Pellagra

Well, if ever there were a great out-of-the-blue case, this is it.  This patient was scheduled to see us for an unspecified rash, and when we scheduled him, he forgot to keep the appointment.  When we saw him, he had this rash in a necklace distribution, rash on the dorsal hands, also on the back of his neck.  He had been complaining of diarrhea and lethargy, and all this despite eating a relatively varied diet.  

His PCP says he's in pretty good shape, but I'm not so convinced.  The rash is classic for Casal's Necklace, and the diarrhea and confusion (pre-dementia?) would certainly make one think this patient has pellagra.  In that case, two things to do: First, start him on Niacin supplementation.  Second, get him a workup for a carcinoid tumor.  That's the PCP's job, but we advised the PCP on the reasonable route to evaluation.  

What else could it be?  Well, photoallergic contact dermatitis could theoretically give one this kind of rash, should he be allergic to something he is applying to the skin, such as sunscreen, but he is unlikely to have this problem since he is African American.  Other than that, and ESPECIALLY in the face of the associated symptomatology, this is pellagra until proven otherwise.

This month's winner is Karen, from the Tampa area.  She will be hoovering up some fine Pinot Noir wine from her local wine distributer, and will forward a picture soon.

Factitial Dermatitis

Who hasn't had one of these?  They are among the most maddening of patients, because they are absolutely convinced that something organic is going on with their skin, and often their PCPs have also bought in to their story.  

Multiple ulcerations and scars in an odd distribution (why do these lesions stop in such a sharply demarcated fashion?) give it away.  When on the back, look for lesions in places they can't reach.  It is rare to be evenly distributed across the chest in women, and although common on the face, it is pretty unusual around the eyes.  

Any patient who tells you they see bugs or worms coming out of their skin is de facto factitial, as is anyone who brings in specimens of bugs they get out of their skin (unless, of course, they are real bugs, such as crab lice, which earn them a pass in my book.  I'm just sayin', show me the bug or beat it.)

I really do feel sorry for these patients, because they are being driven mad by these perceived infestations, but they really should be another specialty's problem, not yours.

Digital Pitting from Scleroderma

Scleroderma is a wicked disease.  The disease first robs patients of their well-being, then their dignity, and finally their health and perhaps their life.  The picture we see above is a harbinger of future problems.  These pits, which are classically found on the fingerpads, are part of the diagnostic criteria for systemic scleroderma.  Those criteria are as follows:

Major criterion:

1. Sclerodermatous change proximal to the MCPs.

Minor criteria:

1. Sclerodactyly

2. Digital pitted scars of fingertips or loss of substance of the finger pads

3. Bibasilar pulmonary fibrosis

In order for the diagnosis to be made, the patient must fulfill either the major criterion, or two of the minor criteria.

Raynaud's Phenomenon, nail fold capillary changes, eosinophilic fasciitis, and nephrogenic systemic fibrosis can also be part of the syndrome, along with myriad other changes that involve virtually every organ system in advanced cases.

The pitting associated with Gorlin's Syndrome are far more subtle, and usually more numerous.  The various disorders in keratinization usually result in a much higher number, and more dramatic individual presentation.  Plus, I gave you the clue that you should have surmised it was part of a syndrome.

If you named an associated feature of scleroderma, I gave you credit for each non-redundant one that was reasonable.  A few of you really cleaned up on this one.  Nice work.

Atopic Bleach Baths

Atopic dermatitis is a morass of immune interactions which play out in a most unpleasant way, as the "itch that rashes".  Flexural creases are classic, but extensor surfaces, classic LSC areas and facial areas can be involved.  Every once in a while, a therapeutic measure comes up which has a profound and positive impact on these patients, but for the most part, it is just a hard slog of management.  Moisturizers, avoidance of irritants and use of antiinflammatories, as well as control of the environment, all play a role.  

One of the more interesting strategies to come out in the last decade is the use of bleach baths.  The usual dilution ratio of 1/2 to 1 cup of water for a forty gallon bath lowers the Staphylococcal burden on the skin, which in turn downregulates the immune response.  The result?  Clearer skin, less itching, better sleep, less of the hyperallergic responses (allergic rhinitis and asthma) and overall increase in the quality of life.  Not bad.  Not bad at all.  Best if you limit these baths to twice a week though. MOre than that and they will dry out.

.I gave everyone credit for this one, more because I wanted to raise consciousness on this subject, and ensure we were all on the same page re: how to incorporate bleach baths into atopic therapy.

Scleroderma en Coup de Sabre

Localized scleroderma is a strange disease, often showing up as a discoloration that looks scar-like, which then evolves into an atrophic plaque, usually in a linear fashion.  In this case, you can see the linear aspect in two areas, one more supraorbital and one more temporal.  

There is an even stranger variant of this (and I gave credit for those of you who made this dx) called Parry Romberg Syndrome, which is linear scleroderma with regression of the underlying tissue, with hemiatrophy of the face.  Even the tongue is hemiatrophic! It is distinguished from Coup de Sabre by the involvement of the complete half of the face, as opposed to just the forehead, as in Coup de Sabre.  

"En coup de sabre" is a French term and means "of the blow of the saber" because it resembles a wound of a foot soldier who survived a blow to the head from a cavalry saber.  

Trichomycosis Axillaris

From the name of this entity, you would sure think this is a fungal infection of the underarm hairs, but it isn't.  It is a concretion of Corynebacterium bacteria which gives the unfortunate victim smelly underarms, stained tee shirts and a perplexing diagnostic dilemma.  The picture shows normal hair shafts with interspersed hairs that are thicker, slightly yellowish, and distinctly different from their surrounding cousins.  

This condition differentiates from white piedra, in that it is usually NOT nodular, which white piedra is.  The treatment for this condition is to shave the underarms and use benzoyl peroxide.  

BTW, saw a case today that you will see next week, after we get better pics.  It will blow you away!!

Cobblestoning from Cowden's Disease

Cowden's Disease is an autosomal dominant condition with variable penetrance known for a constellation of disease manifestations, all related to abnormal genetic expression, primarily through multiple hamartomas, which lead to its other name, Multiple Hamartoma Syndrome.

The skin findings include trichilemmomas (you should make sure all pts who have trichilemmomas on biopsy do not have Cowden's), oral cobblestoning, skin papillomas, as well as gut, neurologic and, most important, breast lesions and thyroid lesions.  Both can lead to cancer, with female breast cancer in greater than 75% of patients.

Several of you mentioned gingival hyperplasia, and the distingishing difference between this and gingival hyperplasia is that you will see overgrowth of the gums, particularly at the papillae, for hyperplasia, whereas in the cobblestoned mucosa you merely see papillomatosis without the overgrowth.

For those of you who nailed this: nice work.  It's rare, it's important and this is a pretty good example from which to learn.

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