Dermatomyositis

Without beating to death the difference between polymyositis and dermatomyositis, suffice it to say polymyositis is the disease without skin manifestations, whereas DM does have skin changes.

The skin changes associated with DM are as follows:

1. Gottron's Papules (see above).  Violaceous papulosquamous eruptions over the MCPJs and IPJs.  

2. Heliotrope rash.  The classic violaceous rash on the eyelids is textbook, but it may be subtle at times.

3. Shawl Sign.  Diffuse, flat erythematous rash over back, neck, upper chest (often in a V shape)

4. Facial erythema

5. Periungual telangiectases

6. Flagellate truncal erythema

Not skin, but still part of the diagnostic picture:

7. Proximal muscle weakness (ask them to stand up out of a chair without using their hands)

8. Muscle tenderness, particularly the proximal thighs.

Just like with Beau's lines, the best place to find DM periungual telangiectases is the fourth finger.  

Although this is a discussion about adult DM, there is a juvenile variant which also has cutaneous calcifications.

Systemic Lupus Erythematosus

SLE is a pretty strange disease.  It manifests itself as a photosensitivity in many cases, and can be thought of as an allergy to the sun with protean medical manifestations.  When one looks at the patients (mostly women) who have the butterfly rash, what we look for is such subtle signs as whether or not the nasolabial fold is spared, the submentum is less involved than the chin, and, if there is a hand rash, whether or not the rash spares the knuckles.  If it does, slide all your chips onto the "SLE" spot on the table, and expect to cash in.  

In contradistinction to dermatomyositis, (see the next post) even though there is periungual erythema and perhaps telangiectasia, the sparing of the MCPJs and the PIPJs is classic SLE, whereas with DM one would expect it to involve the knuckles, the so-called Gottron's papules.

Why couldn't this be SCLE??   Well, it could, but SLE is a far better answer given the photo, because there really isn't a papulosquamous look to this at all, but more just that inflammatory look.  Remember, when faced with a choice, pull the trigger on the MOST likely of the dxes.

One last point to make.  Obviously, this is a systemic disease (or they would  have named it something else, wouldn't they?) but one of the main systems it affects that most people forget is the brain.  When their antibody titers are cranked up and they are really sick, they get a cerebritis which can make them loopy, unpleasant or, in rare cases, quasi-psychotic.  So, forgive them if they are flaring and are cranky with you.  They probably don't mean what they say.

Your job, above all else, is to get them to stay out of the sun.  Yell at them if they disobey you.  Hurt their feelings if necessary, because with each flare they get, they can further damage their kidneys, joints, etc. and those don't recover from the damage.

Neurofibromatosis Type 1

This very unfortunate woman has Von Recklinghausen's Disease, aka NF-1.  Despite the obvious neurofibromas (which buttonhole, as you remember) we also see axillary freckling/pigmentation, which we know as Crowe's Sign.  We should also examine the eyes, and would expect to see Lisch Nodules;  we would also expect to see cafe au lait patches (6 or more).  We could image them, looking for optic gliomas and would also expect a family member to have the disease (as it is inherited as an autosomal dominant disease), as well, although 50% of cases are spontaneous, i.e. not inherited.  

Chronic Paronychia

Chronic paronychia is a condition that results in nail dystrophy that usually extends across the entire breadth of the involved nail.  It is caused when there is disruption of the proximal nail fold/ cuticle resulting in a space under the free edge of the nail fold, which creates a chronic wet area which is an ideal environment for yeast, often of the candidal persuasion. 

The way to distinguish this from, say, Beau’s lines is to look at the proximal nail fold/ cuticle unit to see if it is intact.  Next, look at the character of the transverse grooves.  If they are irregular and kind of ragged looking, it is usually a result of chronic paronychia.  If, on the other hand, it is a smooth, even curvilinear line across the nail plate, it is usually a Beau’s line.  Remember:  Beau’s lines are a result of a physiologic stress of one sort or another; it is a reflection of a systemic issue, not a local one.  It is the same process (slowing of the growth of the matrix) which in the hair also leads to telogen effluvium.  In fact, if I am trying to work someone up for telogen effluvium, the first thing I do is look at their nails. The best place to find Beau’s lines is the fourth (ring) fingernail for reasons that are completely unclear to me, but chronic paronychia can be on any nail. 

The reason I put this picture in is because chronic paronychia was discussed in the blog a couple of days ago under habit tic deformity.  NOBODY should have missed this question!!

Metastatic Melanoma to the Scalp

When looking at lumps and bumps on the skin, the first algorithm we go through is “benign or malignant?”  There are usually multiple clues.  First, history. Of course, I didn’t give you the history on this one, because it would give away the answer (he was being treated for metastatic melanoma). Second, the character of the lesion.  As we all know, cancers grow as fast as the body will let them, but they are all marked by a relatively high metabolic rate compared to the surrounding tissue (even the lowly basal cell carcinoma induces blood vessel formation, just so it can be fed).  High metabolism demands a good blood supply, so most cancers, if they are going to be successful, will be surrounded in some way with an increased blood supply.  Third, what is the context, exam-wise?  We see here numerous other nodules on the scalp, which would make us think “Hmmm.  Multiple vascular lesions on the scalp.  Unlikely to be a primary cancer.”

So, run the algorithm.  Benign versus malignant.  Primary versus metastatic.  And the metastases seem to like skin.  Although many metastases like the skin, the ones that stick out in my head are clear cell carcinoma of the kidney and melanoma.  So, since there is no pigment in the lesions, I would have guessed metastatic clear cell ca, but nevertheless a metastatic process.  I would biopsy with a scoop shave, and let the pathologist sort it out with immunohistochemical studies.

Why metastasize to the scalp?  Well, the scalp, much like the lungs, and the liver, sees a lot of blood go through it, and it has multiple small vessels in it.  If a metastasis is floating around in the bloodstream, having arrived (usually) through the lymphatics, getting dumped into the subclavians via the thoracic duct and into the general circulation.  They then float around until they get wedged into a small vessel, where, if they have enough nutrition, they set up shop.  They secrete cytokines which cause local proliferation of blood vessels, which allows the metastases to grow.  In some cases, the growth is explosive, and in others, it is not. 

Many patients are a little reticent to have their picture taken, especially when they are faced with serious consequences of disease or with a disfiguring condition.  This gentleman wanted  me to take his picture, so other people would be warned about melanoma and hopefully avoid the pain he endured.  He passed away roughly a week after this picture was taken, from brain and lung metastases.  

  

Habit tic deformity

The habit tic deformity is a self-induced condition that is caused by damage to the external part of the nail matrix, usually by pressing or scraping on the lunula of the nail with another fingernail.  Since the matrix is softer than the fully-formed nail, it is more easily damaged than the distal nail.  The way to tell it is a habit tic deformity is to see if the dystrophic changes extend all the way across the nail, as they would with chronic paronychia, or if they are longitudinal and well defined, like it would be with a median canaliform dystrophy.  Also, with chronic paronychia, one would see interruption or complete disruption of the proximal nail fold.  

Median canaliform dystrophy

Use this as a moment to reflect on the way the nail is made.  The lunula, which is largely obscured by the proximal nail fold, is the matrix.  The most proximal of the lunula/matrix ends up being the outer part of the nail plate, the distal part of the lunula/matrix is the deep part of the nail plate.  So, since this dude was primarily picking at the distal part, the changes of the dystrophy actually go all the way through the nail.  To compare it to the rainbow below, the red part is originated at the proximal matrix, the violet part is formed at the distal part of the matrix.

We are coming down to the final days of March Madness, and we have a two-way tie for the lead, with two others one point behind.  Who will win the Gewurztraminer?  Stay tuned...

The Best Damn Band in the Land

The Ohio State University is not my alma mater (Miami of Ohio is), and I never played a band instrument in my life, but growing up in Columbus, listening to the Ohio State Marching Band play Fight on Down the Field, or watching them do "Script Ohio" are two of the goofy pleasures of my life.  Growing up in Columbus and being an OSU fan is much like growing up in Dublin, Ireland.  Almost everyone in Dublin is Roman Catholic, and almost everyone in Columbus can give you a blow-by-blow account of the last OSU-Michigan game.  It's religion, sort of.  

When my son, who did play saxophone in the University of Florida's marching band, went with the Gators to the BCS National Championship game versus OSU, his comment was this:  "We beat them in football" (I don't want to talk about that)"but, they were the best marching band I ever saw." Well said, Danny Boy.  Well said.

Drug Induced SCLE

Subacute cutaneous Lupus Erythematosus is a chronic, nonscarring photosensitivity disease which can occur in drug-induced or non drug-induced variants.  Females predominate, in a ratio of roughly 4:1, and some of these patients will go on to develop some of the signs of systemic lupus erythematosus.  The clues for this patient were largely those of testmanship:  it is in a photodistributed area, annular and I gave you the clue that it was caused by a medication. In my residency, we all got sick and tired of hearing "use all available clues", but in reality, that's what you should do for everything.  

The meds most commonly associated with it are antihypertensives (particularly HCTZ) and antifungals, with terbenafine being the chief culprit.  

Just as an aside, if you see a photodistributed rash, always biopsy it.  It'll make your life so much easier, because 95% of the time you can cut to the chase with the diagnosis, especially if you combine it with serology and clinical examination.  Serology in both "wild type" SCLE and drug induced often are Ro+, often ANA positive as well.  

The ddx is pretty broad, if you just consider the papulosquamous nature of the rash, but if you do a biopsy, if you consider the distribution of the rash, and if you consider the serology you can nail it the vast majority of the time.  For instance, some SCLE looks psoriasiform, but is photodistributed.  That is unlike most psoriasis, right?  Same with CTCL. It is unusual to get true papulosquamous CTCL in a photo distribution.  GA, which is rarely photodistributed, is almost never papulosquamous, true?  And acute LE patients are usually sick, which is unusual with SCLE. 

For those of you who don't have access to Jerry Litt's manual, the Drug Eruption Reference Manual, please ask your docs to order one.  It is immensely helpful in deciding which drugs cause which kind of eruption.  If you've been to the AAD, Jerry will sign them, give you a copy of his poem he wrote for JAMA, I think called "A Hunk of Skin" which was, he told me, the second poem they ever printed, way back in the sixties.  He is a spry old guy, and a lot of fun.  For those of you who have had the pleasure of meeting Jerry, here's a little blast from the past:  

http://www.youtube.com/watch?v=Xhw50rT0pWM .

Porokeratosis of Mibelli

Porokeratosis of Mibelli is a distinctive quasi-benign neoplasm of the skin that is characterized by a raised ridge that surrounds the abnormal keratinocytic process. It comes in several variants, including palmoplantar keratoderma, Disseminated Superficial Actinic Porokeratosis, Porokeratosis of Mibelli, linear porokeratosis, and punctate porokeratosis.  This case is Porokeratosis of Mibelli (PM), the largest of the porokeratoses.  Occasionally, they can transform into SCC or BCC.

Clinically, PM is distinguished by its size, which is usually > 1 cm, its solitary nature and its oval shape.  I've seen them over 5 cm wide, but those are really rare. Often, if examined closely, it has the dual wall (like the Great Wall of China) somewhere around the cornoid lamella.  In this case, the dual wall is most prominent around the nine o'clock border. 

I gave credit if you called this porokeratosis, PM or DSAP.  

Cheilitis Glandularis

Cheilitis glandularis is a crazy, rare disease characterized by an enlarged lower lip which is everted, and usually has pronounced openings of the minor salivary glands.  The lip is usually glazed with a thick mucoid discharge, and upon squeezing of the lower lip, prominent mucoid discharge can be seen from the patulous gland openings.  If you touch your (gloved) finger to the lip, you will get the "string sign" of sticky mucus, as is shown in the picture.  There is a suppurative variety which involves infection of the minor salivary glands, but it is uncommon.  The only realistic ddx for this condition is Miescher-Melkersson-Rosenthal Disease, which involves a granulomatous swelling of the lips, plicated tongue and sometimes facial palsy but does not have the thick mucus discharge.  You will find cheilitis glandularis in both genders and any age group, but middle aged men are most likely to have it.  It can be associated with SCC of the lower lip.

This was a very, very difficult case, but a classic one nevertheless.  

Erythema ab igne

Erythema ab igne is a relatively common pigmentary disorder that results from chronic heat, which can either be red (hence "erythema") or brownish red, as in the above example.  The reticulated rash is a result of the damage from heat of the superficial vessels, and the hypermelanosis is a result of pigmentary incontinence.  

Confluent and reticulated papillomatosis of Gougerot and Carteaud is more scaly and elevated than is erythema ab igne, and is pretty easy to figure out in person.  

Congrats, all of you got this one correct.  The Challenge this month is too close to call thus far.  There are five of you within a point of one another, and another five of you two points back from the leader.  There is plenty of time to have this lead change hands, but at present Christine shares the lead with Emily.

Scabies Mite

Not much to say about this little guy, except this: the way to actually get a good scabies prep is to scrape, on average, 25 lesions that look like burrows or inflamed papules.  Use a microscope slide, a 15 blade and a source of light mineral oil or microscope oil, and dip the scalpel blade in the oil, then scrape a few lesions, then wipe the blade on the slide, ensuring that you get enough debris, then repeat.  

You should be scraping deep enough to slightly abrade the papillary dermis, so you should occasionally get blood.  Pay attention to the areas like the interdigital region, wrists, areolae and also the itchiest part of the patient's body.  Those round objects floating in the oil are not scyballa or eggs, but are bubbles in the oil.  

Those oval objects above ARE scabies eggs, and are pretty easily identified as such.  

Both of these pictures were taken with my iPhone through the microscope eyepiece.

Syringoma

Syringomas are common, usually multiple benign neoplasms, which are formed from eccrine sweat ducts.  They can be generalized, but commonly are limited to the periorbital area.  They are skin colored or slightly yellow, and they are almost always asymptomatic, although I once had a patient who complained of itching and burning on perspiration.  

If they present classically, they are pretty easy to diagnose, but they can be confused with other benign adnexal tumors (BATs- which are tumors of the adnexal elements of the skin, such as hair follicle tumors, other sweat gland tumors, nerve and blood vessel tumors, etc.) as well as basal cell carcinoma and microcystic adnexal carcinoma, which is a sweat duct cancer that is highly infiltrative.  

The rule is this: if it is a solitary tumor, even if it looks like a syringoma, biopsy it.  

The difference between "us" and "them":  I showed my wife this picture and said "Hey, look!  It's syringomas!"  Her reply; 'Oh my my God, that's disgusting!"

Juvenile Xanthogranuloma

Juvenile Xanthogranuloma is a benign, usually asymptomatic condition of neonates and young children that almost always resolves spontaneously.  It occurs cutaneously most of the time, primarily on the head and neck but can also show up on the trunk and on the extremities.  Most of the time, they are solitary.  Although they are usually small, <5mm, they can be up to 2 cm and rarely even larger than that.  Notably, they can show up extracutaneously in the eyes, primarily in the irides.

The differential is pretty slim, including Spitz nevi, eruptive xanthomas (which are always multiple) and Langerhans Cell Histiocytosis.  

In differentiating a JXG from a Spitz Nevus, I would look at both color and texture.  The color of JXGs tends to be yellowish or yellow brown or yellow-red, whereas the Spitzes tend to be red (although they can be slightly orange in color, that's unusual) but the main differentiation is that the JXGs are really smooth and almost blister-like, such as the example I gave you, versus Spitz Nevi, which tend to have more of a texture similar to nevi. I would biopsy these just to be assured this wasn't a solitary lesion of LCH, but I would assure the parents it most likely represented a benign growth.  

How to Use a Scalpel and Make Dr. Halsted Proud

We all know how to hold a 15 blade scalpel, as shown above, but the 15 blade is not always used to its best advantage.  Many people still hold the handle vertically as they are inscribing their cuts, and paradoxically (perhaps to gain more control over the blade) flattening out the blade when at the tip of an ellipse.  Here's a short primer on how to handle a 15 blade:

Think of a scalpel blade as a saw.  Since there are teeth on a saw, you draw the saw across the wood so as to use the teeth to cut the surface of the wood.  If you look at a scalpel blade, there are similarly little serrations on the blade similar to the teeth of a saw.  So, rather than imagining that the super sharp blade  separates the tissue, think of the serrations as cutting through like a saw.

When inscribing either an arc or a straight line, keeping the blade perpendicular to the skin but the handle at a fairly small (< 30 degree) angle, draw your scalpel down the line you have either pre-drawn or imagined in your mind.  This allows the scalpel blade to work like a keel, keeping the line straight and preventing the pushing of tissue ahead of the blade, making a nice, clean cut.  Use the belly of the blade (which, if you are holding the scalpel correctly, you will) and draw your hand toward you if possible.

If, on the other hand, you are making an arc that is more sharply angled, or if you are approaching the tip of the ellipse, bring the handle up more vertically, closer to forming a right angle with the cutting surface.  This will make the cutting surface the shoulder of the blade, which allows for finer, more sharply angled cuts, but requires more control from the surgeon.  One uses less of a stroking motion with the blade, and usually there is more pressure applied to the blade when using it thusly.

As an additional point, when cutting ellipses on a flat surface (the pretibial region is an exception to this), the usual ration of length to width is 3:1.  If I'm operating on an engineer, I tell them it is Pi:1, because that intrigues them enough to make them stop asking questions about the current of the hyfrecator.

If operating on a concave surface, we tend to shorten the length of the incision, and if on a convex surface, we lengthen it.  We usually, but not always, work parallel to the relaxed skin tension lines 

 http://doctorsgates.blogspot.com/2010/10/skin-tension-lines-and-how-to-get-it.html and we try to, of course, evert the wound edges on closure, but that's a story for another day.

Back in the day, William Halsted was the head of surgery at Johns Hopkins, and was in many ways the father of modern surgical training, but he had an annoying habit.  If any of his "boys" held the scalpel wrong or used it incorrectly, he would rap them sharply across the knuckle with whichever instrument he was holding at the time, which as you can imagine, hurt like a son of a gun. Amazingly, he was never killed by one of his "boys", and lived to the ripe old age of seventy.  As a side note, his teaching method may have been explained by his alleged addiction to cocaine.  Apparently, it made him jumpy.

  

The J-shaped Curve

I thought it was worth revisiting the question of the J-shaped curve as it applies to alcohol intake, not so much because I thought it was a stumper, but because I wanted to ensure that we all have a common base of knowledge from which to work.  As caring providers, we want to know as much as possible about health topics, and when we ask about healthy lifestyles, we want to ensure the patients aren't ignorant about the negative effects of teetotalling or the overconsumption of alcohol.  

I think of it as cholesterol medicine (which it really isn't, but it works for the illustration).  We wouldn't advise the patients who need it NOT to take their cholesterol medicine, nor would we advise them to take too much of it, nor would we say "if you don't take your medicine daily, just take a lot of it on the weekends". The right dose (1-2 glasses of alcohol) on a daily basis is good for the patients, and that is that.

There are always confounding variables in our lives, such as smoking, exercise and other lifestyle risks, but if we factor those confounding variables out, the data regarding moderate intake of EtOH still stands alone as a positive effect on longevity, on health, on cardiovascular status and on maintenance of intellect into the later years.  

There are several proposed mechanisms for the positive effects of red wine, in particular, and some of them bear mentioning.  Although the polyphenols from red wine in vivo exert no significant antioxidant effects (Vitamin C, vitamin E, glutathione, etc. have far greater effects) they do decrease inflammation, decrease endothelial cell adhesion, increase nitric oxide production and decrease platelet aggregation.  Other sources of polyphenols such as black tea, cocoa and its derivative dark chocolate also exert similar effects.

For those who really want to hone in on red wine with the highest polyphenol content, the tannat and sacramentino grapes seem to have the highest levels, and coincidentally seem to coincide with local populations where these grapes are derived that seem to be unusually long-lived.  

Fun quotes regarding wine and health:

"Drink a glass of wine a day, and steal a ruble from the doctor"- Old Russian Proverb

"Wine is the foremost of all medicines; where there is no wine, medicines become necessary" The Talmud

"It's not the use of a bad thing, but the abuse of a very good thing" that causes harm.  Abraham Lincoln

Finally, for those wine nerds among you, I went to a lecture by Matt Kramer, The Wine Spectator's great critic and explainer, and he had some tips for finding great wine without spending a bundle:

1. Australia: Look for wines from Clare Valley; it has the best reds and whites from Oz.  Margaret River also serves up some great Cabs and Merlots.

2. New Zealand: Pinot noirs from the Central Otago Valley

3. Italy:  Mount Aetna-grown wines from Sicily, Barbera d'Alba and Nebbiolo d'Alba are good buys

4. Argentian: Malbecs from Salta

5. Hungary: Tokajs

6. Champagne: Ask for "grower champagnes" rather than the more well known negociant champagnes such as Veuve Cliquot, Dom Perignon, Mumms and the like.

7. Loire Valley:  Ask for Muscadets, Chinons (Cabernet Franc)

8.  Ask for "mountain wine" from California.  Those wines made from vines that had to work to survive are, in his opinion, superior to those valley grown wines that had an easier life.

Everyone got the correct answers, so very nice work!

Shark Bite BCC

This was the slide I showed at Saint-Louis that generated the most interest.  As you can imagine the French were fascinated that a guy could be walking around with a basal cell so long as to develop the huge ulceration you see here.  They offered all sorts of theories.  Insane.  Poor.  No socialized medicine (to which I replied au contraire, mes amis, as a retired member of the military he has free access to medical care"). Since this was being presented as interesting cases from my residency in San Antonio, finally a guy in the back cracked up the entire room when he shouted out "Parce que il est Texàn!" ("Because he is a Texan"- they thought all Texans were badasses).  Adding to the Texan mystique was the fact that the week before, during a robbery attempt on the RER train on the way to Versailles I told the robber-who pretended he had a gun in his pocket- 'va te faire foutre', which shall go untranslated.  So, they thought the Texans could walk around for years with a giant, eroded BCC.  

Believe it or not, he did not get a fore-quarter amputation, although his brachial plexus was so badly damaged he had limited use of his arm.  He was Mohsed to clear the margins, then reconstructed by the ortho boys at Wilford Hall.

Giant Keratoacanthoma

Keratoacanthomas are a variant of squamous cell carcinoma which are characterized by rapid progression, a crateriform appearance and sometimes spontaneous resolution.  Some dermatologists, such as those I met in France, consider them NOT to be SCCs, but almost without exception here in the U.S. we do consider them to be SCCs. In fact, all of our pathologists report them as "Squamous cell carcinoma, keratoacanthoma type."

Although the splitters among us will describe multiple variants of KAs, they really come in the solitary and the multiple eruptive varieties.  We have all seen the patients who get numerous KAs on the legs and arms, and go through periods where they grow lots of them in a short timeframe. 

I presented this patient at Grand Rounds at Saint-Louis, and the French trained docs all insisted (after reviewing photomicrographs of the glassy eosinophilic cytoplasm characteristic of KAs) this was a keratoacanthoma, not an SCC and that it should, by nature, involute.  Their enthusiasm for this theory waned when I told them the patient died from the tumor via metastases to the brain.

The reason this lesion was so large was that the patient was a member of a religion which proscribed the use of transfusions, and even though the Mohs team assured her she wouldn’t need one, she refused to go under the knife until it was way too late.  The only thing she consented to was intralesional bleomycin, which did nothing. 

How could you be sure this was not a giant BCC?  First, the BCCs are generally glassy or waxy at the edges, or a little translucent if nodular.  Second, if they are that large, they are not exophytic but are eroded.  Third, the induction of blood vessels would be much more brisk than this KA.  Fourth, BCCs don’t have that keratotic core. 

"Limes Disease"

One of my very favorite pet peeves is when people refer to medical diseases by incorrect names.  "Old Timer's Disease" comes to mind, as does "Lyme's Disease".  Well, in this case this patient really does suffer from "Limes Disease" because she is suffering the consequences of chewing on a lime peel, then exposing herself to the sun.  The result was first a weird, localized sunburn in the area where the lime juice contacted her skin, followed by this hyperpigmentation at the lateral oral commisure and on her cheek (presumably she rubbed it with her limey fingers).  This condition is known as a phytophotodermatitis.

Phytophotos are pretty easy to call, once you know what they are.  Anyone who has a localized hyperpigmentation should be questioned about whether or not they had a preceding burn in the area, and if they deny it but the hyperpigmentation is figurate or especially if there are drip marks on the skin, arch your eyebrow, fix them with a steely gaze and say "Really?  Would you like to change your answer?"  because, of course, they are lying.  Or forgetful.  Or extraordinarily unobservant. In which case they deserve your abuse, anyway. 

There are a lot of plants that have the capacity to cause phytophotos, and limes are among them, but not the only ones.  All citrus has the capacity, but limes, lemons and bergamot oranges are among the most common.  Also, celery and brussel sprouts (especially from the northeast), figs, and certain wild plants  such as some meadow grasses and wild carrots can cause it as well.  My pateint who is a grocery guy refers to them as "celery burns" and apparently it is well known to the guys who work with the veggies at Publix.

As a paleodermatologic note, Shalimar perfume used to contain Oil of Bergamot and thus caused many a phytophotodermatitis until they changed the formula.  It was known as 'berloque dermatitis"which means "charm (or trinket) dermatitis" because the perfume was applied to the neck below the ears, and so the phytophoto would look like it was in the shape of earrings or hanging charms.

The underlying pathophysiology is that of UVA coming in contact with a furocoumarin derivative, which leads to the initial burn, followed by the hyperpigmentation. The hyperpigmentation is caused by two mechanisms:  one, there is pigmentary incontinence (i.e. the pigment leaks from the damaged melanocytes into the superficial dermis after the burn phase) and there is an induction of melanocytes, resulting in the increased number of the pigment-producing cells in the epidermis.

The clue on this patient is the well defined hyperpigmentation in the absence of any clues that made you think it was a nevus, cafe au lait macule or nevus spilus.

Allergic Contact Dermatitis from Cashews

There is a society of dermatologists known as the Holmes Society which get together every year to challenge one another with pictures such as this one. They take their societal name from the hero of every diagnostician, Sherlock Holmes, and they thrive on extracting every possible clue from the clinical photos with which they are challenged.  I am not a member, since I am primarily a derm surgeon, but the idea is very cool nonetheless.   So, if we were to put on our deerslayer, grab our magnifying glass and try to extract all the clues, what exactly would we come up with? What would Sherlock say?  

The first is the historical data.  Primarily left hand, but to a degree it is bilateral.  Second, it is episodic, otherwise I would not have described it as recurrent. Third, going to visual clues it is a bullous eruption.  The bulla has small vesicles in the surface of it, which has significant implications. Fourth, it is pretty well localized to the central palm, with a little "bleeding off" of the rash onto the thenar eminence. The fingertips are spared!!  So, given that spare bit of data, what are the possibilities?

We generally know that the differential for bullous eruptions run from the immunobullous diseases (BP, PV, DH and their ilk), epidermolysis bullosa, porphyria and pseudoporphyria, thermal trauma, chemical trauma, bullous fixed drug to spongiotic dermatitis.  The fact that it is so localized pretty well rules out immunobullous disease.  Thermal trauma?  C'mon.  Chemical?  Same.  


Recurrent bullous fixed drug would almost always have some measure of hyperpigmentation, and the fact it is sometimes on the other palm kills that diagnostic possibility.  Wrong location for porphyria and pseudo, and it is only on palms, which makes EB very, very unlikely.So, if we run the ddx for spongiotic derm (contact, id, nummular, dyshidrotic and dermatophyte- the mnemonic is CINDD) then you can rule out id and nummular and dyshidrotic (it would be pretty symmetrical) and dermatophyte by history and by exam, so you are left with... contact.  

Contact derm can be this bullous, especially after the third or fourth or umpteenth exposure, as was the case with this litttle person.  In fact, the other clue this person had was an extremely itchy anal area, which could really nail down your diagnosis.  So, what was the exposure??  In a word, Anacardiacea.

The Anacardiaceae are a group of plants that encompass a wide variety of fruits and nuts and shrubbery, but in this case, the Anacardiacea of choice for this patient was the humble cashew nut.  That explains why the palmar distribution (how do you hold 'em before you pop 'em in your mouth?) and the relative sparing of the fingertips.  Also explains the downstream symptomatology, too.  The undigested allergenic oils are distributed on defecation to the perianal area, and patients can often get a significant spongiotic derm in the perianal area. 

The Anacardiacea family encompasses poison ivy, poison oak, poison sumac, cashews, mangoes and the marula tree (for a hilarious video of animals getting drunk from the fermented fruit of the marula tree, click here:     http://www.youtube.com/watch?v=D5E5TjkDvU0  ).  Recent taxonomic reclassification had included pistachios in this group, although I have yet to see a patient who has gotten an allergic contact reaction from them.  So, if they are allergic to rhus, they are possibly allergic to cashews.  And mangoes.  

Dr. B

Classic Kaposi's Sarcoma

Moritz Kaposi, a Hungarian dermatologist practicing at the famed Allgemeine Krankhaus in Vienna, Austria, described an interesting syndrome in which he noted purplish nodules on the lower legs of elderly men, most often of Mediterranean extraction. The pathology revealed it to be a  syndrome of proliferation of spindle cells, with multiple vascular channels. Until recently, that's pretty much where our understanding of this unusual disease began and ended.  Of late, though, we have found an association with an infective organism and, if tested by immunohistochemical means, the lesions will counterstain positive for its etiologic agent, Human Herpes Virus 8 (HHV8).  

There are four main subtypes of KS:  They are the Classic KS (as the elederly Italian man displayed), which is notable for being discovered on the lower legs of primarily elderly men of Mediterranean or Jewish heritage; African Endemic Cutaneous KS, which differs from Classic KS in that it occurs in African men between 20 and 50; African Lymphadenopathic KS  which is found in African children under ten; and AIDS and Immunosuppression Associated KS, which are sometimes lumped together and sometimes split apart.

The diagnostic clues to this patient were both in examination and by history.  I gave you the most important clues:  he was elderly, Mediterranean, the "rash" was asymptomatic (which rules out lichen planus). The admittedly somewhat unclear pic showed a proliferation of purplish papules which clearly were not stasis-related.  Purplish spots + Old Italian guy + asymptomatic = Classic KS.

As a point of diagnostic importance, in my experience AIDS associated KS often looks like a very subtle bruise that does not resolve like the usual contusion.  It is often on the head and neck rather than the lower extremities, although it can be anywhere.  

A couple of things about KS.  First,  it is not a true sarcoma, but instead it is a cancer of lymphatic vessels.  Second, it can affect the mouth and GI tract, particularly in the AIDS associated variety.  When I was a surgical intern at the University of Pennsylvania, while working in the Emergency Department we had a young (twentyish) black man stumble through the door, asking for help.  He, with help, walked to the gurney in the back, laid down and as part of his workup I started an IV and drew blood from the opposite arm.  I got a call back from the lab, saying in essence "You idiot, you drew his blood upstream from the IV!".  I knew I didn't, but the lab demanded I draw the blood again.  Again, from the opposite arm I drew his blood, to which I appended a drawing of the patient, his IV arm and the arm I drew the blood from.  The head pathologist got on the phone to congratulate me.  "Boy, you got yourself a facultative anaerobe down there.  His hemoglobin is 1, his hematocrit is 4."  The subsequent workup showed that he had KS in his gut, and obviously had bled so slowly into his gut that he was able to tolerate his astonishingly low numbers.  He, of course, had HIV and died on that admission from pneumocystis carinii pneumonia.

One last point.  If we know Kaposi was Hungarian, it sheds some light on how we ought to pronounce his name.  As many of you know, S is pronounced as a Sh sound in Hungarian, and A is often pronounced Ah.  So, the proper pronunciation of Kaposi's Sarcoma is not Ka-PO-si's, but rather KAH-po-she's.  Won't do you much good, but maybe, just maybe, you'll one day be able to lean into the microphone and confidently say "Alex, I'll take Hungarian Names for a thousand."

PMLE/Actinic Prurigo

Polymorphous light eruption is a disease that is as easily made from the history as from the physical examination.  The salient history of the typical PMLE/AP patient is the onset of itching, burning and painful lesions that usually begin in the springtime, and often after a significant outdoor exposure.  The reason for this is complex, but I think of it as a true allergy to ultraviolet light.  There are good studies which show the lesions of PMLE (erythematous papules, patches and plaques on sun exposed skin) are very similar in their pathophysiology to Type IV cutaneous hypersensitivity (allergic contact dermatitis).  

In Native Americans (aka First Nations People if you are Canadian) this condition is usually referred to as Actinic Prurigo and is inherited in many tribes as an autosomal dominant condition with a very high penetrance.  It is more common in females.  In addition to the usual PMLE symptoms, they also have a cheilitis which mimics the vernal onset of the other symptoms.  The patient we saw in the pic was from the Pine Ridge Sioux Reservation, which is one of the more painful places in the US to live.  High degrees of alcoholism, child and spouse abuse, early pregnancy and illegal drug use makes the Pine Ridge Reservation a sad place, indeed. 

The history gives this away as PMLE, and there is no reasonable DDX other than solar urticaria, which has less of the vernal periodicity.  As a point of interest, we rarely see PMLE here in Florida because our chronic UV exposure hardens our skin to ultraviolet light and thus we don't get that exposure-loss of exposure-reexposure cycle that the northerners do.  

Many of you picked PMLE, and a couple picked Actinic Prurigo. Excellent work, everyone.

Derm Challenge: More Than a Game...

Welcome to the Derm Challenge.  This game is designed for Physician Assistants and Nurse Practitioners who practice in the field of dermatology.  I am a board-certified dermatologist practicing in Melbourne, Florida, and I currently have two PAs and one NP working with me.  We practice as part of a multi-specialty group, Melbourne Internal Medicine Associates, and we think we have the best of all lives.  We love derm.

The Derm Challenge is a game, nothing more or less.  It is knowledge based, much like Trivial Pursuit, but since it is our specialty, it is anything but trivial.  The rules are pretty straightforward, as you will see below, and at the end of each month, I will award a nice bottle of wine to the winner of the month.  

Here are the rules:

1. Don't take it too seriously.  Seriously.  It's just a game.

2, Each day I will post a question, usually in the form of a clinical photo, with some additional questions added on as bonuses from time to time.

3. All rulings by the judge (me) are final.  No quibbling.

4. Please do not cooperate among players, and also please do not use dermatologists as a source of answers.  It's not fair to the other players.

5.  Some time after 5 PM on the day after the question is posted, I will reveal the answer and an explanation.  The answers generally are based on diagnostic criteria and some background about the illness, but my answers almost never will discuss treatment, in that it is far beyond the scope of the game.

6. The images are of real patients, and should be respected as such.  The photos either come from the National Library of Dermatologic Teaching Slides, or they come from my own personal collection of over  three thousand clinical photos I either took myself, or copied with permission from the phenomenal image library at Wilford Hall Medical Center's dermatology residency teaching library. These images are intended for teaching purposes only.

7.  At the end of the year, I will award a prize to the Grand Winner, who won more monthly prizes than anyone else.  This year, it will be a bottle of Dom Perignon champagne.

8. All answers, all scores (except for the winners) and all emails and email addresses will be kept confidential by me, to the limits of my abilities.  I realize your privacy is important, and your privacy will be protected as much as possible.

As a last word, please share this game with your colleagues.  The goal is to have some fun, challenge yourselves, and maybe score some vino in the process.  À vos santé!