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Sometimes You Can’t Blame the Sun
ANSWER
The correct answer is all of the above (choice “e”).
DISCUSSION
Most cases of dermatomyositis, which the patient’s presentation and lab results suggested, require nerve conduction studies, a check of serum aldolase levels, and skin and muscle biopsies to complete the work-up. However, the arrival at a diagnosis is only the first step.
Patients with dermatomyositis, particularly those older than 60, require evaluation for occult malignancy. There is evidence that the body’s immune response to the cancer is what drives the disease process. Hence the need for the studies listed, looking for breast, lung, and gastrointestinal cancers especially.
Dermatomyositis is thought to be an inflammatory myopathy, possibly driven by autoimmune factors. It is rare (about 1 to 22 per 100,000) and affects women more than men.
The “sunburn” rash is typical, especially on the face, chest, and dorsal hands, and usually clears completely when the cancer is found and cured. Other common findings include elevated creatine kinase, hand rashes (known as Gottron’s papules), and dystrophic calcification in skin and/or joints.
TREATMENT
Aside from addressing a possible malignancy, treatment of dermatomyositis usually starts with glucocorticoids, eventually tapered and replaced by steroid-sparing agents such as azathioprine or cyclosporine. These drugs have dramatically increased the chances of survival and eventual cure.
It’s common for the photosensitivity to persist long after the myositis has resolved, which is why sunscreen and other sun-protective measures are advised.
ANSWER
The correct answer is all of the above (choice “e”).
DISCUSSION
Most cases of dermatomyositis, which the patient’s presentation and lab results suggested, require nerve conduction studies, a check of serum aldolase levels, and skin and muscle biopsies to complete the work-up. However, the arrival at a diagnosis is only the first step.
Patients with dermatomyositis, particularly those older than 60, require evaluation for occult malignancy. There is evidence that the body’s immune response to the cancer is what drives the disease process. Hence the need for the studies listed, looking for breast, lung, and gastrointestinal cancers especially.
Dermatomyositis is thought to be an inflammatory myopathy, possibly driven by autoimmune factors. It is rare (about 1 to 22 per 100,000) and affects women more than men.
The “sunburn” rash is typical, especially on the face, chest, and dorsal hands, and usually clears completely when the cancer is found and cured. Other common findings include elevated creatine kinase, hand rashes (known as Gottron’s papules), and dystrophic calcification in skin and/or joints.
TREATMENT
Aside from addressing a possible malignancy, treatment of dermatomyositis usually starts with glucocorticoids, eventually tapered and replaced by steroid-sparing agents such as azathioprine or cyclosporine. These drugs have dramatically increased the chances of survival and eventual cure.
It’s common for the photosensitivity to persist long after the myositis has resolved, which is why sunscreen and other sun-protective measures are advised.
ANSWER
The correct answer is all of the above (choice “e”).
DISCUSSION
Most cases of dermatomyositis, which the patient’s presentation and lab results suggested, require nerve conduction studies, a check of serum aldolase levels, and skin and muscle biopsies to complete the work-up. However, the arrival at a diagnosis is only the first step.
Patients with dermatomyositis, particularly those older than 60, require evaluation for occult malignancy. There is evidence that the body’s immune response to the cancer is what drives the disease process. Hence the need for the studies listed, looking for breast, lung, and gastrointestinal cancers especially.
Dermatomyositis is thought to be an inflammatory myopathy, possibly driven by autoimmune factors. It is rare (about 1 to 22 per 100,000) and affects women more than men.
The “sunburn” rash is typical, especially on the face, chest, and dorsal hands, and usually clears completely when the cancer is found and cured. Other common findings include elevated creatine kinase, hand rashes (known as Gottron’s papules), and dystrophic calcification in skin and/or joints.
TREATMENT
Aside from addressing a possible malignancy, treatment of dermatomyositis usually starts with glucocorticoids, eventually tapered and replaced by steroid-sparing agents such as azathioprine or cyclosporine. These drugs have dramatically increased the chances of survival and eventual cure.
It’s common for the photosensitivity to persist long after the myositis has resolved, which is why sunscreen and other sun-protective measures are advised.
A 60-year-old woman was referred to dermatology for evaluation of “sunburn.” The rash was painful and unrelieved by topical medications, including class IV steroid creams. The redness was tender and warm to touch.
The rash had been present for months. During this period, the patient also had grown increasingly weak, leading her to quit her job. In the clinic, she was unable to stand from a seated position without difficulty. She reported no other health concerns and had quit smoking 5 years previously, after 30 years.
On examination, diffuse blanchable macular erythema on the patient’s face and chest was immediately observed. There was also an odd rash, composed of hundreds of tiny confluent papules, concentrated over the interphalangeal joints and dorsal hands. These too were warm and tender to touch. Most of her cuticles were peeling off; closer examination under magnification revealed tortuous capillaries on the distal cuticles of several fingers.
Bloodwork revealed a creatine kinase level slightly greater than 1000 U/L, and a positive antinuclear antibody, dilution unknown.
In (K)need of Help
ANSWER
The correct answer is bullous impetigo (choice “a”).
Had the source of the problem been MRSA (choice “b”), there would have been marked tenderness and swelling. Psoriasis vulgaris (choice “c”) was a possibility; however, it almost never manifests with blisters, it rarely comes on as quickly as this patient’s problem did, and it produces scale that is far thicker and more tenacious than that seen in bullous impetigo. Nummular eczema (choice “d”) does not manifest with blisters and would likely have caused itching.
DISCUSSION
Impetigo is one of many common skin diseases—other examples include granuloma annulare and lichen planus—that have bullous variants, which can make diagnosis challenging. Impetigo is easy to diagnose in its more common papulosquamous form. But it also can manifest with flaccid blisters that last only a short time, leaving the round, scaly lesions seen in this case.
Staphylococcus aureus, the organism responsible for bullous impetigo, elaborates serine proteases, which bind to and cleave desmoglein-1. This effectively destroys the connections between skin layers, creating a space that is quickly filled with serum. The level of this separation is typically subcorneal, which allows the formation of a very thin, friable roof for the bulla.
This modern version of impetigo is not considered dangerous, despite its association with staph aureus. In pre-antibiotic times, the predominant organism was streptococcal, some types of which could be nephritogenic—that is, capable of causing Bright disease or, as it is now known, acute post-streptococcal glomerulonephritis. Fortunately, this potentially fatal condition is only rarely seen in modern times.
Two items of note about this case: Bullous impetigo, contrary to what was seen in this patient, typically favors intertriginous areas. And a key factor is the history of atopy, which renders the patient more susceptible to skin infections of all kinds.
Treatment
The patient responded well to topical mupirocin ointment, applied three times a day. In rare instances, impetigo can require an oral antibiotic, such as cephalexin.
ANSWER
The correct answer is bullous impetigo (choice “a”).
Had the source of the problem been MRSA (choice “b”), there would have been marked tenderness and swelling. Psoriasis vulgaris (choice “c”) was a possibility; however, it almost never manifests with blisters, it rarely comes on as quickly as this patient’s problem did, and it produces scale that is far thicker and more tenacious than that seen in bullous impetigo. Nummular eczema (choice “d”) does not manifest with blisters and would likely have caused itching.
DISCUSSION
Impetigo is one of many common skin diseases—other examples include granuloma annulare and lichen planus—that have bullous variants, which can make diagnosis challenging. Impetigo is easy to diagnose in its more common papulosquamous form. But it also can manifest with flaccid blisters that last only a short time, leaving the round, scaly lesions seen in this case.
Staphylococcus aureus, the organism responsible for bullous impetigo, elaborates serine proteases, which bind to and cleave desmoglein-1. This effectively destroys the connections between skin layers, creating a space that is quickly filled with serum. The level of this separation is typically subcorneal, which allows the formation of a very thin, friable roof for the bulla.
This modern version of impetigo is not considered dangerous, despite its association with staph aureus. In pre-antibiotic times, the predominant organism was streptococcal, some types of which could be nephritogenic—that is, capable of causing Bright disease or, as it is now known, acute post-streptococcal glomerulonephritis. Fortunately, this potentially fatal condition is only rarely seen in modern times.
Two items of note about this case: Bullous impetigo, contrary to what was seen in this patient, typically favors intertriginous areas. And a key factor is the history of atopy, which renders the patient more susceptible to skin infections of all kinds.
Treatment
The patient responded well to topical mupirocin ointment, applied three times a day. In rare instances, impetigo can require an oral antibiotic, such as cephalexin.
ANSWER
The correct answer is bullous impetigo (choice “a”).
Had the source of the problem been MRSA (choice “b”), there would have been marked tenderness and swelling. Psoriasis vulgaris (choice “c”) was a possibility; however, it almost never manifests with blisters, it rarely comes on as quickly as this patient’s problem did, and it produces scale that is far thicker and more tenacious than that seen in bullous impetigo. Nummular eczema (choice “d”) does not manifest with blisters and would likely have caused itching.
DISCUSSION
Impetigo is one of many common skin diseases—other examples include granuloma annulare and lichen planus—that have bullous variants, which can make diagnosis challenging. Impetigo is easy to diagnose in its more common papulosquamous form. But it also can manifest with flaccid blisters that last only a short time, leaving the round, scaly lesions seen in this case.
Staphylococcus aureus, the organism responsible for bullous impetigo, elaborates serine proteases, which bind to and cleave desmoglein-1. This effectively destroys the connections between skin layers, creating a space that is quickly filled with serum. The level of this separation is typically subcorneal, which allows the formation of a very thin, friable roof for the bulla.
This modern version of impetigo is not considered dangerous, despite its association with staph aureus. In pre-antibiotic times, the predominant organism was streptococcal, some types of which could be nephritogenic—that is, capable of causing Bright disease or, as it is now known, acute post-streptococcal glomerulonephritis. Fortunately, this potentially fatal condition is only rarely seen in modern times.
Two items of note about this case: Bullous impetigo, contrary to what was seen in this patient, typically favors intertriginous areas. And a key factor is the history of atopy, which renders the patient more susceptible to skin infections of all kinds.
Treatment
The patient responded well to topical mupirocin ointment, applied three times a day. In rare instances, impetigo can require an oral antibiotic, such as cephalexin.
The parents of a 6-year-old boy were quite concerned about several lesions on the child’s left knee. The asymptomatic blisters had first appeared about 2 weeks prior. A topical steroid cream prescribed by the family’s primary care provider had not helped.
No one else in the family was similarly affected. They all were reportedly quite healthy, although all were atopic—prone to seasonal allergies and eczema.
Examination revealed at least 6 round, scaly, red lesions on the patient’s knee, ranging from 3 mm to 3 cm in diameter. According to the parents, these had first appeared as intact blisters. There was little to no tenderness or redness around the lesions, and there was no palpable adenopathy in the groin.
The child was in no distress and was afebrile.
Adding Insult to Injury
ANSWER
The correct answer is inclusion cyst (choice “c”).
DISCUSSION
Inclusion cysts are also called traumatic inclusion cysts or implantation cysts and are quite distinct from “sebaceous,” epidermal, or epidermoid cysts. An inclusion cyst results from traumatic implantation of surface adnexal structures (eg, sebaceous glands) that continue to function, eventuating in the formation of an organized sac whose wall is composed of stratified squamous epithelium with a granular layer, no significant atypia, and surrounding pasty lamellated acellular keratin.
Hands are the most commonly affected area, although the precipitating puncture wound doesn’t have to be as impressive as this patient’s was. Nails and sewing needles can produce the same result.
The patient’s lesion was removed, at which point its pasty contents (a diagnostic clue) were revealed, and the wound closed. Although the absence of redness or tenderness helped to rule out some items in the differential (eg, felon, abscess), and the lesion demonstrated clear cystic features, the specimen was sent for pathologic examination for confirmation, since cancer would also belong in the differential for such a lesion.
ANSWER
The correct answer is inclusion cyst (choice “c”).
DISCUSSION
Inclusion cysts are also called traumatic inclusion cysts or implantation cysts and are quite distinct from “sebaceous,” epidermal, or epidermoid cysts. An inclusion cyst results from traumatic implantation of surface adnexal structures (eg, sebaceous glands) that continue to function, eventuating in the formation of an organized sac whose wall is composed of stratified squamous epithelium with a granular layer, no significant atypia, and surrounding pasty lamellated acellular keratin.
Hands are the most commonly affected area, although the precipitating puncture wound doesn’t have to be as impressive as this patient’s was. Nails and sewing needles can produce the same result.
The patient’s lesion was removed, at which point its pasty contents (a diagnostic clue) were revealed, and the wound closed. Although the absence of redness or tenderness helped to rule out some items in the differential (eg, felon, abscess), and the lesion demonstrated clear cystic features, the specimen was sent for pathologic examination for confirmation, since cancer would also belong in the differential for such a lesion.
ANSWER
The correct answer is inclusion cyst (choice “c”).
DISCUSSION
Inclusion cysts are also called traumatic inclusion cysts or implantation cysts and are quite distinct from “sebaceous,” epidermal, or epidermoid cysts. An inclusion cyst results from traumatic implantation of surface adnexal structures (eg, sebaceous glands) that continue to function, eventuating in the formation of an organized sac whose wall is composed of stratified squamous epithelium with a granular layer, no significant atypia, and surrounding pasty lamellated acellular keratin.
Hands are the most commonly affected area, although the precipitating puncture wound doesn’t have to be as impressive as this patient’s was. Nails and sewing needles can produce the same result.
The patient’s lesion was removed, at which point its pasty contents (a diagnostic clue) were revealed, and the wound closed. Although the absence of redness or tenderness helped to rule out some items in the differential (eg, felon, abscess), and the lesion demonstrated clear cystic features, the specimen was sent for pathologic examination for confirmation, since cancer would also belong in the differential for such a lesion.
A 38-year-old man sustained an on-the-job injury to his finger, which was punctured by a tiny drill bit. He was promptly seen by a clinician, who administered a tetanus injection and prescribed a 2-week course of an antibiotic. However, within a few weeks, the patient noticed a lesion on the affected finger. It caused little to no pain but grew steadily, until it interfered with his functioning and prompted a return to the clinic.
The patient was in good health, despite being a long-term smoker. X-rays of the finger showed no bony changes or evidence of a foreign body. A referral to dermatology was provided.
Examination revealed a firm, painless, 2-cm subcutaneous mass in the pad of his left index finger. The surface felt quite tense, but there was no disruption nor any redness. Palpation of relevant nodal locations failed to reveal any palpable masses.
Facing Up to the Diagnosis
ANSWER
All of these items were rightly considered to be in the differential for this lesion, so the answer is choice “f.”
DISCUSSION
Individuals with type IV skin are less likely than those with types II and III to develop skin cancer. So, although cancer was definitely in the differential, the other items were considered just as, if not more, likely in this case.
The only way to sort through these diagnostic possibilities was to perform a biopsy. In this case, the entire lesion was removed by saucerization technique, under local anesthesia. The specimen provided would be adequate to detect any cancer, which a smaller specimen could easily miss.
The pathology results showed pigmented basal cell carcinoma. Given the patient’s extensive history of sun exposure, and the steady growth of the lesion, this was hardly a surprise. But prior to the biopsy, one could just as easily imagine the lesion to be, for example, a wart.
The take-home message is obvious: Nothing can take the place of biopsy in establishing a precise diagnosis. With that information in hand, the patient was referred for consultation with a Mohs surgeon. Surgical removal and closure would likely set him back several thousand dollars and leave a considerable scar.
ANSWER
All of these items were rightly considered to be in the differential for this lesion, so the answer is choice “f.”
DISCUSSION
Individuals with type IV skin are less likely than those with types II and III to develop skin cancer. So, although cancer was definitely in the differential, the other items were considered just as, if not more, likely in this case.
The only way to sort through these diagnostic possibilities was to perform a biopsy. In this case, the entire lesion was removed by saucerization technique, under local anesthesia. The specimen provided would be adequate to detect any cancer, which a smaller specimen could easily miss.
The pathology results showed pigmented basal cell carcinoma. Given the patient’s extensive history of sun exposure, and the steady growth of the lesion, this was hardly a surprise. But prior to the biopsy, one could just as easily imagine the lesion to be, for example, a wart.
The take-home message is obvious: Nothing can take the place of biopsy in establishing a precise diagnosis. With that information in hand, the patient was referred for consultation with a Mohs surgeon. Surgical removal and closure would likely set him back several thousand dollars and leave a considerable scar.
ANSWER
All of these items were rightly considered to be in the differential for this lesion, so the answer is choice “f.”
DISCUSSION
Individuals with type IV skin are less likely than those with types II and III to develop skin cancer. So, although cancer was definitely in the differential, the other items were considered just as, if not more, likely in this case.
The only way to sort through these diagnostic possibilities was to perform a biopsy. In this case, the entire lesion was removed by saucerization technique, under local anesthesia. The specimen provided would be adequate to detect any cancer, which a smaller specimen could easily miss.
The pathology results showed pigmented basal cell carcinoma. Given the patient’s extensive history of sun exposure, and the steady growth of the lesion, this was hardly a surprise. But prior to the biopsy, one could just as easily imagine the lesion to be, for example, a wart.
The take-home message is obvious: Nothing can take the place of biopsy in establishing a precise diagnosis. With that information in hand, the patient was referred for consultation with a Mohs surgeon. Surgical removal and closure would likely set him back several thousand dollars and leave a considerable scar.
“Like a berry,” was how a 38-year-old Hispanic man described the lesion that had been slowly growing on his face for 4 years. His family was alarmed by it, but he reasoned that since it didn’t hurt and the surrounding area wasn’t especially red, it likely wasn’t much of a problem.
The patient worked as roofer up to 7 days per week when the weather was good and had been doing so since he was old enough to work. He had no insurance and was not inclined to spend money on a health care visit. When his wife finally convinced him to go to the urgent care clinic, he paid $100 just to be told he needed to see a dermatologist. He was so disgusted he almost refused to wait the 6 weeks it took to get into the dermatology office.
The patient, who had type IV skin with little evidence of sun damage, had an obvious, large, coarsely mamillated nodule on the left upper nasolabial area. The lesion measured 1.8 cm and was reddish blue. It was moderately firm, but no increased warmth could be detected. No nodes could be felt in the area. His skin elsewhere was free of any notable changes.
Explaining Away Those Shades of Gray
ANSWER
The correct answer is that new hairs growing in to replace those lost from alopecia areata tend to be white (choice “b”). They usually regain their normal color, eventually.
DISCUSSION
Alopecia areata is an autoimmune phenomenon implying an increased tendency to develop other autoimmune diseases (eg, vitiligo [choice “a”], which can appear initially in the scalp).
This case turned out to be simple but had the potential to be far more serious. The biopsy of the dark patch showed benign seborrheic keratosis, but it was possible that another section could have demonstrated features of melanoma (choice “c”). When present, melanoma can occasionally trigger an immune response that destroys pigment cells in hair follicles, causing the hairs to lose their pigment. This is why the entire dark patch was later excised. Fortunately, the pathology report ruled out melanoma.
While it has been reported that stress can cause hair to turn gray (choice “d”), there were better (and more accurate) explanations for this patient’s presentation.
This case, though fairly straightforward, serves as a reminder that it is our job as clinicians to connect the dots to rule out worst-case scenarios.
Outcome
This patient’s hair all grew back, regaining its normal color, without any treatment.
ANSWER
The correct answer is that new hairs growing in to replace those lost from alopecia areata tend to be white (choice “b”). They usually regain their normal color, eventually.
DISCUSSION
Alopecia areata is an autoimmune phenomenon implying an increased tendency to develop other autoimmune diseases (eg, vitiligo [choice “a”], which can appear initially in the scalp).
This case turned out to be simple but had the potential to be far more serious. The biopsy of the dark patch showed benign seborrheic keratosis, but it was possible that another section could have demonstrated features of melanoma (choice “c”). When present, melanoma can occasionally trigger an immune response that destroys pigment cells in hair follicles, causing the hairs to lose their pigment. This is why the entire dark patch was later excised. Fortunately, the pathology report ruled out melanoma.
While it has been reported that stress can cause hair to turn gray (choice “d”), there were better (and more accurate) explanations for this patient’s presentation.
This case, though fairly straightforward, serves as a reminder that it is our job as clinicians to connect the dots to rule out worst-case scenarios.
Outcome
This patient’s hair all grew back, regaining its normal color, without any treatment.
ANSWER
The correct answer is that new hairs growing in to replace those lost from alopecia areata tend to be white (choice “b”). They usually regain their normal color, eventually.
DISCUSSION
Alopecia areata is an autoimmune phenomenon implying an increased tendency to develop other autoimmune diseases (eg, vitiligo [choice “a”], which can appear initially in the scalp).
This case turned out to be simple but had the potential to be far more serious. The biopsy of the dark patch showed benign seborrheic keratosis, but it was possible that another section could have demonstrated features of melanoma (choice “c”). When present, melanoma can occasionally trigger an immune response that destroys pigment cells in hair follicles, causing the hairs to lose their pigment. This is why the entire dark patch was later excised. Fortunately, the pathology report ruled out melanoma.
While it has been reported that stress can cause hair to turn gray (choice “d”), there were better (and more accurate) explanations for this patient’s presentation.
This case, though fairly straightforward, serves as a reminder that it is our job as clinicians to connect the dots to rule out worst-case scenarios.
Outcome
This patient’s hair all grew back, regaining its normal color, without any treatment.
About 2 months ago, a 55-year-old man suddenly experienced complete hair loss in 1 confined area of his scalp. There were no accompanying symptoms. Some of the hair subsequently grew back, but it was partially gray—a phenomenon that greatly disturbed the patient.
In general, the patient’s health was quite good, although he reported that the initial hair loss occurred about 1 month after he lost his job and got divorced.
Most of the hair was missing from a roughly round, 5-cm, ill-defined area of the left parietal scalp. The few hairs left were gray. More disturbing, though, was a dark (brown, tan, and black), oddly shaped, 2.8-cm patch in the center of the alopecic area.
Punch biopsy from the bald area showed clear evidence of alopecia areata (T-cells surrounding hair follicles, and lack of features that would support other items in the differential). Shave biopsy of the dark patch showed seborrheic keratosis, with no atypia.
He Needs More Than Lip Service for This Lesion
ANSWER
The correct answer is all of the above (choice “g”).
DISCUSSION
Squamous cell carcinoma (SCC) of the lip—almost always the lower lip—is quite common and appears to be caused by several factors. These can include exposure to ultraviolet light, ionizing radiation, arsenic (through contaminated groundwater or certain medications), tobacco, and human papillomavirus.
Early on in its manifestation, this patient’s SCC could have been excised with margins, producing an excellent prognosis. But with the extended delay in diagnosis and apparent related adenopathy, the patient’s future looked much less certain. At the very least, he would face extensive surgery, possible lymph node dissection, and maybe chemo and radiation therapies. Metastasis to the brain and lung(s) were very real possibilities.
The history associated with this case is far from uncommon, since these cancers are often mistaken for infection, which further delays correct diagnosis and treatment. Compounding this problem—and for reasons unclear to this author—affected patients are often referred to the wrong specialist. The critical missing piece of information was a diagnosis, which could only have been obtained through an incisional biopsy. Only then could the patient be referred appropriately.
Fortunately, in this case, the patient ended up under the care of a head and neck surgeon who planned to address this tumor after imaging of the head, neck, and lungs was completed.
ANSWER
The correct answer is all of the above (choice “g”).
DISCUSSION
Squamous cell carcinoma (SCC) of the lip—almost always the lower lip—is quite common and appears to be caused by several factors. These can include exposure to ultraviolet light, ionizing radiation, arsenic (through contaminated groundwater or certain medications), tobacco, and human papillomavirus.
Early on in its manifestation, this patient’s SCC could have been excised with margins, producing an excellent prognosis. But with the extended delay in diagnosis and apparent related adenopathy, the patient’s future looked much less certain. At the very least, he would face extensive surgery, possible lymph node dissection, and maybe chemo and radiation therapies. Metastasis to the brain and lung(s) were very real possibilities.
The history associated with this case is far from uncommon, since these cancers are often mistaken for infection, which further delays correct diagnosis and treatment. Compounding this problem—and for reasons unclear to this author—affected patients are often referred to the wrong specialist. The critical missing piece of information was a diagnosis, which could only have been obtained through an incisional biopsy. Only then could the patient be referred appropriately.
Fortunately, in this case, the patient ended up under the care of a head and neck surgeon who planned to address this tumor after imaging of the head, neck, and lungs was completed.
ANSWER
The correct answer is all of the above (choice “g”).
DISCUSSION
Squamous cell carcinoma (SCC) of the lip—almost always the lower lip—is quite common and appears to be caused by several factors. These can include exposure to ultraviolet light, ionizing radiation, arsenic (through contaminated groundwater or certain medications), tobacco, and human papillomavirus.
Early on in its manifestation, this patient’s SCC could have been excised with margins, producing an excellent prognosis. But with the extended delay in diagnosis and apparent related adenopathy, the patient’s future looked much less certain. At the very least, he would face extensive surgery, possible lymph node dissection, and maybe chemo and radiation therapies. Metastasis to the brain and lung(s) were very real possibilities.
The history associated with this case is far from uncommon, since these cancers are often mistaken for infection, which further delays correct diagnosis and treatment. Compounding this problem—and for reasons unclear to this author—affected patients are often referred to the wrong specialist. The critical missing piece of information was a diagnosis, which could only have been obtained through an incisional biopsy. Only then could the patient be referred appropriately.
Fortunately, in this case, the patient ended up under the care of a head and neck surgeon who planned to address this tumor after imaging of the head, neck, and lungs was completed.
Several years ago, the patient developed what he thought was a cold sore, which waxed and waned but never quite healed. It grew considerably over time but caused no pain, so the patient and his wife assumed it was benign. A few times over the years, his primary care provider (PCP) prescribed oral antibiotics, then later acyclovir, to treat the lesion. When it became large enough to interfere with normal speech, the patient’s PCP referred him to a dentist, who in turn arranged an urgent consultation with dermatology.
The patient was retired but had spent more than 50 years working and recreating outdoors without any protection. For 40 of those adult years, he had smoked. His health was good in other respects.
The 3-cm warty mass on the vermillion surface of the lower lip, just to the left of center, was focally eroded and quite firm to touch. Palpation of local nodal locations revealed several fixed nontender nodes, most notably in the submental area. His sun-exposed skin was quite sun damaged, with stellate scarring, abundant dyschromia, telangiectasias, and marked solar atrophy.
A 5-mm punch biopsy was performed, showing poorly differentiated squamous cell carcinoma.
Why Size (of Your Differential) Matters
ANSWER
The correct answer is all of the above (choice “f”).
DISCUSSION
The most likely diagnostic explanation for this patient’s presentation is erythema annulare centrifugum (EAC; choice “c”). However, this diagnosis is often difficult to establish, in part because of the broad differential and also because the very existence of EAC is far from well established.
The overwhelming consensus is that EAC represents a hypersensitivity reaction to an unknown antigen. It can be triggered by a wide variety of micro-organisms, stress, or even pregnancy.
In this case, there was no clinical or historical reason to suspect an underlying cancer, Lyme disease, or lupus, nor did the biopsy results suggest any of these.
The key takeaway here is to urge providers to avoid jumping onto a diagnostic bandwagon before considering a wider differential. Indeed, size matters when it relates to the length of one’s differential diagnosis list. If you don’t consider it, you can’t diagnose it.
TREATMENT
Fortunately, EAC nearly always resolves, with or without treatment. This patient received reassurance as such but was scheduled to return for a check of his lesions in 2 months. At that point, they had resolved.
ANSWER
The correct answer is all of the above (choice “f”).
DISCUSSION
The most likely diagnostic explanation for this patient’s presentation is erythema annulare centrifugum (EAC; choice “c”). However, this diagnosis is often difficult to establish, in part because of the broad differential and also because the very existence of EAC is far from well established.
The overwhelming consensus is that EAC represents a hypersensitivity reaction to an unknown antigen. It can be triggered by a wide variety of micro-organisms, stress, or even pregnancy.
In this case, there was no clinical or historical reason to suspect an underlying cancer, Lyme disease, or lupus, nor did the biopsy results suggest any of these.
The key takeaway here is to urge providers to avoid jumping onto a diagnostic bandwagon before considering a wider differential. Indeed, size matters when it relates to the length of one’s differential diagnosis list. If you don’t consider it, you can’t diagnose it.
TREATMENT
Fortunately, EAC nearly always resolves, with or without treatment. This patient received reassurance as such but was scheduled to return for a check of his lesions in 2 months. At that point, they had resolved.
ANSWER
The correct answer is all of the above (choice “f”).
DISCUSSION
The most likely diagnostic explanation for this patient’s presentation is erythema annulare centrifugum (EAC; choice “c”). However, this diagnosis is often difficult to establish, in part because of the broad differential and also because the very existence of EAC is far from well established.
The overwhelming consensus is that EAC represents a hypersensitivity reaction to an unknown antigen. It can be triggered by a wide variety of micro-organisms, stress, or even pregnancy.
In this case, there was no clinical or historical reason to suspect an underlying cancer, Lyme disease, or lupus, nor did the biopsy results suggest any of these.
The key takeaway here is to urge providers to avoid jumping onto a diagnostic bandwagon before considering a wider differential. Indeed, size matters when it relates to the length of one’s differential diagnosis list. If you don’t consider it, you can’t diagnose it.
TREATMENT
Fortunately, EAC nearly always resolves, with or without treatment. This patient received reassurance as such but was scheduled to return for a check of his lesions in 2 months. At that point, they had resolved.
A 50-year-old man is astonished when his “fungal infection” fails to respond to an unknown OTC topical cream a friend advised him to use.
For several weeks, he’s been plagued by the slightly itchy lesions that appeared on his leg without known cause. His friend assured him that the shape and configuration of the lesions could only represent one thing: “ringworm,” that is, fungal infection.
However, in clinic, the patient denies any contact with animals or children and reports that his job is inside only, never involving the great outdoors. He has felt fine throughout the lesions’ tenure, going so far as to say he is “in perfect health.” He has been in a mutually monogamous relationship for many years.
The lesions (4 in total) are located on the medial aspect of the left leg, extending into the popliteal area. At first glance, they appear to be peripherally scaly, pink, and annular. Close inspection reveals that most of the scaling is not on the outer edge; it is confined to the inside of the border, a phenomenon termed trailing or centripetal scaling. The lesions all show an arciform morphology in the shape of a “C.”
KOH examination of the scale shows no fungal elements. Shave biopsy reveals a dense perivascular lymphocytic infiltrate, moderate parakeratosis, and perhaps most importantly, no fungal elements in the stratum corneum.
There are no palpable lymph nodes in the groin on the affected side.
All’s Well That Ends Swell(ing)
ANSWER
The correct answer is elephantiasis nostras verrucosa (ENV; choice “d”).
DISCUSSION
ENV is a rare condition of advanced cutaneous hypertrophy secondary to a combination of contributing factors including: a sedentary lifestyle, obesity, chronic venous stasis, repeated bouts of lymphangitis, cellulitis, and congestive heart failure (CHF). Most commonly affecting the lower extremities, it is occasionally seen in other dependent areas such as the scrotum and the abdominal pannus. It is, essentially, an exaggerated form of cutaneous lymphedema that causes the skin to become increasingly thick and fibrotic, changes which also reduce blood flow to or from the area.
Despite its name, ENV is not associated with elephantiasis, more commonly known as lymphatic filariasis (choice “b”). Although that condition manifests with similar skin changes, it is typically seen only in those who live in tropical areas where these organisms are endemic—places this patient has never visited.
There was no reason to believe that these skin changes were attributable to warts (choice “a”). Biopsy would have settled that question but also would have run the risk of creating a nonhealing wound, which could easily turn into an ulcer.
Lymphedema (choice “c”) was clearly present, but it was quite advanced—far beyond what is usually seen in venous insufficiency. This diagnosis would not, by itself, explain the nodules or extreme fibrosis.
Other potential causes for these skin changes include postradiation and pretibial myxedema, which had been ruled out prior to the dermatology consult.
TREATMENT
As with simple venous insufficiency, treatment of ENV consists of compression, elevation, and weight loss. For this patient, the diuretics prescribed as part of her CHF treatment might help a bit, but her prognosis is guarded at best.
ANSWER
The correct answer is elephantiasis nostras verrucosa (ENV; choice “d”).
DISCUSSION
ENV is a rare condition of advanced cutaneous hypertrophy secondary to a combination of contributing factors including: a sedentary lifestyle, obesity, chronic venous stasis, repeated bouts of lymphangitis, cellulitis, and congestive heart failure (CHF). Most commonly affecting the lower extremities, it is occasionally seen in other dependent areas such as the scrotum and the abdominal pannus. It is, essentially, an exaggerated form of cutaneous lymphedema that causes the skin to become increasingly thick and fibrotic, changes which also reduce blood flow to or from the area.
Despite its name, ENV is not associated with elephantiasis, more commonly known as lymphatic filariasis (choice “b”). Although that condition manifests with similar skin changes, it is typically seen only in those who live in tropical areas where these organisms are endemic—places this patient has never visited.
There was no reason to believe that these skin changes were attributable to warts (choice “a”). Biopsy would have settled that question but also would have run the risk of creating a nonhealing wound, which could easily turn into an ulcer.
Lymphedema (choice “c”) was clearly present, but it was quite advanced—far beyond what is usually seen in venous insufficiency. This diagnosis would not, by itself, explain the nodules or extreme fibrosis.
Other potential causes for these skin changes include postradiation and pretibial myxedema, which had been ruled out prior to the dermatology consult.
TREATMENT
As with simple venous insufficiency, treatment of ENV consists of compression, elevation, and weight loss. For this patient, the diuretics prescribed as part of her CHF treatment might help a bit, but her prognosis is guarded at best.
ANSWER
The correct answer is elephantiasis nostras verrucosa (ENV; choice “d”).
DISCUSSION
ENV is a rare condition of advanced cutaneous hypertrophy secondary to a combination of contributing factors including: a sedentary lifestyle, obesity, chronic venous stasis, repeated bouts of lymphangitis, cellulitis, and congestive heart failure (CHF). Most commonly affecting the lower extremities, it is occasionally seen in other dependent areas such as the scrotum and the abdominal pannus. It is, essentially, an exaggerated form of cutaneous lymphedema that causes the skin to become increasingly thick and fibrotic, changes which also reduce blood flow to or from the area.
Despite its name, ENV is not associated with elephantiasis, more commonly known as lymphatic filariasis (choice “b”). Although that condition manifests with similar skin changes, it is typically seen only in those who live in tropical areas where these organisms are endemic—places this patient has never visited.
There was no reason to believe that these skin changes were attributable to warts (choice “a”). Biopsy would have settled that question but also would have run the risk of creating a nonhealing wound, which could easily turn into an ulcer.
Lymphedema (choice “c”) was clearly present, but it was quite advanced—far beyond what is usually seen in venous insufficiency. This diagnosis would not, by itself, explain the nodules or extreme fibrosis.
Other potential causes for these skin changes include postradiation and pretibial myxedema, which had been ruled out prior to the dermatology consult.
TREATMENT
As with simple venous insufficiency, treatment of ENV consists of compression, elevation, and weight loss. For this patient, the diuretics prescribed as part of her CHF treatment might help a bit, but her prognosis is guarded at best.
A 61-year-old black woman presents for worrisome skin changes on her lower extremities. She reports that the condition is generally uncomfortable, but during occasional flares, it causes serious pain. She’s been affected for “many years” without diagnosis or resolution. It was her new primary care provider who, after seeing the lesions, sent her to dermatology.
The patient’s medical history includes diabetes, congestive heart failure, and obesity. All are being managed reasonably well.
Examination, performed while she is in a recumbent position, reveals legs swollen out of proportion to the rest of her body. Little or no erythema is noted. Both legs are affected equally, but only from just below the knees down to and including the feet. These areas, including her feet, are quite swollen, though no pitting edema can be provoked. The skin is quite firm and studded with multiple discrete and confluent 1-2 cm firm nodules. The skin around her ankles feels almost “woody” to the touch. There is no tenderness or increased warmth on palpation, nor is any drainage noted. (She also has a dystrophic great toenail that was partially avulsed by recent trauma.)
Making the Rounds to Diagnosis
ANSWER
The correct answer is granuloma annulare (GA; choice “d”).
DISCUSSION
The biopsy showed rings of dermal epithelioid histiocytes surrounding a core of central mucin, configured in rows (the latter conferring the diagnostic term “palisaded” granulomas). Both the pathology results and the morphology of the lesions—color, shape, etc—were classic for GA. What was somewhat unusual about this case was the size and number of lesions, which are typically fewer and smaller with GA.
GA is seldom much of a problem and has no connection to serious disease. But it can, as this case illustrates, mimic some rather worrisome conditions. Had this been sarcoidosis (choice “a”), there would be no central “delling” (concavity), and the biopsy would have shown necrotic (caseating) nonpalisading granulomas.
Cutaneous T-cell lymphoma (CTCL; choice “b”) can manifest with plaques, but there wouldn’t be any delling and the shapes would not be so consistently round. CTCL will eventually give rise to palpable adenopathy.
Lupus profundus (choice “c”) also manifests with deep plaques, without delling. It is a truly rare variant of lupus, which would have been detected on biopsy.
Concern about these differential items is what drove the decision to perform full-thickness punch biopsies as opposed to taking a simple shave sample.
TREATMENT
The most common treatment for GA is intralesional steroid injection (eg, 10 mg/cc triamcinolone) of large lesions. Oral medications such as methotrexate or pentoxifylline have been used with some success. Often, the condition is self-limiting.
ANSWER
The correct answer is granuloma annulare (GA; choice “d”).
DISCUSSION
The biopsy showed rings of dermal epithelioid histiocytes surrounding a core of central mucin, configured in rows (the latter conferring the diagnostic term “palisaded” granulomas). Both the pathology results and the morphology of the lesions—color, shape, etc—were classic for GA. What was somewhat unusual about this case was the size and number of lesions, which are typically fewer and smaller with GA.
GA is seldom much of a problem and has no connection to serious disease. But it can, as this case illustrates, mimic some rather worrisome conditions. Had this been sarcoidosis (choice “a”), there would be no central “delling” (concavity), and the biopsy would have shown necrotic (caseating) nonpalisading granulomas.
Cutaneous T-cell lymphoma (CTCL; choice “b”) can manifest with plaques, but there wouldn’t be any delling and the shapes would not be so consistently round. CTCL will eventually give rise to palpable adenopathy.
Lupus profundus (choice “c”) also manifests with deep plaques, without delling. It is a truly rare variant of lupus, which would have been detected on biopsy.
Concern about these differential items is what drove the decision to perform full-thickness punch biopsies as opposed to taking a simple shave sample.
TREATMENT
The most common treatment for GA is intralesional steroid injection (eg, 10 mg/cc triamcinolone) of large lesions. Oral medications such as methotrexate or pentoxifylline have been used with some success. Often, the condition is self-limiting.
ANSWER
The correct answer is granuloma annulare (GA; choice “d”).
DISCUSSION
The biopsy showed rings of dermal epithelioid histiocytes surrounding a core of central mucin, configured in rows (the latter conferring the diagnostic term “palisaded” granulomas). Both the pathology results and the morphology of the lesions—color, shape, etc—were classic for GA. What was somewhat unusual about this case was the size and number of lesions, which are typically fewer and smaller with GA.
GA is seldom much of a problem and has no connection to serious disease. But it can, as this case illustrates, mimic some rather worrisome conditions. Had this been sarcoidosis (choice “a”), there would be no central “delling” (concavity), and the biopsy would have shown necrotic (caseating) nonpalisading granulomas.
Cutaneous T-cell lymphoma (CTCL; choice “b”) can manifest with plaques, but there wouldn’t be any delling and the shapes would not be so consistently round. CTCL will eventually give rise to palpable adenopathy.
Lupus profundus (choice “c”) also manifests with deep plaques, without delling. It is a truly rare variant of lupus, which would have been detected on biopsy.
Concern about these differential items is what drove the decision to perform full-thickness punch biopsies as opposed to taking a simple shave sample.
TREATMENT
The most common treatment for GA is intralesional steroid injection (eg, 10 mg/cc triamcinolone) of large lesions. Oral medications such as methotrexate or pentoxifylline have been used with some success. Often, the condition is self-limiting.
A 60-year-old woman is feeling fine—no fever, arthralgia, or malaise—but the sudden appearance of lesions on her extremities unsettles her to the point that she can think of little else. Visits to her primary care provider, the emergency department, several urgent care clinics, and a naturopath yield a consistent diagnosis: ringworm. Yet none of the topical or oral antifungal medications she is prescribed make the slightest difference. Thus, she finally agrees to consult a dermatologist.
The patient reports decent health, although she was a heavy smoker for years before quitting 2 years ago. She recently tested negative for diabetes.
History taking reveals no sources from which she could have acquired a fungal infection. No one else at home is similarly affected.
About 10 lesions, mostly located on the patient’s extremities, are examined. All are very similar in appearance: round, brownish-red intradermal plaques with no epidermal component (eg, scale, broken skin). The lesions vary from 6 mm to 4 cm in diameter. The centers of most lesions are slightly concave, with well-defined raised margins. On palpation, there is neither increased warmth nor tenderness. No nodes can be palpated in the groin, axillae, or epitrochlear locations.
A deep punch biopsy is performed on a thigh lesion, with the specimen submitted for pathologic examination.
A Rash Against the Grain
ANSWER
The correct answer is dermatitis herpetiformis (DH; choice “b”).
DISCUSSION
DH is unusual, although not rare, and it is often overlooked in the investigation of itchy rashes—even by dermatologists. As is so often the case, the biopsy was the missing piece of the puzzle, since it clearly showed changes consistent with this condition.
The history of DH has some interesting overlap with that of biopsy. It was Rudolph Virchow, an 18th century Austrian pathologist, who first connected clinical disease to specific arrangements of disordered tissue seen microscopically—at that time, a giant leap for medicine. An American dermatologist, Louis Adolphus Duhring, had the good fortune to study under Virchow and his successors in Vienna and brought this cutting-edge knowledge back to this country, where he began the process of categorizing diseases by their histologic patterns as well as by their presentation and clinical course. This was when he discovered the basis for the condition that became known as Duhring disease, also known as dermatitis herpetiformis.
It was much later (1967) that a connection was made between DH and the ingestion of gluten. Our patient experienced a rapid decline in his itching and rash as soon as he started a gluten-free diet. However, since many patients find such a diet difficult to maintain, there are pharmacologic options as well: dapsone, colchicine, sulfa drugs, drugs from the tetracycline family, and nicotinamide. All have potential adverse effects with which the prescriber needs to become familiar.
With a combination of medication and reduced gluten intake, it’s entirely possible that the patient’s DH will permanently resolve, although he will remain at increased risk for other autoimmune conditions.
ANSWER
The correct answer is dermatitis herpetiformis (DH; choice “b”).
DISCUSSION
DH is unusual, although not rare, and it is often overlooked in the investigation of itchy rashes—even by dermatologists. As is so often the case, the biopsy was the missing piece of the puzzle, since it clearly showed changes consistent with this condition.
The history of DH has some interesting overlap with that of biopsy. It was Rudolph Virchow, an 18th century Austrian pathologist, who first connected clinical disease to specific arrangements of disordered tissue seen microscopically—at that time, a giant leap for medicine. An American dermatologist, Louis Adolphus Duhring, had the good fortune to study under Virchow and his successors in Vienna and brought this cutting-edge knowledge back to this country, where he began the process of categorizing diseases by their histologic patterns as well as by their presentation and clinical course. This was when he discovered the basis for the condition that became known as Duhring disease, also known as dermatitis herpetiformis.
It was much later (1967) that a connection was made between DH and the ingestion of gluten. Our patient experienced a rapid decline in his itching and rash as soon as he started a gluten-free diet. However, since many patients find such a diet difficult to maintain, there are pharmacologic options as well: dapsone, colchicine, sulfa drugs, drugs from the tetracycline family, and nicotinamide. All have potential adverse effects with which the prescriber needs to become familiar.
With a combination of medication and reduced gluten intake, it’s entirely possible that the patient’s DH will permanently resolve, although he will remain at increased risk for other autoimmune conditions.
ANSWER
The correct answer is dermatitis herpetiformis (DH; choice “b”).
DISCUSSION
DH is unusual, although not rare, and it is often overlooked in the investigation of itchy rashes—even by dermatologists. As is so often the case, the biopsy was the missing piece of the puzzle, since it clearly showed changes consistent with this condition.
The history of DH has some interesting overlap with that of biopsy. It was Rudolph Virchow, an 18th century Austrian pathologist, who first connected clinical disease to specific arrangements of disordered tissue seen microscopically—at that time, a giant leap for medicine. An American dermatologist, Louis Adolphus Duhring, had the good fortune to study under Virchow and his successors in Vienna and brought this cutting-edge knowledge back to this country, where he began the process of categorizing diseases by their histologic patterns as well as by their presentation and clinical course. This was when he discovered the basis for the condition that became known as Duhring disease, also known as dermatitis herpetiformis.
It was much later (1967) that a connection was made between DH and the ingestion of gluten. Our patient experienced a rapid decline in his itching and rash as soon as he started a gluten-free diet. However, since many patients find such a diet difficult to maintain, there are pharmacologic options as well: dapsone, colchicine, sulfa drugs, drugs from the tetracycline family, and nicotinamide. All have potential adverse effects with which the prescriber needs to become familiar.
With a combination of medication and reduced gluten intake, it’s entirely possible that the patient’s DH will permanently resolve, although he will remain at increased risk for other autoimmune conditions.
A 56-year-old man began to itch almost 10 years ago, when lesions appeared on his extensor forearms; they later branched out to his knees, scalp, and waistline. He has continued to suffer despite seeing numerous providers, including dermatologists.
An allergist pronounced him free of any significant allergies. Another provider was certain the patient had scabies, although his household was unaffected and the prescribed treatment—permethrin lotion and oral ivermectin—had no impact on the rash or the symptoms. Most of the other consulted providers diagnosed eczema, despite a complete lack of atopy in the patient or his family of origin. Furthermore, treatment with topical, oral, and intramuscular steroids had offered minimal and very short-lived relief. No one took a scraping or performed a biopsy of the rash.
The patient claims to be otherwise healthy, with no gastrointestinal symptoms and no new medications. Examination reveals a slightly overweight man in no distress.
The lesions in question are patches of excoriated papulovesicular lesions. Microscopic examination of a KOH prep shows no scabetic elements. His volar wrists, the sides of his fingers, and his genitals are free of lesions.
A shave biopsy is performed under local anesthetic. The pathology report indicates subepidermal collections of eosinophils.
