Frontalis-Associated Lipoma

In 1989, Stu Salasche, Bill Grabski, Martha McCollough and Vince Angeloni described an entity they described as being either enveloped in the frontalis muscle of the forehead, or just beneath it.  Their term, Frontalis-Associated Lipoma, has stuck with this entity ever since.  

The tricky thing about FAL is they are not easily removed, and in fact are a real pain in the neck because of the investiture in the frontalis.  My preferred technique, which involves a horizontal incision following the rhytids, followed by significant undermining and then a vertical incision in the frontalis, following the muscle fibers, down to the lipoma, is by far the best way to handle them.  I learned that technique from the authors themselves, while they were on faculty at our sister residency at Brooke Army Medical Center in San Antonio.  

The clues that we were not dealing with an epidermal inclusion cyst are primarily that the lipomas are soft and much more mobile than a cyst of that size.

Leukemia Cutis Secondary to CLL

This very nice gentleman is a patient of mine who has been with me for well over a decade, and he presented about two weeks ago with a nodule on the scalp which was biopsied and found to be a squamous cell carcinoma.  Interestingly, though, my pathologist (she is very good!) noted sheets of lymphocytes around the lesion and asked me if, perchance, our patient had CLL.  The answer, of course, was yes, but it was well controlled.  Up til now, that is.

We scheduled him for surgery within two weeks, as we do all invasive SCCs, and when he came to the office for the surgery, he mentioned he had some swelling on his forehead for the last couple of days.  The plaques were slightly erythematous, firm to the touch and warm.  Well, the differential diagnosis in my head really came down to infectious versus neoplastic, but his CBC showed 20K lymphs with zero activity going in the PMN range, but a vast predominance of lymphs. This makes the diagnosis of leukemia cutis a virtual certainty.   Had it been infectious, we would have seen a much less lymphocytic, much more PMN-rich differential. Additionally, we had the comments from the pathologist regarding the SCC to base our opinion on.

Leukemias are proliferations of clonal populations of leukocytes (in this case lymphocytes) which are maturationally arrested. Leukemia cutis represents clonal proliferations in the skin which may be seen in AML, ALL, CML, CLL, as well as hairy cell leukemia and myelodysplastic syndrome.  If it is associated with AML or ALL, it has a very grim prognosis.  

Palmoplantar Keratoderma

Palmoplantar keratoderma, aka Keratosis Palmaris et Plantaris, is a not really as much a diagnosis as it is a symptom of another underlying condition.  It is, as you see, characterized by a thickening of the palms and soles which may be diffuse or, as above, not.  It can also be punctate, but in my mind that condition doesn't really fit with the others.  

It may be associated with underlying malignancy, resulting sometimes in bizarre patterns such as tripe palms; it may be associated with ectodermal dysplasias, or the straightforward genetic maladies which are too numerous to count in this forum.

In an acquired setting, it can be associated with menopause, meds, infections and systemic disease.

Pinot Noir

Pinot noir is a finicky, thin-skinned grape which only grows in a fairly narrow climate, which means that it is the high-maintenance cousin of happier, more well adjusted grapes such as Cabernet and Shiraz.  This essentially means that there are inevitably going to be good years and bad years for pinot noir, and so it pays to know whether the wine you buy is from one of those good vintages.

Pinot is grown in a major way in two areas of the US, Chile, Argentina, South africa, Australia and the Burgundy region of France.  For a bonus point, where are the best Cabernets from in France? The only serious Pinots are from Cali, Oregon and Burgundy.  All the others are pretenders.

As you can tell from this pic, taken at Domaine Drouhin winery in Oregon (Domaine Drouhin 1991 pinot was the first Oregon wine I ever drank, on August 14, 1994.  Don't ask me how I remember this.  I am the Rain Man of fine dining.  I remember everything.)

My favorite wines from Oregon come from Domaine Serene, especially their Evanstad Reserve, but overall my favorite of the winemakers is Laura Penner-Ash, who makes both an outstanding Pinot and also a brilliant Shiraz.  

If you ever go to the Willamette Valley, I highly recommend two things:  The Allison Inn is one of the most striking hotels I have ever stayed in, and the Joel Palmer House, a resto that specializes in wild mushrooms.  One of my foodie friends and I had their mushroom tasting menu, which was insanely out of this world.  

Well, enough about epicurean delights.  Time to close out October.  Three more days to weed out the wheat from the chaff, the pips from the grapes.

Alkaptonuria

Alkaptonuria is an autosomal recessive disease that is a result of abnormal phenylalanine and tyrosine metabolism, characterized by excretion of large amounts of homogentisic acid (aka alkapton) in the urine, hence alkaptonuria.  The circulating HGA causes significant damage to all classes of cartilage (ergo damage to joints and heart valves), leads to kidney stones and a characteristic calcification of the annulus of the intervertebral disks which leads to a syndrome similar to bamboo spine.

It generally is either noted in infancy with discoloration of the urine, or, if undetected then, in the late thirties or early forties, when accumulation of the pigment begins to stain the sclera or, in advanced cases, the tendons.  It also can declare itself, as in this case, by chromhidrosis.  It can be excreted in the sweat glands of the face, the axillae or the areola.  

Now, to the nitty gritty.  I gave you all clues that the meds were not the problem.  I also mentioned she had a stone cold normal examination, including her axillae which were normal as pumpkin pie.  That implied her sclerae were normal.  All this means we are catching her on the front end of the clinical expression of her disease.  For two bonus points, which two countries are noted for high incidences of alkaptonuria. Finally, and most critically, I mentioned (in a separate email, which should have told you it was very, very important) she was in her late thirties.

Which question should you ask her?  Most importantly, you should ask her about dark urine, which she denies.  My next question, which was answered with a "how the hell did you know that?" look was, did she have back problems.  In fact, she had just gone to a back doc the week before and had her MRI the day before I saw her.  She was sitting rigidly straight, as if she were HLA B-27 incarnate.

Labs?  Urine HGA and a CBC, to chase down the rare bleeding diathesis.  If, for some reason, she were negative on the HGA, I would then start looking at heavy metal assays (trying to link the chromhidrosis with the tremor).  

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Delusions of Parasitosis

The Ant
by Ogden Nash

The ant has made himself illustrious
Through constant industry industrious.
So what?
Would you be calm and placid
If you were full of formic acid?

If you really want to cast a curse on someone, instead of wishing that they might live in interesting times, as the Chinese do, you may wish they would develop delusions of parasitosis.  It's a nightmare for these patients, because they so completely believe that the lint, or hair follicles they pull out, or the occasional unlucky stray ant are imbedded in their skin, and they obsess  about them,  which drives them batty. Or, to be more accurate, battier. And they can't understand why you think they're wrong.

Examination reveals linear excoriations, but no primary lesions.  Often, they go from amateur to pro and develop prurigo nodules and ulcers.  This can be the only symptom of psychiatric disease, or it can present secondarily to bipolar illness, schizophrenia, anxiety disorders, and most commonly with obsessive states such as OCD.  

It usually occurs in white middle-aged or older women, although the condition has been reported in all age groups and in men.  Morgellon Disease describes a condition characterized by the delusion of fibers being attached to the skin. Morgellon's has become a new designation for delusions of parasitosis.

Median Canaliform Dystrophy

MCD is a condition wherein the nail matrix (NOT nail bed, NOT nail plate, NOT proximal nail fold) is damaged, Often through trauma, but it can also be idiopathic or can be related to genetics.  Think of the nail as a rainbow.  It is formed as ROYGBIV and arcs across the sky, but always the orientation remains the same: ROYGBIV.  So, when you have a MCD as above, the defect is at G (in other words, pretty much in the middle of the nail matrix).  This patient also has a paronychia, but that was a red herring.  

The weird thing about these defects is that they often wax and wane, and may disappear, only to recur years later.  

Merkel Cell Carcinoma

It's kind of humbling to realize that this cancer was first described in 1972 by Toker.  The Merkel Cell is a pluripotential cell that is of neuroendocrine differentiation, and as a malignancy it tends to be rapidly growing, tends to pick elderly patients and often leads to metastasis and death.  

Nearly half the time it is on the head and neck, but can be virtually anywhere.  The prognosis is worse than that of invasive melanoma, and it has a high rate of local recurrence (one of my patients currently has that) and distant metastases occur up to 75% of the time.  

The tough thing about this clinically is that the tumors begin innocuously, and then accelerate so that by the time the patient goes from thinking it is a pimple until the time of metastasis is fairly short.  

How best to differentiate this from BCC?  If you look at the margin of this tumor, it has a "collarette" of normal skin extending onto the tumor.  BCC almost never does.  Also, it has none of the pearliness of a large nodular BCC.  Nor does it have the telangiectatic surface typical of large BCCs. 

How about SCC?  This is a much tougher call.  It certainly does not fit the keratoacanthoma picture in that there is no central crater, but a large SCC could look like this, but usually it would be more eroded, and instead of the collarette it would be more likely to be wall to wall.  This is typical of a Merkel Cell both in patient age, in location and in appearance.

Malignant melanoma Breslow's 0.6 mm, with regression 

When you look at a red octagonal street sign, you immediately know what it is and what it signifies.  The same should apply any time you see a lesion like this.  Not many things scare me.  I'm not afraid of spiders, snakes, or heights but I am afraid of anything that looks like this.  The thundercloud gray to pink look in the center of what can only be a melanoma means only one thing:  Regression.  

As you know, regression means that the lymph node has already gotten a taste of the melanoma, even though it is not very thick, and the lymph node then mounted an attack on the primary lesion.  Even in the case of a non-palpable node, it is a metastatic problem  from the moment regression is evident.  Will the metastasis kill the patient?  Maybe, maybe not, but nevertheless it is there.

All of you recognized this was a melanoma, but if you did not mention regression, you did not get full credit for this answer.  When you see a lesion like this, check their nodes and think about SLNBx and heme-onc referral.  

Probable Lid Margin Melanoma

We all see pigmented lesions on the lid margin as a matter of daily practice, and the challenge is in distinguishing between benign and malignant lesions.  This case is illustrative of the challenges which face us when we are confronted by such a lesion.

The patient presented to me yesterday and stated she had the lesion for six months or so, and that it had grown significantly over the last six months.  She had permanent eye liner put in approximately seven years ago.

Since I don't have the path yet, I will give you what I see and you can take it from there.  I see a bluish-black pigmented papule on the free margin of the lid, which looks like it extends roughly 2 mm above the surrounding skin.  It is a bit sunken in in the center, and on the conjunctival aspect of the lid, there are areas of what appears to be regression.  Extending further toward the lateral aspect of the conjunctiva, we see extension of the pigment, blue-black proximally,  jet black distally.  

When dealing with pigmented lesions of the lid, there is one rule which almost always is valid: if it extends onto the conjunctiva, it is bad.  In this case, really bad.

The only serious ddx for this (besides perhaps a cellular blue nevus) is that of a pigment granuloma, but the extension onto the conj is so unlikely in that scenario as to be eliminated as a serious consideration.

I will inform you when the dx is out, but I am at this time only giving credit for r/o melanoma.

Erythema Elevatum Diutinum

EED is a disease that looks neoplastic, but is inflammatory in nature.  Most of the primary lesions of EED are shiny, smooth papules or plaques that have the appearance of rubbed warts.  Unlike rheumatoid nodules they are mostly epidermal and dermal in appearance, as opposed to dermal and subcutaneous.  Primarily they are found over the hands and feet, knees and elbows.  

The classic histopathology of EED is a leukocytoclastic vasculitis. Attendant to the LCV is fibrosis which gives the EED papules their firm presentation.  

Associated diseases include HIV, IgA monoclonal gammopathy and other hematologic abnormalities, celiac disease, Wegener's, as well as chronic, recurrent strep infections.

The Papulonecrotic Differential Diagnosis

1. PLEVA (pictured here)

2. Lymphomatoid papulosis

3. Papulonecrotic tuberculid ( a rare form of cutaneous tuberculosis)

4. Arthopod assault

This is an important picture, in that the papulonecrotic ddx should roll off your tongue just as easily as ROYGBIV.  This is one of the fundamental descriptors in dermatology.  Congrats to those who got it right!

Staphylococcal impetigo

Impetigo can be caused by either staph or strep (GABHS) but these days, more is caused by staph than strep.  Bullous impetigo is mostly a newborn disease, and this is clearly not a newborn.  If it were staph scalded skin syndrome, it would be caused by two different exotoxins, which are generated by both a chromosomal process as well as a plasmid-generated process.

Since nearly everyone got this, I will not belabor the diagnostic cues.  

Graves' Disease

Graves' Disease is a veritable treasure trove for dermatologic signs and symptoms.  So many of the signs the old dermatologists and internal medicine docs used to look for are now obviated by routine thyroid panels, well before the thyroid disease declares itself by hair loss, hyperhidrosis, melanoderma, Plummer's nails or the like.  In other words, the lab rats are stealing our thunder!  

Pretibial myxedema occurs in roughly 4% of cases, presumably due to thyroid stimulating antibodies which also trigger production of glycosaminoglycans pretibially, preradially and retroorbitally.  The female to male ratio is 7:1.  Bonus q:  is Graves' Disease associated with hyper or hypothyroidism?  

Post Streptococcal Erythema Nodosum 

Erythema (red) Nodosum (painful) is an inflammation of the subcutaneous fat, and is triggered by a variety of circumstances, but most of them involve immune complexes being deposited in the medium sized vessels of the septae of the panniculus.  

The appearance is of a slightly elevated erythematous plaque often with, as you see above, a "vascular steal" phenomenon.  They are, as the name implies, very tender.  The location as well as the multiplicity and the tenderness all are clues as to its identity.

Most cases occur in young adult females, are usually multiple, usually pretibial and can be associated with other symptomatology associated with immune complexes such as joint pain, fever, headache and malaise.  Ulceration does not occur and they heal without scarring.  

Historically, tuberculosis was the most common associated disease, but currently we see it most commonly in association with  streptococcal disease, particularly pharyngitis.  Of course, it can also be associated with gut pathogens such as Yersinia, Salmonella and Shigella; but also a kazillion other infectious diseases (too numerous to be listed here) and also, of course, Sarcoidosis.  However, for two additional points, name two deep fungal diseases that cause EN. Also, the estrogens in oral contraceptives can trigger this condition, as can other meds such as sulfonamides.  

Eczema Herpeticum

Eczema herpeticum is a condition wherein the patient has a preexisting condition such as atopic dermatitis, and then suffers a herpetic outbreak, which subsequently seeds the inflamed, eroded skin with HSV.  This is such a case.  

This patient originally had a herpetic outbreak on the lips, which then quickly generalized to nearly all his skin.  Luckily, he was immunocompetent, otherwise this would have killed him.  He did very well on systemic (IV) Acyclovir.  

The clues to the diagnosis are history, especially the perioral history with the spread like wildfire, and also the primary lesions, which are vesiculopustular.  

Also known as Kaposi's Varicelliform Eruption, eczema herpeticum is a devastating disease that can be life threatening if not treated promptly.  The take home message is this, though:  In a patient with a chronic inflammatory condition such as atopic dermatitis or Darier's, if they also have a history of heprpes labialis (or genitalis) please ensure they are prophylaxed so as not to end up with the disseminated variety of their condition.

Incontinentia Pigmenti

IP is an X-linked Dominant genodermatosis noted by abnormalities in multiple organ systems.  It got its name from the whorled, irregular distribution of pigment in Blaschko's lines.  It is almost exclusively represented in females, being considered commonly a fatal mutation in males, with the exception of XXY Klinefelter's Syndrome males.  It is considered one of the ectodermal dysplasias, and thus will have many of the manifestations of ectodermal dysplasias.  Weirdly, they have a defective X-inactivation system, which causes both normal genetic expression and mutated genetic expression to occur at the same time in the same individual.

The lesions begin as vesiculobullous lesions, progress to verrucous linear and whorled plaques, progress to hyperpigmentation and then eventually they fade, often to hypopigmentation and atrophy.  Occasionally, these women may not know the diagnosis, and with the extremely subtle findings of some IP adult patients, the only clue may be multiple spontaneous abortions (presumably of male babies). 

As with many ectodermal dysplasias, they can have associated hair, nail, ocular or dental abnormalities, as well as neurologic abnormalities including seizures and mental retardation.

In contradistinction to the group of epidermal nevus syndromes, the lesions of IP are more swirled, and instead of being raised they tend to be flat or atrophic.

Superficial Spreading Melanoma with Signs of Regression

Try to imagine what this lesion would look like clinically.  What you see is scattered nests and individual melanocytes which have a "dusty (finely granular) cytoplasm, enlarged blood vessels and some evidence of regression in that you can see clumps of melanin in the superficial dermis.  There is superficial perivascular inflammation as well.  How does this translate to the naked eye?

The scattered melanocytes mean that the edge of the lesion will be more diffuse and hazy rather than clear cut, because the usual well-defined melanocytic lesion has nests of cells, rather than individual cells of pigment production at the edge.  

The pigment incontinence gives it a very, very dark appearance, often jet black, because of the depth of the pigment.  This pigmentary incontinence comes from the lesion being attacked by the immune system, which in turn causes the pigment in the melanocytes to leak out into the dermis.  

The enlarged blood vessels give it a slightly pinkish look at the periphery, and that, along with the inflammatory cells, makes it look slightly edematous.

I gave credit for any answer which involved melanoma, because it was a very subtle call between SSMM and MIS with regression.