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Frank Bures: Schamberg’s disease 2022: Progressive pigmentary dermatosis

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Maybe it’s time for a break from writing about COVID almost every week for 2½ years. This inspiration, if you will, came after meeting people out walking, a couple of whom asked, “Say can you take a look at something (on my skin)?” My reply always is “Sure,” because if someone has something bad percolating, like a melanoma, it should be seen as early as possible. These encounters over the years have taken place in virtually any setting you can imagine, even grocery store lines. What a discount.

Twice in the past few weeks, what was displayed for me (and the world) to see (discretely) was a condition called Schamberg’s disease, something not discussed in this space for at least 10 to 12 years. Here goes. Schamberg’s disease (SD), or progressive pigmentary dermatosis or purpura (bruising) is a modestly common, curious, and harmless condition worth being aware of, so that you don’t have to worry. It occurs as “cayenne” red pepper, pinhead sized spots in brownish patches usually on lower legs. It’s seen more often, but not exclusively, in “mature gentlemen.”

The blotches produce no symptoms, and do not cause nor signify any medical trouble. Nothing can be found wrong internally. It is all a skin issue. The only test to do is a biopsy (taking a piece out to examine microscopically), which should show inflammation of the uppermost skin capillaries by lymphocyte type white blood cells, and slight leakage of a few red blood cells into skin, which is called a capillaritis.

Since nobody feels anything with SD, it is usually noticed accidentally in its later stages, when the hemoglobin from the red blood cells has broken down into its protein and iron. The iron creates a pigment called hemosiderin. This produces the brownish-yellow pigmented patches with new red pepper spots at the margins as it slowly and silently enlarges. The iron tends to stay in skin on lower legs, and somehow stimulates normal pigment cells to make more brown, adding a darker hue to the color.

SD never develops injurious skin changes, such as internal inflammation or skin ulcers. No one has a clue why it exists. There is never any clotting, bleeding, or allergic source found. It is not associated with varicose veins! Any and all blood tests will be normal, if nothing else is happening as an undercurrent. For those elder statesfolks so blessed, it likely will last as a permanent fixture without harm. It just messes up their chances in the summer’s beauty pageant.

It actually can pop out anywhere, like arms, or less commonly on body, but never on the face. As a capillaritis, it can be seen at almost any age, from 9 to 10 years old until you become room temperature. Interestingly, Dr. Jay Schamberg, a well-known Philadelphia dermatologist, published his case report, allegedly the first, of a 15-year-old boy in 1901. In younger ages and at earlier stages, a shot of cortisone may clear it up completely. I have seen a 40-year-old woman with just that result in a return visit.

Otherwise treatment attempts just don’t work (excluding amputation). Schamberg entitled his paper, “A Peculiar Progressive Pigmentary Disorder of Skin.” Now, the label is more often progressive pigmentary dermatosis (skin condition) or purpura. It is almost not a disease because it causes no dis-ease, just dislike of the look.

About three other purely skin diagnoses share the capillaritis biopsy findings. Their long, confabulating names include purpura annularis telangiectoides described in 1895 by an Italian dermatologist, Majocchi; pigmented purpuric lichenoid dermatosis reported in 1925 by Gougerot and Blum (impressed yet?); and lichen aureus, sort of a localized SD patch.

A paper published in 1951 by Randall and others from the Mayo Clinic did a microscopic comparison of all these. They concluded that, because they all looked alike microscopically, they all had the same derivation. That has never been proven. Oddly, almost 70 years later that paper still holds sway, even though it really doesn’t seem likely. Nobody is forking over research money for that level of problem.

Suffice it to say for SD, the color of your lower legs may come from getting a little “rusty” (iron in skin, right?), but you needn’t worry about it, compared to the other “rust,” atherosclerosis, building up in the arteries in your legs. How about Rusty for a nickname? Next week’s Hint will likely climb back into the COVID cave.

Dr. Bures, a semi-retired dermatologist, since 1978 has worked Winona, La Crosse, Viroqua and Red Wing. He also plays clarinet in the Winona Municipal Band and a couple dixieland groups. And he does enjoy a good pun.


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