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DSAP - Disseminated Superficial Actinic Porokeratoses (porokeratosis)
LAST UPDATED: 1/31/2016
DSAP is such an uncommon skin disorder that there is very little information available on it. I am not a doctor, but I've learned that amazingly (and sadly) the majority of Dermatologists haven't even heard of DSAP or just plain can't recognize DSAP. General practice doctors? Forget about it! I found this out the hard way, learning 6 years later that I had some of these genetically predisposed spots that are brought on by sun and/or ultraviolet exposure, and in some cases possibly by some sort of relaxation of the epidermal immune system. Amazingly I had visited one family physician and four dermatologists over the years before I finally got the correct diagnosis. These various doctors misdiagnosed it as a fungus, "nothing", seborrheic keratosis, folliculitis, actinic keratosis (AKA solar keratosis). One of my dermatologists amazingly did a biopsy for cancer before concluding that my skin condition was "nothing". Dermatologists most commonly misdiagnose DSAP patients as having AK (actinic keratosis). I received one email from a patient who was even misdiagnosed as having lupus of the skin! I have even heard cases of psoriasis and SCLE being mistaken as being DSAP. In my case the one doctor who finally correctly diagnosed me was actually unsure whether I had actinic keratosis or DSAP or a combination of both. I think it's a major error to be misdiagnosed as having actinic keratosis because AK is a much more serious (precancerous) condition.
After having misdiagnosed or even after having correctly diagnosing a DSAP patient, doctors will often put the patient on a topical medication called Efudex (AKA Fluorouracil, Fluorouplex, Carac), which only makes DSAP look horribly red and irritated for many weeks during treatment and then for a few months afterwards, while really doing nothing to improve the skin condition. After reviewing many emails and guestbook posts I have come to my own conclusion that Efudex is an aggravating waste of time when it comes to "treating" DSAP. This medication was designed for AK (Actinic Keratosis) - Not DSAP. Carac is the newest variation of Efudex. I am skeptical as to whether to expect anything different from Carac.
Based on email reports from patients, I am of the opinion that you should also avoid any other medications that cause a similar harsh, red reaction (such as Tazorac, Retin-A, and Aldara). I have not received one single report of these meds clearing up DSAP. I really don't know why doctors insist on prescribing these meds unless there is a suspicion that a patient might have AK or a combination of AK and DSAP. Also I say avoid Levulan light therapy or "Blue Light" therapy. I've had four reports on this treatment and they were all negative. Based on years of feedback from patients I have concluded that at this point in time there is no medication (topical or oral) that is really worth it for this condition, although Diclofenac sounds potentially promising.
Important discovery made at Anhui Medical University in China
NEWS STORY - Sept 16, 2012 - A team of researchers at Anhui Medical University in China and BGI has found strong evidence that there is a link between mutations of the mevalonate kinase gene (MVK) and DSAP. It is a major step toward discovering the genetic mechanism that causes DSAP, and sheds light on its further molecular diagnosis and treatment. According to Tao Jiang "Our study provides new insights into the pathogenesis of DSAP, and the identified MVK mutations offer the best candidate targets for gene diagnosis and clinical treatment of the disease". According to Xuejun Zhang, President of Anhui Medical University, this study "provides scientific basis for revealing DSAP pathogenesis, genetic counseling, risk prediction, prenatal diagnosis, new drug development, clinical diagnosis and treatment." Let's hope that this leads to new drug developments!
Welcome side effect from chemotherapy
I have received two reports from DSAP patients who were delighted to see their DSAP spots "shed off" as a side effect from being on chemotherapy, but both reported that DSAP simply grew back a year or two later. Accordingly this makes me skeptical whether ANY treatment can hold up over the long term. Getting rid DSAP spots (if at all possible) is certainly not as simple and easy as burning off a common wart.
NEW: Diclofenac sodium topical gel 1%
For what it's worth, I received two emails from patients who were prescribed the topical anti-inflammatory drug Diclofenac. One was very pleased with results using 3% Diclofenac, but when they ran out of medication after just a few months their condition "got bad again". More recently someone emailed me saying that Solaraze did nothing. So I am very skeptical of this topical drug.
I am currently testing out this medication on some test spots. After 4 months it has done nothing. This leads me to believe that reports of it "working" actually may be a case of it working on spots of actinic keratosis -- not dsap. Sometimes doctors confuse the two, or patients may have a combination of the two conditions. I don't know. Nevertheless I am trying to gather more reports on this drug from others who may email me. The other email was from someone who says that they visited a dermatologist who looked at their DSAP and declared "I have a cure for that". Keep in mind that doctors routinely prescribe Efudex when (in my opinion) Efedux is an aggravating waste of time for reasons discussed earlier. Of course this patient immediately had skepticism. This doctor has apparently prescribed Voltaren Gel to be used once in the evening for 6 months to a year. That's a long time but there is not supposed to be any harsh reaction, like you would experience with other meds like Efudex. This doctor believes that DSAP is somehow rooted in inflammation, hence the use of Voltaren, which is an anti-inflammatory. If anyone else has any stories to report on Disclofenac for DSAP then PLEASE email me. Again I am in the very early stages of gathering reports about this med. My main concern is that if this med works at all, it might just temporarily suppress the condition (much like the chemotherapy story above), although for some this might be welcome even if they have to continue using Diclofenac. I am told that Diclofenac is $150 per tube with insurance --or $800 without! However another patient living outside the country reports that Voltaren costs only 10 USD. Upon further research I've found Mexican online pharmacies that charge only $25.75 and probably don't require a prescription. Another from Bulgaria sells Diclac 5% on Ebay for about $18 including shipping. Speak to your doctor about using Voltaren.
Dovonex / Tacalcitol / Calcipotriol
There are two reports of DSAP patient's skin clearing from using Tacalcitol and from Calcipotriol. Unfortunately in all of my years of running this site and receiving emails from patients, I haven't ever heard of one single patient ever having success from using topical D3 analogs like Tacalcitol, which is usually prescribed for psoriasis. In the USA Calcipotriol is sold under a different name called Dovonex. I will say that it can't hurt to try Dovonex or Calcipotriol because (unlike drugs like Efudex) there is no harsh reaction from using this topical cream.
Avoiding both the sun and harsh medical treatments may be your best course of action
One thing that really does help for DSAP improvement over time is simply using the strongest sunscreens and avoiding direct sunlight as best you can. I promise you will see improvement! This is especially true when you consider that when patients are first diagnosed with this condition, cosmetically it seems to be at its very worst. Often this is because patients had been hanging out in the sun with little regard for the need for strong sunscreen. Also avoid all of the harsh treatments described on this site. They will only cause additional cosmetic setback. I believe that some of the redness of DSAP is maybe a bit like the knees you scarred as a kid. It can take years for that redness to improve once you commit to giving your skin a chance to "recover". And when combined with some creative use of sunless tanners you can actually find yourself looking pretty normal again.
Lack of sun exposure has been linked to high blood pressure
As a note of warning, you really need to get some sun exposure for the vitamin D benefits as well as other benefits. Sunlight alters levels of the messenger molecule nitric oxide in the skin and blood, which in turn reduces blood pressure.
All of this doesn't mean that you should be getting sun on areas that are affected by DSAP, such as arms and legs. Your back is a nice large area to soak up lots of ultra-violet rays. You don't want to burn either. Visit this page for information on sun exposure.
Also take vitamin D suppliments.
Current "treatment" for DSAP is only said to be suppressive or palliative (to try to make appear less offensive) rather than curative. Most patients (especially myself and those with light complexions) will argue that these harsh "palliative" treatments (like Efudex, lasers, freezing, and others) are a waste of effort and will worsen your appearance short term and may even worsen your long term appearance. I have however read and received reports of people's varying degrees of improvement from deep freezing and from using laser treatment, but for every positive report I get a negative report from patients who tried freezing but wound up looking worse or no better long term. Accordingly save yourself the aggravation; If you decide to venture down the freezing or laser treatment route I suggest trying only a test spot or two to start. Read some of the guestbook posts in this site BEFORE trying any treatment. Email as many people as you can with questions. Again, people with very fair skin might be best suited to just avoid freezing or laser treatment.
My gut feeling is that the only real DSAP treatment that will ever really work will emerge from an off label use of medication(s) intended for psoriasis and/or actinic keratosis, or emerge someday from proteonics (protein expression) or stem cell therapy. Hopefully more studies like the one in China continue. One of the first hurdles in getting research under way is finding patients to donate DNA samples. Fortunately as a result of maintaining this page for over ten years I already have contact info for many DSAP patients. I would expect that a good number of these people would be more than happy to help in research studies if one ever materializes.
DSAP is considered a rare disorder so most dermatologists have little (if any) experience with "treatments". Therefore it would be wise to do your OWN foot work and second guess your doctor's "treatment" advice, especially if they want to put you on a harsh treatment like Efudex, or chemical peel or extensive freezing treatment without trying test spots first and waiting several months. When first diagnosed, DSAP patients are often desperate to "try anything", and doctors feel compelled to at least "try something", however the best path may just be to simply make an effort to avoid the sun, wear strong sunscreen and take no medications at all, or maybe try "test spots" with various medications and/or freezing. Ask your doctor how many patients he/she has worked with. Just how knowledgeable is he/she? Does your doctor even know what DSAP is? It is YOUR OWN responsibility to find a knowledgeable doctor! Based on my experience there are very few who know their field inside and out. I believe that you might be best off finding an older (experienced) doctor who has practiced a long time and works out of an office with perhaps other dermatologists from whom they can get 2nd opinions. DSAP mainly affects people of European decent (white people) so perhaps you might get better diagnosis from a Caucasian doctor. There seems to be too little knowledge on the disorder. Much of the knowledge that IS out there is clouded and confused. But after reading this page YOU might become more knowledgeable on DSAP than the vast majority of dermatologists! I have sorted through the mess (by scouring the net, talking to some doctors and many patients) and finally created this page to share my experience, and for all of us to gather info from around the world about DSAP. Bookmark www.dsap.net and periodically check back for the latest DSAP news.
Some facts about DSAP:
Analysis of DSAP shows altered DNA ploidy, chromosomal instability, and abnormal over-expression of tumor suppressor p53 protein.
DSAP is characterized by annular (round-ish), dry patches (or lesions) that eventually become pigmented (red, brown) with continued sun exposure, and (from my experience) from scratching /picking, and time. In a perfect world doctors would be able to identify DSAP years in advance when only a few faint round outlines of DSAP are becoming visible, with no discoloration (or sometimes with even HYPO-pigmentation). Areas affected are usually sun exposed areas of the arms and /or legs. I've read about other areas of the body being affected such as the back and belly. It affects the LEGS of women more than men (I have to theorize that this may be from wearing dresses rather than from genetic reasons). In men it is said to affect the arms more (than legs). In about 15% of patients the cheeks of the face are affected, otherwise (oddly enough) the face is usually spared. If a male patient has keratotic spots on their arms and / or legs but NOT on their face, a savvy doctor will pick up on this (as more of an indicator of DSAP than Actinic Keratosis). But don't rule out that AK (or DSAP) may be less prevalent on a female patient's FACE due to habitually wearing makeup (which can contain or simply act as a sunscreen). Nearly 1/3 of patients experience minor itching. DSAP seems to be 3 times more likely in women. The average age at which patients start to see DSAP become prominent is about 40.
RIGHT: These are smaller DSAP spots. Perhaps a few years earlier these spots appeared as nothing more than very slight raised borders (with NO discoloration) viewed only up close by a discriminating eye against a backdrop of light, and dry skin. The difficulty in getting early detection is that DSAP doesn't appear with hyperpigmentation (redness) until years after the accumulated sun exposure. People who have been so lucky as to get diagnosed very early on (before there is hyper pigmentation) really need to wise up to the fact that they need to make lifestyle changes and stay out of the sun starting NOW! There is a phenomenon about DSAP that I have noticed (and so have other patients who have emailed me) and that is that the redness of DSAP spots always seems to become prominent rather suddenly. Perhaps all in one summer it suddenly becomes a very noticeable cosmetic issue. Larger DSAP spots typically aren't as perfectly round as depicted in this photo shows.
In most cases DSAP is inherited, but sometimes people develop DSAP (or at least have very high susceptibility to develop DSAP) when their immune systems are not working well. Radiation therapy can severely exacerbate DSAP! You don't even want to see the horrific photos of DSAP lesions that develop after going through radiation cancer therapy.
A disservice paid to patients by dermatologists is the failure to inform DSAP patients about hereditary factors. It is vital that family members be informed if a parent (or sibling) has been diagnosed as having DSAP spots! In the late 1960's it was concluded by a researcher that DSAP acts autosomal hereditary. From my understanding of heredity, each offspring has a 50% chance of inheriting the DSAP gene if one parent has the faulty "DSAP gene". If both parents have the DSAP gene then I believe there is either a 50% or 75% chance of gene transmission. With these odds I'd say family members might like to know the risks! Keep in mind that a certain level of sun exposure is required before the lesions appear on the skin. For example: one may have the DSAP gene BUT live in long sleeve climate, or habitually avoid the sun, or habitually wear sunscreen thus NEVER developing DSAP spots or significant DSAP spots. DSAP is brought on by sun exposure BUT other factors may be involved. DSAP lesions have "thread-like" outer boarders, making them perhaps look sort of like craters on the moon when viewed at an angle against a backdrop of light. (Note: These "raised borders" are in actuality much smaller than sewing thread. Also the "thread-like" borders are much less noticeable when skin is wet from vitamin E, sun screen, etc. Skin should be DRY and oil free to really see the characteristic defined borders.) DSAP spots usually encircle pore(s) that are sort of "hardened", clogged, not functional, and stick up slightly. DSAP has been labeled as "pre-cancerous" but development of true skin cancer in DSAP is "uncommon" and in fact one study seemed to suggest that the only DSAP patients who developed cancer were those who very irresponsibly continued to be exposed to the sun. For most people DSAP is a cosmetic problem and a lifestyle changing problem but NOT a cancer concern. (Note: I have received emails from people who have had DSAP for 40 years and been cancer free.) DSAP compares with actinic keratosis in which some spots can develop into cancer within 6 months. I have heard talk of DSAP "remission" but it appears that those who develop DSAP slowly over the years do NOT experience remission.
I e-mailed a doctor who authored a web page that spoke of DSAP "spontaneous resolution":
MY QUESTION: Your article on DSAP reads "Protection from ultraviolet radiation may lead to spontaneous resolution." and "Lesions may resolve with discontinuation of ultraviolet exposure." I'm wondering just how rare or common this occurrence can be. Have DSAP patients seen their skin clear up?
RESPONSE: Yes, DSAP patients have seen their skin clear up after exposure to ultraviolet radiation is discontinued. I cannot give you a percentage for resolution. The reports of this occurrence generally have a clear causal relation, eg. fair skinned European who goes to the beach for 6 weeks, develops DSAP, which then resolves after protection from further ultraviolet exposure.
MY QUESTION: 6 weeks? OK. What about patients who develop DSAP slowly over the years. Is reversal seen in these instances?
RESPONSE: Improvement is seen, but not complete resolution. Reduced risk of malignant
degeneration is the major reason to protect from ultraviolet radiation.
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