The End of Our Challenge- For Now

Of course, the last Q was CREST, a variant of scleroderma, and from my description there is no way you could tell whether or not it was systemic scleroderma or merely CREST, so you got credit for either answer.

Several of you wanted to know a little about me, so I will fill you in on who I am and why I chose to do the Challenge.

As most of you know, while I was in residency, I managed to amass a collection of clinical images which rivaled many of the academic programs with which I had been associated, and I always felt I needed to share them with my PAs, in a somewhat controlled way which would enhance learning and engender a desire to learn more.  After casting around a bit, I hit on the idea of using wine as a reward, because it coincided with my love of wines and gastronomy in general.  

After a while, we generalized it to other PAs and ARNPs, which eventually ended up with our blog getting in the neighborhood of 25,000 hits from all parts of the US and the world.  Even Romania and Bulgaria!  It turned out to be a lot of fun from my perspective, because I got to know some really bright, motivated people along the way, and it also honed my knowledge base a bit.  I've always enjoyed general as well a surgical derm, although 90% of my practice is related to derm onc, and it forced me to remember a lot of details which otherwise would soon slip into the part of my brain devoted to baseball statistics and differential equations.

I am happily married and have a daughter Liana (seated on the couch above); her boyfriend Josh (in plaid); a son Daniel (blue shirt, who has been a subject of a couple of Q's); a daughter in law Caroline who is Quebecoise; and a marathon-running, five bar exam passing, bicycling, book writing wife with whom I spend on average an hour a night, drinking wine and talking about the world.  What a family.  What a life...

My hobbies are modernist cuisine, wine and bicycling, and I am currently preparing to ride all of the significant climbs of the Tour de France this July, in the Pyrenees, the Alps and Provence.  I am training with Kevin Livingston, who used to be He-Who-Shall-Not-Be-Named's lieutenant on the Postal Team and Kevin now works in Austin at Mellow Johnny's, and I am lucky to have him as my coach.  He's a bit of a sadist, though.  He says it's for my own good.  We'll see.  

And, for the record, I truly believe what Lance did was no better, but no worse than any other cyclist of his era.  He was just better at it, in all phases of both riding and doping, and so he became the whipping boy.  And his personality is a bit strong, to put it mildly, so that also had something to do with it.  It is well known that the most widely admired cyclist of all time, Eddy Merckx, used to take amphetamines prior to the mountain stages, which was a hell of a lot more dangerous than EPO and Testosterone.  

So, for those of you who struggled and swore and pored over your texts (and Google), I have both admiration and a sense of kinship.  You struggled, and the knowledge you walk away with is not only the specific (e.g. how to diagnose PCT) but also more important, you learned that between what you know and what you can access, there is virtually nothing you can't figure out.  Your patients are very, very lucky to have you.  

Lastly, BE YOUR OWN BRAND!  If you do, you will always, ALWAYS have a way to make a good living, and you will be immeasurably important to those for whom you care.

Dr. B

Pyoderma Gangrenosum

PG is, to say the least, an exasperating disease, both from the standpoint of the patient as well as the treating provider.  It can be refractory to every treatment in Shelley and Shelley, or it can heal on its own, or it can respond one time to one regimen, then be refractory the next go-round.  

PG is notorious for, in its smaller iterations, being confused with spider bites.  If you EVER hear of anyone who says they get multiple spider bites with subsequent necrosis, nine times out of ten, you are dealing with PG.  

The hallmarks of PG are all there for you in the pic above:  Purplish, undermined skin, evidence of precious ulceration, and the history of pathergy, that is a pathologic response to trauma.  Think of it like the Koebner Phenomenon of psoriasis; it is an abnormal physiologic response to external trauma.  

Necrobiosis Lipoidica is a different animal in that it tends to have much smoother plaques that are more uniform, and tend to be more pretibial, although that is not always the case.  

If you answered correctly, then click on this link and enjoy my favorite song from the eighties : http://www.youtube.com/watch?v=EHJmPcILfg8

If, however, you didn't, punish yourself by listening to this link, which is possibly the worst song ever recorded : http://www.youtube.com/watch?v=-rfD8NQLO8w

Late Phase Porphyria Cutanea Tarda

This was a toughie for a lot of you, and so I will describe what I see which, in my mind, are the critical cues for making the correct diagnosis.  What I see are multiple erosive-looking scars on the dorsum of the hand, with a milium on the fourth digit.  Any time I see any changes, either blisters (which I rarely see) or exaggerated aging, which I commonly see, or scarring, as above, I think the patient has PCT or pseudoporphyria until proven otherwise.  

As you all know, PCT has a predilection for the dorsal hands and, along with other findings of PCT (name three for three bonus points) is a marker for internal disease.  Usually, it is associated with an underlying condition, with hemochromatosis and alcoholic cirrhosis being common factors, but by way of pathogens, Hep C and HIV are the usual suspects.

Why wasn't this LP?  Well, I have seen LP just about everywhere, but the dorsal hands are not a really common location.  I like the wrists, legs, lower back, nuchal scalp, etc., but dorsal hands, not so much.  So, once again, if you see changes on the dorsal hands, think PCT.

Linear Scleroderma en Coup de Sabre

Linear scleroderma is a condition characterized by localized linear atrophy that resembles the findings one would find, at the end of the Franco-Prussian War, in soldiers who had been injured from cavalry charges from the saber-wielding Prussian Cavalry.  Since this description is in French and not in German, one would correctly presume the French bore the brunt of these kinds of injuries.

The onset of this condition is, as above, in childhood and usually is absent of underlying neurological deficits, unlike its cousin Parry Romberg Syndrome, which is a more severe condition involving progressive facial hemiatrophy, which can result in the entire affected side of the face being smaller than the other.  On a clinical basis, without this difference from side to side, one would have to lean on en Coup de Sabre being the preferred diagnosis.  This is just such a case.  

Linear Scleroderma en Coup de Sabre is usually unaccompanied by routine laboratory abnormalities, except in research settings.  Thus, although Scl-70 and ANA titers can be out of whack, usually they are not.  In fact, some authors question whether this entity is at all related to morphea or scleroderma.  

This disease does not, in its pure form, extend below the orbital ridge, although there are overlapping cases with Parry Romberg which go lower.  If you study the case above, you can see the atrophy, the superficial nature of the vasculature and the linear nature of the defect.

Fabry's Disease

Trust your eyes.  When you see something that looks like cherry hemangiomas, trust your eyes.  Many of you ended up working this question backward, by trying to put something together that had both skin lesions and urine pathology, and came up with Henoch Schoenlein Purpura, which, of course, looks nothing like these angioma-like structures in the top picture.  You didn't trust your eyes.  

Fabry's Disease is an X-linked disorder which leads to deposition in the endothelium of a certain kind of lipid which leads to the pathophysiology.  Many times the disease manifests itself with symptoms of a CVA, or renal insufficiency, or myocardial infaction in a relatively young person, and if it is in association with these lesions we see above, known as angiokeratomas (which look  a  lot like cherry angiomas) then one should consider Fabry's Disease.  The finding is classically called angiokeratoma corporis diffusum.  

The classic finding in the urine is the lipid droplets, as seen above.  It is rare that derm will be the first to make the diagnosis of Fabry's but at the same time we often times can help the other specialties tie it all together.

Primary Syphilis

Given that the answer is "no" to the question you should be asking (which is "does it hurt?") the differential diagnosis is mercifully brief.  A large, painless ulcer on the penis?  There is one and only one answer.  Our little treponemal friends are just setting up shop, preparing for what they hope is a long, fruitful (from their perspective) relationship with Mr. Happy's friends including the skin, the brain, the spinal cord and eventually the deep tissue.  

Disseminated Intravascular Coagulation

The way to think of this problem is not so much to look at it and say "DIC" but rather to look at it and say to yourself "This is clearly a vascular problem which resulted in small vessel obstruction, followed by bleeding into that same compromised vascular distribution".  As such, you would come up with a ddx which would, eventually, include DIC.  

Think of DIC as not really a disease, but rather a manifestation of disease, whether it be from sepsis, trauma, systemic disease (e.g. pancreatitis), malignancy associated thrombotic changes, envenomations from certain snakes, or anything else which could trigger the coagulation cascade (abruptio placenta, weird manifestations of antiphospholipid syndrome, etc.)

On a localized level, therefore, I would include frostbite, which most of you listed, as one of these phenomenae, so don't feel bad for listing it.  I've seen frostbite that looks just like this, and although I have never seen DIC in person, I suspect there would be plenty of other clues to figure that out.  I also doubt I would have the presence of mind to take a pic of DIC unless, like above, the patient survived.

A few of you listed DIC and one of you (you know who you are) listed ONLY DIC, which was a pretty bold move.  

Interstingly, the inflammatory cytokines seen in certain trauma patients mimic the profile seen in sepsis, and the current theory is that it is the cytokines which trigger the cascade leading to DIC.

Cholesterol embolism

Cholesterol embolism usually occurs after invasive vascular procedure, and it marked by discoloration of distal circulatory fields such as the pads of the toes, as above.  The blotchy, discolored appearance, especially if sudden in onset, is referred to as "blue toe syndrome" and is very highly suggestive of cholesterol emboli.  Some of you noted the pincer nails and onychomycosis, but the reality is, given the clues all of you should have honed in on the vascular hints and nailed this one.  Pun intended.  

Pruritic Urticarial Papules and Plaques of Pregnancy

Pruritic Urticarial Papules and Plaques of Pregnancy, aka PUPPP, is a benign condition that arises classically in the third trimester for a primiparous female.  Of note, a common dermatosis of pregnant females is atopic dermatitis of pregnancy, which occurs earlier in the pregnancy.

The classic description of PUPPP is the development of intensely pruritic urticarial papules within the striae of a primip.  HOwever, in some cases, it can occur in multips, and the lesions may not be confined to the striae.  Typically, there are no bullae, but there may be very small vesicles.

Of course, the IF is negative in PUPPP.  It would be positive in pemphigoides gestationis, but is never positive in PUPPP.