What is erythropoietic protoporphyria?
The word ‘erythropoietic’ means associated with red blood cells (‘erythro-’) and their formation (‘-poietic’). The porphyrias are a group of uncommon diseases caused by something going wrong with the production of chemicals known as porphyrins. These chemicals are the building blocks of haem, which, when combined with a protein (globin), forms haemoglobin, the material in red blood cells that carries oxygen round the body. In the case of EPP, there is a build up of one of these porphyrins (protoporphyrin) in the blood, especially in the red blood cells. This leads to a sensitivity to sunlight.
What causes EPP?
An enzyme is a protein that helps to convert one chemical substance into another. In EPP, there is a shortage of one particular enzyme (ferrochelatase), which normally helps to convert protoporphyrin into haem by adding iron to it. As a result of this enzyme deficiency, protoporphyrin levels build up in the blood. As blood passes through the skin, the protoporphyrin absorbs the energy from sunlight and this sets off a chemical reaction that can slightly damage surrounding tissues. The nerve endings in the skin interpret this as itching or burning pain, and if the blood vessels are affected, they can leak fluid, causing swelling.
The light that protoporphyrin absorbs is different from that which causes ordinary sunburn. Usually sunburn is caused by the shorter wavelengths of ultraviolet light (UVB), but in EPP the skin is more sensitive to longer ultraviolet wavelengths (UVA) and to visible light.
Is EPP hereditary?
Yes, but there is not always a family history of the condition. Everyone has two genes for ferrochelatase in each cell in their body (one coming from their mother and one from their father). In most families, EPP occurs when an affected individual inherits a gene for a severely underactive ferrochelatase enzyme from one parent, and a less severely affected gene from the other parent. The less severely affected gene is quite common, being present in about 10% of the general population, but it never causes EPP by itself. The genetics is quite complex and advice from your local genetics service may be useful.
What are the symptoms of EPP?
Typically EPP starts with abnormal sensitivity to sunlight. Exposure to sunlight causes tingling, itching or burning, which may be associated with redness and swelling. These symptoms usually occur within a few minutes of skin exposure to sunlight, and often they take hours or days to resolve. During this time the skin may feel more sensitive than usual to extremes of temperature. The light producing these changes need not be direct – light reflected off water and sand, or passing through window glass, including car windscreens, can also cause the symptoms.
EPP usually starts in childhood, and affects males and females equally. Infants may cry or scream after being taken out into the sunlight; and older children may complain of burning and try to wave their hands in the air, or put them into cold water to try to relieve the pain. A very small number of people who have had with EPP for many years may develop liver damage. Fortunately this is rare.
What does EPP look like?
Despite severe discomfort, there may be nothing abnormal to see on the skin. Sometimes there can be swelling of the skin, initially like a nettle rash. With time, some people develop thickening of the skin over their knuckles, and small scars on sun-exposed skin such as that on the cheeks, nose, and backs of the hands. However these skin changes show wide variation between different individuals.
How is EPP diagnosed?
The diagnosis is usually suspected from the story, and can be confirmed by a blood test. This measures the amount of protoporphyrin in the blood (serum protoporphyrin) and in the red blood cells (erythrocyte free protoporphyrin). Some doctors will also ask for a stool sample to measure the level of protoporphyrin in the faeces. No urine tests are relevant to this condition except to exclude other types of porphyria.
Although it is unlikely that you will develop liver problems as a complication of EPP, your doctor may monitor the way your liver is working by yearly blood tests. If there is any evidence of a deterioration in liver function, there are certain interventions that may help to halt or reverse this.
As EPP affects the production of haemoglobin, it is not uncommon for people with EPP to be slightly anaemic. Your doctor will probably also measure your blood count to make sure that you are not becoming too anaemic.
Can EPP be cured?
At present there is no cure for EPP.
For information on available treatments please go to this page on the website of the British Association of Dermatologists

How Do You Protect Your Skin: Revolutionary New Lotion Solves Age-Old Problem

With all the over-the-counter and prescription products available for treating a wide array of skin care conditions, most people are not aware of which dry skin treatment lotions are the best for one's skin.

BEVERLY HILLS, CA, February 02, 2013 /24-7PressRelease/ -- With all the over-the-counter and prescription products available for treating a wide array of skin care conditions, most people are not aware of which dry skin treatment lotions are the best for one's skin. Hundreds of new lotions come out onto the market each year; most dermatologists don't actually know the ingredients that go into them or the actual tests that were done to support the healing claims. There are only a few points that consumers need to be on the lookout for, when deciding about proper dry skin treatment: #1. Using the wrong lotion can cause dry skin Some conventional lotions contain heavy artificial moisturizers and dangerous synthetic chemicals. These lotions coat the skin in a greasy film, giving the illusion of moisture, while they are actually damaging the way a person's skin functions naturally. Artificial moisturizers trick skin into thinking that it has enough moisture, causing a signal for the skin to stop producing its own natural oils. A revolutionary discovery in the field of skin care, Skin MD Natural Shielding lotion, will not cause this to happen; it works with the body's skin and not against it. #2. The best way to heal dry skin is to strengthen the skin's own natural barrier There is only one type of lotion designed to work with the skin and restore the skin's barrier - a Shielding Lotion. Dr. Brain Zogg, a board-certified dermatologist comments, "Shielding lotions are lotions that form a protective shield with the outer layer of skin and help keep out irritants and chemicals. They also allow the natural oils and moisture to remain in the skin so it can heal better. It is a big advantage that shielding lotions are light and go on without a greasy mess. Your skin needs help to keep out these moisture-robbing irritants. A fundamental difference between Shielding Lotions and traditional lotions are that Shielding Lotions enhance the skin's own natural protective abilities. Shielding Lotions turn the outer layer of skin into what could be described as a hydrating invisible shield, making them many times more effective than mere protective lotions and moisturizing creams." #3. Most skin conditions are cause by the overwhelming amount of chemicals skin comes into contact with daily Shampoos, antibacterial soaps, cosmetics, fragrances, colorants and other irritants bombard and penetrate a person's skin, breaking down the skin's natural protective ability and strip away moisture. Limiting one's exposure to products containing unnecessary chemicals can help one prevent against dry skin and other skin irritations. Dr. Peter Helton, a board certified dermatologist, explains how one can protect their skin by using a shielding lotion: "The concept of a shielding lotion is relatively new to dermatology and skin treatment. It binds to the skin such that the environment is not allowed to penetrate and desiccate the skin, essentially forming an invisible shield around and throughout the skin just like an invisible glove would. This is important as it allows the moisture to be maintained within the body and disallows, stops any products from harming the skin, like cleansers, acids, and all everyday solvents that we come into contact with." A revolutionary discovery in the field of dry skin treatment, Skin MD Natural Shielding Lotion, has been found to help eliminate dry skin because of its unique properties. The lotion is the by-product of years of research into understanding the intrinsic properties of skin and the effects that modern-day chemicals have on the human body. Purchase SkinMD Natural Shielding lotion at any pharmacy in the United States or by calling 21st Century Formulations, Inc. located in Beverly Hills, Ca. They can be contacted at 1-800-540-4790 or can be contacted through their website: http://www.skinmdnatural.com, or emailed directly: jhsoeder@skinmdnatural.com 21st Century Formulations is a company dedicated to creating healthy skin care shielding lotions. For more information please contact 1-800-540-4790. --- Press release service and press release distribution provided by http://www.24-7pressrelease.com

Read more: http://www.digitaljournal.com/pr/1050350#ixzz2KKLXe175

http://www.digitaljournal.com/pr/1050350

Keloids

What is a keloid?
When a wound heals, it leaves a scar. A keloid is a special type of scar: one that grows too much and can even become larger than the original wound. It is not uncommon for surgical or injury scars to become a little lumpy (hypertrophic). A keloid differs from these in several ways:
A keloid can come up after very minor skin damage, such as an acne spot, or even if there has been no obvious damage to the skin at all.
It can spread outside the original area of skin damage.
It may last for many years.

Are keloids hereditary?
They can be - a tendency to get keloids certainly runs in some families.
What are the symptoms of a keloid?
Usually there are none; but some are tender, painful, itchy, or cause a burning sensation. The main problem is that their appearance may cause embarrassment. If they are very tight, they can limit movement at nearby joints.
What does a keloid look like?
Keloids look like exaggerated scars. They are raised above the skin around them and sometimes they are domed. They can extend beyond the limits of the skin damage that caused the scar to come up in the first place. They are shiny and hairless; usually they feel hard and rubbery; and new ones are often red or purple, becoming browner and sometimes paler as they age. Most people with keloids have only one or two. However some people have many, especially if they have come up after acne or chickenpox scars.
How will it be diagnosed?
Your doctor will be able to make the diagnosis of a keloid just by looking at your skin. No investigations are usually needed.
Can a keloid be cured?
It is unusual for a keloid to be cured after treatment. The main problem is that cutting a keloid out often leads to an even bigger one forming later in the same place.
For information on available treatments please visit this page on the website of the British Association of Dermatologists

Skin drugs being sold as bleaching cosmetics

Some unscrupulous traders are making a kill through selling wrong bleaching agents to unsuspecting Ugandans.

The Sunday Monitor can reveal that some of the drugs meant to be strictly sold by hospitals and licensed clinics for the treatment of skin diseases, are now being promoted by traders as cosmetics that can lighten dark skin.

For instance, creams such as Diproson and Betasol can now be found in local shops in the country.

Dr Sam Opio, the Secretary Pharmaceutical Society of Uganda, said the creams are “only supposed to be used under prescription of a doctor for the right quantity and duration for it to be applied”. The medical officer said these creams were used to bleach leather material.

Kikuubo flooded with cosmetics
This newspaper found these creams in cosmetic shops around Kampala being sold by shopkeepers. They were found in nearly all cosmetic shops in Kikuubo, a retail corridor in Kampala, and also in some suburbs like Namuwongo. Diproson cream goes for Shs1,500 while the liquid one costs Shs2,500. Betasol, on the other hand, was a little more expensive at Shs3,000.

The low prices mean a better part of the population is able to access these creams and use them. In fact, Betasol was scarce because stock had run out, according to the shopkeepers.

However, abuse of these drugs can cause the skin to crack, exposing one to several skin disease and even cancer.

“These drugs being steroids are anti-inflammatory and are supposed to be prescribed for skin rushes, itchiness or irritation. Using steroids without specification lowers the body’s immunity system as some of them are absorbed into the skin and lowers its ability to fight infections,” Dr Opio said, adding, “For example, even a simple cough may refuse to go away or take very long to cure.”

Other harmful ingredients in skin products that people should be aware of, according to Dr Opio, include; Mercury and Hydroquinone. These have been linked to blood diseases, muscle weakness, acne and many other skin disorders.

Although not qualified to give prescriptions, shopkeepers go ahead to give instructions on how to use the creams, well aware of their bleaching aspect. One shopkeeper, without specifying the quantity, for instance, said a reasonable amount of liquid Diproson can be mixed with ordinary lotion to achieve a desired lightening effect. Another told this reporter to first apply a layer of diproson tube on the skin before applying any other lotion to get good results.

According to the Uganda Revenue Authority Enforcement Performance report for July to December 2012, some 2,448 tubes of Diproson and Betasol creams have been intercepted in the past two years.

Leaflets of the two creams indicate that they are supposed to be used to treat Eczema, Psoriasis, Lichen simplex, contact dermatitis and other skin diseases.

A source at National Drug Authority said they at times ambush these retailers in Kikuubo and impound the cosmetics but are currently looking for their suppliers.

http://www.monitor.co.ug/News/National/Skin-drugs-being-sold-as-bleaching-cosmetics/-/688334/1682828/-/7a1aiz/-/index.html

Uncommon skin disease has no uniformly effective treatment

Very often we are too uncomfortable to ask our healthcare providers questions about our own health. Sometimes we ask the question and do not understand the answer or don't want to challenge the answer given. Recognising this, Body & Soul has partnered with Johns Hopkins Medical Centre to provide you with answers to questions you have about your health.

If you have such questions please e-mail them to gazettehealth@yahoo.com and we'll have an expert from Johns Hopkins answer it.

Body & Soul will feature a question with its answer once a month. To protect your privacy no names will be published.

The question for this month was answered by Ginette Hinds of the Department of Dermatology at Johns Hopkins Bayview Medical Center. Dr Hinds was assisted by Natalia Vlassova of Johns Hopkins Mercy Hospital.

Q: I often develop boils in my armpits and groin area. I have no idea why this happens. I thought it might be because I am overheated, but the boils occur even in cold weather. What causes them?

A: The boils in your armpits and groin area are most likely the result of an uncommon skin disease called hidradenitis suppurativa, an inflammatory skin condition that usually begins after puberty and is more common in females than males. HS is characterised by frequent boils in the places you mention, and also on the buttocks, and they tend to leave scars. The boils can be very painful as they enlarge, but once they “come to a head” and leak their contents, the pain subsides. Some people with HS also have severe acne, cysts on other areas of the skin and painful boils on the scalp.

Q: What causes HS?

A: HS is caused by inflammation of the hair follicles that reside deep in the skin. Although scientists do not yet know exactly why this inflammation occurs, they do know that HS can run in families, and that obesity, sweating, smoking and stress can aggravate it. HS is not caused by poor hygiene, and it is not contagious.

Q: How can you prevent HS flare-ups?

A: There is no cure for HS at this time, but here are a few things that can help minimise the frequency and severity of the boils:

l Stop smoking

l Lose weight

l Avoid tightfitting clothing, antiperspirants and shaving if you notice that they incite flare-ups of the condition.

Q; How is HS treated?

A: There is no uniformly effective treatment for HS, and physicians will likely try different therapies depending on the severity and extent of disease.

Common treatments may include hot compresses applied to the boils; antiseptic washes with chlorhexidine or benzoyl peroxide, topical and systemic antibiotics, zinc supplements, steroid injections into the boils, laser hair removal and surgery to remove the boils.

HS can be debilitating and can cause embarrassment and social isolation. It's important to discuss symptoms and treatment options with your physician. There is still a lot to be learned about this disease, and several research studies are being conducted by Dr Hinds. Johns Hopkins Bayview also hosts a HS support group that meets three to four times a year. For more information on the support group meetings e-mail stovecat@yahoo.com.

http://www.royalgazette.com/article/20130129/ISLAND05/701299999

Sensitive skin problems aren't skin deep

SINGAPORE: Most might assume that sensitive skin is mainly due to in an intake of the wrong food, or the application of the wrong product. However, the main reason for sensitive skin lies on the surface of the skin, and what that layer is subjected to from the daily environment. In a sensitive skin survey held by Curél, the dermatological research oriented brainchild of Japanese chemical and cosmetics giant Kao, almost half of the respondents from various countries claimed to suffer from sensitive skin. And within this number, Singaporeans make up a whopping 70 percent. Part of the reason lies in Singapore's tropical climate –from wet and rainy, to sunny but humid – coupled with hours spent in drying air-conditioned environments whether at work, home or even shopping. More precisely, as explained by Curél's skincare expert Koicihi Ishida, skin in the Singapore environment is subject to "enhanced blood circulation and skin dryness due to the rapid change of the temperature and humidity within a short time frame". "At high temperatures and humidity, skin cells swell when sweat is produced, reducing the skin's barrier function" he explains. "On the other hand, low temperatures and a humidity level of 50 percent or less will cause the skin's moisture to decrease directly and become dry. Either way, the skin becomes sensitive and easily irritated." In other words, the root of the problem of sensitive skin isn't skin deep. In fact, it lies with the outermost layer of the skin, known as the stratum corneum (SC). It is the first barrier between the skin and the external environment, and offers protection to the underlying tissue from infection, dehydration, chemicals and physical stress. The top layer stratum corneum is largely held together by ceramide. According to Curél, about half of all sensitive skin cases is due to the SC's impaired function, making it the main cause, as well as solution, to various sensitive skin conditions. As skin expert Ishida points out, sensitive skins possess low levels of ceramide, a key element in the SC. In developing a skincare range that targets sensitive skin, Curél therefore focused its attention on ceramide and replenishing the skin's supply of this intercellular lipid. Tackling the problem from within also helps, with an intake of naturally occurring ceramide in food stuffs such as soya beans, spinach, rice, wheat flour and the high-fibre, jelly-like konjac or konnyaku, as the product is known in Japan where it's often served in oden or as noodle strips. Ceramide works by fusing with the skin's moisture and oil to prevent moisture loss while holding skin cells together, similar to a brick and mortar structure that creates a barrier function for the SC. With ceramide care, sensitive skin is directly treated to improve and replenish the impaired SC barrier which will in turn suppresses the sensitive symptoms and conditions, making the skin more resistant to external irritants. But this is not a one-hit, one-week solution as the experts will point out. The newly recovered skin can revert to its troubled state without proper maintenance, or in the case of more serious skin conditions such as atopic dermatitis, where ceramide drops to its original low state. The Curél skincare range for sensitive skin runs a gamut of applications, from face and body to scalp and hair, and everyone from adults to babies, are supposed to be able to use the products. How could so that be so? "Although the two skin types may differ in thickness, appendices and sensitivity, the fundamental structure and function of the skin is common for the young and old of both sexes," explains Ishida. "For the elderly, they have dry skin due to old age and it's usually accompanied with itchiness, which can be seen especially on the lower thigh area. In many cases, their SC's barrier function is damaged." The same concept of reinforcing the SC's protective function applies to babies and the young, as the risk of sensitive skin is in fact higher in the young, than when a person ages. "After birth, a baby's skin tends to be influenced by the environment and thus its skin condition might worsen as it is naturally delicate and sensitive," Ishida points out, while adding that "it is proven by the mild skin care from an early stage that a baby can maintain the healthy skin after growing up." "Therefore, it is important to have a skincare regime from an early stage to prevent skin issues during their growing up process" he advises. So even if you don't suffer from sensitive skin, there's little reason not to protect your skin the way it has been protecting you all this while. -CNA/fl

Darier's Disease

What is Darier’s disease?
It is a rare inherited skin condition, in which the skin in certain areas develops large numbers of small brownish warty bumps.
What causes Darier’s disease?
In the outer layer (epidermis) of normal skin, the skin cells are held together like bricks cemented in a wall. In Darier's disease the sticky junctions that hold the skin cells together are not made properly, and the skin may become scaly or lumpy or even form blisters.  It is not due to an allergy and it is not contagious (catching).
Is it hereditary?
Yes. It runs in certain families, being inherited in a pattern known as dominant inheritance. This means that there is a 1 in 2 (50:50) chance that each child of an affected parent will inherit the condition. It affects both men and women. Its severity may vary considerably within a single family; and if a person is badly affected it does not necessarily mean that other family members who inherit the condition will also get severe disease.
What are the symptoms of Darier’s disease?
Itching is very common.  The affected skin may smell unpleasant, particularly in moist areas. This is probably caused by increased numbers of ordinary skin bacteria growing in the affected skin. The appearance of the rash, and its smell, can be embarrassing.
A quarter of patients notice that the condition improves as they get older. Some people find that the sun causes their Darier’s disease to flare up. Some women notice that it worsens around the time of their periods.
What does it look like?
The first signs of the condition usually appear somewhere between the ages of 6 and 20. Little brownish, rough-topped bumps develop on the skin. The severity of the condition varies a lot and is unpredictable. The rash is often on the chest, neck or upper back at the start but warty bumps may occur on any part of the body including the skin creases and skin under the breasts. It is unusual for people to have much trouble on the face except for the skin on the forehead near the hairline.
The fingernails are usually affected. They tend to be rather fragile, split easily and look as if they have been bitten or appear dirty. There may be very obvious long red or white lines running the length of the nails. Nail changes and/or flat "warts" on the backs of the hands are often present in childhood, well before there are any other skin changes. Pits or small areas of hard skin occur on the palms of the hands and less often the soles of the feet. Occasionally there may be small spots inside the mouth and these may give the roof of the mouth a rough feeling.
How will it be diagnosed?
The diagnosis can often be made on the appearance of the rash and the fact that it runs in families.  To confirm it, a small sample of skin (a biopsy) can be removed under a local anaesthetic and examined under the microscope in the laboratory.
Can Darier’s disease be cured?
No, there is no cure, but there are many ways of helping it.
For information about available treatments please visit this page on the website of the British Associaton of Dermatologists

 http://www.britishskinfoundation.org.uk/SkinInformation/AtoZofSkindisease/DariersDisease.aspx