaria. He had white halo hives, not the red halo-type due to acetylcholine release from stress, heat, or exercise.

Yet, could his hives also be due to nerves, perhaps with a different neurotransmitter? No one had ever described such hives, yet his history clearly implicated “nerves” as a possibility. His truck accident left him with a morbid fear of driving.

“Can I get off cortisone?” was the plaintiff plea of this new patient. “It makes my face so puffy.” She was fifty-one years of age and had suffered a terrible widespread itch since she was twenty-eight years old. It tore holes in her stockings. It kept her awake nights.

She had been told it was due to stress. She was serving on too many committees. “Relax and the itch will go away,” but it did not. It followed her to what should have been pleasant carefree Caribbean vacations. It followed her on her move halfway across the country to a new home. As we watched her scratch, we realized it had followed her into our office. Topical creams, lotions, and sprays were only momentary distractions. Her fingernails were polished from incessant scratching. And, the more she scratched, the more the new spots appeared.

There was no mystery as to her diagnosis. She had generalized lichen planus, now a twenty-three year itch. It had been repeatedly diagnosed clinically and histologically by a half dozen doctors. She had been told that no cause is known and cortisone could give her relief. And it had, but she wanted to escape the side effects of the cortisone she had been taking for so many years. Despite her edematous face and the bulge on the nape of her neck, we could find none of the more serious side effects of cortisone, such as hypertension, peptic ulcer, or osteoporosis.

“Showers give me hives,” said a fifteen-year-old girl. She startled us with her complaint. Could she really have an allergy to water? Careful questioning revealed it had all begun with the sudden appearance of hives thirty minutes after waterskiing last summer. The hives were small mosquito bite-like bumps which appeared over her trunk and upper arms. Each hive had a distinctive red zone around it. They all gave a fierce itch, until fading away in an hour or two, leaving no trace.

Since that time, every bath and shower has been followed within minutes by these same small itchy hives. Indeed, swimming, sweating, or vigorous exercise did the same thing, although being under stress was not a trigger. She got relief by taking an antihistamine before bathing or showering.

We quickly proved the history was no hoax. Placing a wet towel on her upper back induced a fine crop of hives in that area within five minutes after it was removed.

But why? Was it something in the water? Was it the temperature? Distilled water, sea water, and salt solutions all elicited the same hives, whereas contact with alcohol or mineral oil produced no hives. In fact, coating the skin with oil prior to showering or adding bath oil to the bath reduced the severity of the attacks. Temperature played no role; hot as well as cold water produced the hives.

We could find no disease responsible, and extensive blood studies were negative.

A fourteen-year-old girl came with a history of developing large black and blue spots on her arms, thighs, and abdomen. There was no pattern to the lesions, nor could we elicit a monthly or seasonal cycle. The girl denied any symptoms of itch, tenderness or pain. She would awaken to find new ones, which then slowly faded away with a bluish, at times yellowish, color. Her parents were mystified. She had seen a hematologist, whose studies on her showed normal bleeding and clotting functions. She had plenty of platelets so essential in keeping the blood within the blood vessel walls. There was no evidence of an eating disorder which could cause scurvy from vitamin C deficiency. She did not have the black and blue spots of the thin skin of the aged. And, she did not participate in any bruising sports.

A detailed history was essential. We recalled the hurried Monday office visit of a patient worked in as an emergency after she had suddenly developed dozens of black and blue spots. We rushed her to the laboratory before closing time for a complete (and expensive) battery of blood studies. We called for the results of our “stat” bloods only to hear the technician say, “You mean the results on that patient with all the black and blue spots from white water rafting yesterday? They are normal.”

“It all began with blisters behind my ears five months ago,” a sixty-nine-year-old woman told us on her first visit. It would be many visits later before we could find the cause.

She described the blisters as initially being small and very itchy. As the months went by, new blisters appeared on her face, chest and, most dramatically, over her left thigh. She had always been in good health with no prior skin disease. But two years ago she had fractured her left hip. This required extensive orthopedic care, involving screws, bone grafting with a buttress plate, and the implant of an osteogenic bone growth stimulator.

On examination the blisters were large, tense, and filled with a clear fluid, while the surrounding skin was red and slightly elevated. Rubbing the skin firmly did not produce any new blisters. This simple sign (Nikolsky) helped rule out the grave diagnosis of pemphigus.

Clinically, her problem appeared to be the blistering eruption called “bullous pemphigoid,” usually seen in older individuals. However, skin biopsies did not confirm this diagnosis, showing only that the epidermis was lifted off of the dermis by the blister fluid. Extensive blood studies told us that she had a very high serum level of the immune protein, IgE, seen in allergic individuals.

We were able to suppress the blisters with intramuscular cortisone shots and an oral antibiotic, but the problem was not really solved.

“What are these blue spots?” A mother had brought in her three-year-old daughter. Her story was astonishing. Her daughter had diabetes and she had developed pinpoint blue spots on her thighs, buttocks, and arms, each at the site of certain insulin injections. The spots did not itch and were not tender, but had not faded over a period of months. The mother had also noticed blue-black spots on the top of the rubber stopper of each insulin bottle as it was used, including both regular and protamine insulin. Puzzled by the pigment on the rubber caps, they switched from silicone-treated needles to polymer-coated needles. Both types were standard disposable sterile 28-gauge hypodermic needles. They made no change in the insulin brand. Once this was done, neither their child nor the rubber stoppers got any more blue spots. But why? What caused the blue spots?

Examination revealed about twenty blue to blue-black punctate spots on each upper arm with a scattering of these same points of pigment on the thighs and buttocks. They looked like tattoo spots, and a skin biopsy of one showed a streak of fine black particles extending down into the skin. Obviously, they were along the path of a needle insertion. They did not reflect light as seen with talcum powder. A special stain showed they were not iron, and x-ray spectrometer studies proved they were not of metabolic origin.

Examination of the silicone-treated needles and syringes showed no pigment on flushing with either saline or insulin.

“My itch is terrible, but I know what causes it. My itch is caused by a bug.” This sixty-three-year-old man had incessant widespread crawling and biting sensations in his skin for three years. He blamed tiny bugs that were not directly visible on the skin, but could be seen by him when he brushed them off into a wash basin.

It was a serious matter for which he had previously consulted three dermatologists, an infectious disease specialist, a pathologist, a microbiologist, a psychiatrist, an entomologist, and three parasitologists. No one had ever been able to identify any insect, ectoparasite, fungal element, or glass wool in his many many samples. Nonetheless, he spent most of each day cleaning, spraying, and vacuuming his home to eliminate his “pathogens.” He had bought an industrial vacuum cleaner, special air filters, and cartons of disinfectant sprays … anything to kill the bugs. At night he was unable to sleep due to scratching. His life was truly miserable.

At each visit he came equipped with a basin of water, colored blue with a food dye. He averred that the bugs were grossly visible when he brushed them off into the basin if the overhead lights were turned off, illumination being provided by transverse lighting from his flashlight. Strangely, these bugs had no bodies that he could see, but were essentially jagged legs.

Repeated examinations with a 2.75 power lens of the patient's “parasites” floating in the water revealed only dead keratin flakes of his outer skin.

“My fingertips hurt so much, I can't work,” was the tearful complaint of this forty-year-old hairdresser. We looked at her fingertips and there was not a thing to be seen. They were absolutely normal; yet, touching them made her wince. Squeezing them made her scream. What was wrong? Was she malingering? Did she have a neurologic disease?

This was no ordinary case. She had seen a posse of physicians but no one had caught the criminal. The neurologists gave her a good bill of health and so did the psychiatrist. Her family physician had no special insight nor was the report of the peripheral vascular specialist helpful. Several dermatologists had provided no enlightenment.

Well, first things first. What was the exact history? The problem began last fall as marked sensitivity of the lateral side of the tip of the right fourth finger. Shortly thereafter, the medial tips of both thumbs were similarly affected. Eventually, all of the fingers became involved to a greater or lesser degree. Inflammatory changes were absent, but exquisite tenderness to touch continued and pressure on the fingertips always induced pain. There were no other sensory changes and her toes were not affected.

Her past history was noncontributory. Neither she nor anyone in her family had experienced neurologic or psychiatric problems. There was no history of skin disease or diabetes mellitus. She had worked for many years as a hairdresser, but knew of no new contactants at work or home.

“What can you do for my hives?” It would seem that there was nothing more that could be done for this twenty-seven-year-old woman's hives. She already had been carefully studied and treated by a succession of doctors, including an allergist, an internist, and a dermatologist. She had even been hospitalized twice. No cause had ever been found and the hives could be controlled only with steroids.

The attacks had begun six years ago. At first they were only occasional visitors, but soon they developed into severe generalized hives which came every day. For the past three years, whenever she scratched her skin, a hive came up.

The salient features in her hive history were: 1) onset after an automobile accident; 2) anaphylactic shock after injection of the drug, ACTH, suggestive of an allergy to pork; 3) no family history of hives; 4) no association of the hives with either the menses or taking aspirin; 5) usually worse in the morning hours.

Thus began a three-year odyssey of study, tests, and therapeutic trials. Routine blood studies, as well as tests for lupus, were repeatedly normal, as were x-rays of her chest and teeth. There were no dental abscesses evident. Biopsy of the skin showed only the local accumulation of fluid, typical of urticaria.

The absence of basophils from her blood, and the presence of dermographism (skin writing) indicated to us that at all times she carried the antigen responsible for her hives. But what was it?

You have your whole day filled with removals and restorations, to say nothing of documentation and administration. Why become a Sherlock Holmes investigating the crimes committed in your patient's skin? Here's why:

Skin sleuthing is fun. Solving a patient's problem makes for a better day, perhaps not financially, but intellectually. It may take weeks, but the delight of satisfying your curiosity for the benefit of your patient is great. It is not enough to repair a wrecked car. You must know why there was a wreck.

Solving problems, explaining the inexplicable, is a higher order of achievement than making diagnoses. And, bear in mind, your patient is much less interested in the “what” of dermatology than the “why,” but really wants to know both.

So, you want to be a Dermatologic Sherlock Holmes. It is not enough to don a deerstalker cap and smoke a curved pipe. You must acquire skills in these four areas:

THE HISTORY

Because many dermatologic diagnoses are simply labels for reaction patterns, it is up to you to look beyond the diagnosis and look for the cause. This is done best with a sharply focused history directed at probable or possible causes. All this calls for knowledge that comes from experience, from reading the literature, and above all, asking the patient what he or she feels is the cause.

We recall a patient at the University of Minnesota who presented with a swollen painful tongue.

“I am allergic to sunshine” was the complaint of a fifty-nine-year-old man. “And, I have been so for seven years.” Allergy is a wonderfully creative word. Allergy, virus infection, and nerves are used to explain nearly all of a patient's inexplicable problems. Allergy, especially, has many definitions. We recall having offered to buy ice cream cones for two little girls. The older one said, “But my sister can't have chocolate. She's allergic to chocolate.” Really? “Yes, she smears it all over her dress and it stains.”

So, the question was, what was his allergy? We learned that the exposed areas of his forearms, ears, and forehead would become bright red and even blister after just five minutes of direct exposure to sunlight. Later, the areas itched fiercely for a day or two, and then the skin returned to normal. This allergy had kept him indoors for those seven years.

His prior history revealed a gastrectomy thirteen years ago with two-thirds of his stomach having been removed. He took no medicines, used only Ivory Soap for cleansing, and applied no hair sprays, colognes or after shave lotions. Alcohol intake was limited to an occasional beer. There had been no diarrhea or mental disturbance. He slept poorly, however, and said he always felt nervous.

His skin appeared normal, but phototesting with direct sunlight in July revealed a minimal erythema dose of five minutes (the minimum exposure time to give redness).

“Why can't I sweat?” was a question of a twenty-three-year-old roofer. He knew he couldn't sweat. He had avoided the heat as a youngster, knowing that headaches, nausea, and shortness of breath always came with hot weather. He became lightheaded and feverish in the hot sun. His parents had told him it was a family trait, with his grandfather and aunt having the same problems.

He had long complained of tender painful fingers and toes, as well as strange red dots around his “belly button.” The pain in his fingers became much worse when he exercised or became overheated. And the red dots were becoming more numerous. Now they were also on his thighs and scrotum.

As a child he had been able to avoid conditions of high temperature. But now, working in the heat was unavoidable. An hour's work triggered a fever of over 100°F. At work he kept a sponge and a 2-gallon jug of water at his side. He had absolutely no sweat for the evaporative cooling we all enjoy. He had never been seen by a dermatologist, but now, after twice collapsing and becoming unconscious at work, he came to see us about his lack of sweating.

The only thing we saw were numerous pinhead-size reddish purple spots, looking like raised tuffs of blood vessels in the periumbilical, pelvic, genital, and thigh areas. The blood vessels of his eyeballs were also enlarged and tortuous.

“I get hives when I get overheated” was the complaint of a thirty-year-old woman. But the problem was more complex than this. Unraveling her lengthy history we learned that the hives came out not only from being in a hot room, but also whenever she took a hot bath. Furthermore, she suffered from attacks of intense flushing of her skin. All this could be brought on also by emotional stress. Even exercise triggered her problem. She had suffered these attacks for six years without relief, and they were increasing in severity and frequency. In the past year during attacks she had experienced intense itching of her entire skin, along with feelings of weakness, headaches, and palpitations. She had consulted many doctors and taken a wide range of medications without help.

The gravity of her problem demanded hospitalization for complete analysis. With blood studies we were able to rule out the two rare tumors known to produce episodic flushing, carcinoid and pheochromocytoma. A strict elimination diet and avoidance of all medications also did nothing to lessen her attacks. Day by day, as we weathered her attacks with her, we realized her lesions began as hives with tiny red halos, characteristic of so-called “cholinergic hives.” In such cases patients become sensitive to acetylcholine, a chemical released from sympathetic nerve endings in times of stress. This compound also causes blood vessels to dilate, which leaves the skin with a red flush.

“Black sweat” was the complaint of a twenty-nine-year-old woman. For ten long years she had suffered from black droplets appearing all over her face whenever she became excited, tense, or overheated. She had seen numerous doctors who had no explanation and no cure. Indeed, many had doubted her story, because her skin appeared perfectly normal at the time of her office visit. The burst of black droplets would appear when she was dancing, and her partner would wipe them away, remarking, “you've got soot on your face.”

Of far more concern than such embarrassment to this young woman was her mounting anxiety. Could the “black sweat” be a sign of black cancer? Was it a sign of the black plague? Yet, she seemed in perfect health. But neither she, nor anyone she knew, had knowledge of such an ominous secretion. Her doctors could provide no reassurance, because her problem was not in their textbooks of medicine. She was truly an “orphan patient.”

When we first saw her, we were equally puzzled. We had only her history. Her skin was perfectly normal. There were no black spots. Could she be hallucinating? Could it be a form of the bloody sweat described as a stigmatization as in the case of Saint Therese Neumann? Could it be of hysterical origin? Or could it be a case of deception?

We had little to go on in the first visit.

“My friends tell me I'm growing old, but only on one side” was the complaint of a sixty-two-year-old woman. And her friends were so right. This woman, in her sixties, had the aged wrinkled appearance of a woman in her eighties, but only on the right side of her face, the right side of her neck, and her right hand. She had not always looked that way. Over the past year she had noticed the one-sided wrinkles appearing asymptomatically and insidiously. There had been no prior redness, swelling, or pain nor had she had any episodes of toothache, sinusitis, or parotitis (inflammation of the parotid gland), or any other infections. She had never had any trauma to the area, radiation therapy, or unusual ultraviolet light exposure to her skin (in or out of automobiles). She had never had any skin disease. There was no family history of premature aging or wrinkling of the skin. Her health had been excellent.

On close examination the affected skin, especially over her right cheek, was thin and loose, hanging in numerous deep wrinkles. The underlying fat layer felt reduced in thickness. The skin of the left side had the normal appearance for a person her age and was essentially without wrinkles. Neither side showed the aging spots or keratoses of sun damage. The skin on her right wrist and hand also had an aged wrinkled appearance. The covered parts of her body did not show any wrinkling.