Psoriasis... is it contagious?

Today, my first day of a Pediatric Dermatology rotation, I was struck by the significant lack of public awareness about psoriasis. Almost 1 in 50 people have psoriasis. 125 million people worldwide have this condition, and everyone must know someone (if not a few someones) with psoriasis. Yet two patients came in on the same day requesting notes for their teachers stating that their skin condition is not contagious. If there is any way to make a shy adolescent who has so much psoriasis that he wears a baseball cap, long pants, and long sleeve shirts in the Texas heat any more uncomfortable, it is to imply that other kids may not be safe around him. The teacher did not trust either of these students' or their parents' retelling of the diagnoses. These kids know what they have because they are reminded with every pill they take and every ointment they use, not to mention the physical discomfort they endure.

I read this article talking about a drug-company sponsored public health website with a great section onpsoriasis. It is well done and informative. There are many other great websites out there with reliable information, including the National Psoriasis Foundation. I hope you take a few minutes to learn a little more about psoriasis.

Pressure sores in the WSJ

The Wall Street Journal talked about the measures hospitals and nursing facilities are taking to prevent pressure ulcers in this article. They are using fancy mattresses, playing music every 2 hours to remind nursing staff to turn the patients, and doing thorough skin checks more often. This is great news for patients, as ulcers are slow-healing, easily-infected, and preventable. They are also a significant part of the Medicare budget. But not for long. Medicare and some private insurers are slowly implementing a plan to stop paying for preventable conditions, such as pressure sores. I hope that they plan to increase compensation to facilities for the added cost of increased man hours and equipment. These are necessary and important measures to maintain a patient's quality of life, whether or not the improvement in care is cost-induced.

Do you have cancer? (Alopecia Areata)

A lot of kids with alopecia areata get asked that question. Why else would a child be losing his or her hair?
Alopecia areata is an autoimmune skin disease where your lymphocytes attack your own hair follicles. This results in patchy loss of hair all over the body, including head hair, eyebrows, arms, and legs. It is usually a disease of childhood and presents as round patches of smooth hair loss. For most people, the hair grows back and another patch may appear somewhere else. Eventually, most kids grow out of it. But for some, it progresses to alopecia totalis (complete loss of head hair) or alopecia universalis (complete loss of body hair).

Treatments include topical or intralesional steroids, but these are not cures and do not have great efficacy rates.

Because AA is not known well among the public, this can be a difficult disease for kids and adults alike. There were a number of campers with AA last week, and they had lots of stories about being stared at in public, kids making fun of them, and people asking them if they had cancer. They discussed different hair accessories (like hats and bandanas) to cover up their heads. For this group of campers in particular, I think it is therapeutic to be around other kids like themselves and to talk about they're good days and bad days with AA.

The National AA Foundation has a great website, check it out.

Whole-body peel (Erythroderma)

Non-bullous, Congenital Icthyosiform Erythroderma (CIE) is caused by an autosomal recessive hereditary deficiency of an enzyme involved in skin regeneration. The mutated or missing enzyme varies depending on which type of CIE the patient has, but it always leads to a build up of thick, dry skin. Either the patients are regenerating skin too fast or they are shedding epidermal cells too slowly. The transglutiminase-1 gene, the 12R-lipoxygenase gene, or the lipoxygenase-3 gene may be affected.

The patients appear red with partially adherent white scales. They are at high risk for infection because of the fissures they get in the thickened skin. Also, the thick skin can make it difficult to move joints, to the point where patients can end up in wheelchairs because their skin is just too tight and thick.

This is an incredibly difficult disease for kids and their families to deal with. It requires diligence to keep the kids well-moisturized and clean, but also the emotional effects of this disease are huge. Essentially, these kids are always peeling. Their beds and clothes are peppered with scaled off skin. There is also an odor to the hyperkeratotic skin. Patients are prone to overheating and alopecia (loss of hair).

Unfortunately there is no cure. Patients can only treat symptomatically by moisturizing and keeping their skin as supple and clean as possible.

There seems to be a lot of research done on prenatal testing for CIE. Skin samples can be taken of the fetus that are then analyzed for hyperkeratosis, particularly around hair follicles. Parents have even terminated pregnancies based on the positive diagnosis. The study below found that it is difficult to judge the presence or absence of the disease based on skin samples for two main reasons: 1. the random sample may be from an area that is less affected (as was the case in one of their patients) and 2. normal epidermal growth does not occur until the 24th week, at the end of the 2nd trimester

Holbrook KA, Dale BA, Williams ML, Perry TB, Hoff MS, Hamilton EF, Fisher C, Senikas V.The expression of congenital ichthyosiform erythroderma in second trimester fetuses of the same family: morphologic and biochemical studies.J Invest Dermatol. 1988 Dec;91(6):521-31.

OMIM has a nice summary of the disease.

A world without sun (Xeroderma pigmentosum)

Xeroderma pigmentosum is a hereditary deficiency in part of the nuclear repair enzymes, inhibiting the body's ability to repair damage caused by UV rays. UV rays cause pyrimidine dimers to form in our DNA and endonucleases, DNA polymerases, and ligases work to repair the damage. If any of these enzymes are decreased, ineffective, or all together missing, the DNA will not be repaired properly. Increased DNA mutations lead to increased risks of malignant growth.

This past week I had the chance to hang out with a kid who has XP and take a small glimpse into her world. She spends all of her time between 7 AM and 8 PM in doors. If she had to go outside, she would suit up in a jacket, jeans, close-toed shoes, gloves, and a hat/hood that looked like a bee keeper's hood but it was opaque. In the 90+ degree heat, this was less than comfortable. She was diagnosed as a baby when, after 10 minutes outside in the shade, she was severely sunburned. Ever since then, she has lived in this inside world. I can't imagine it is easy being a kid with all that pent up energy and not being allowed to run around outside or play with the other kids. But it is a necessity. These kids can get skin cancer from a very young age and the only protection is UV avoidance.

Wikipedia has a nice list of literary and film references about people with XP. Also, the XP family support group has tips on how to protect yourself against UV damage. From special clothes to window tints to avoiding sun at airports, theme parks, etc, the support group has lots of interesting strategies for avoiding the sun.

CAMP

I was a camp counselor for a group of kids with skin disorders last week (hence the lack of posts). As an ode to my campers, I'm going to do a few days of posts dedicated to their skin conditions and address how these conditions have affected their lives. Middle school is a tough time as is, but when you have a disease that everyone can see, a tough time can become tougher. These girls are remarkably mature, caring, and sensitive, and it was a pleasure getting to know them.

I think people would be less judgmental if they understood what kinds of conditions kids like these have, and hopefully this series of posts will bring together some useful information and helpful links.

Who goes to the dermatologist?

Rising Skin Cancer Rates Are More Likely To Affect Wealthy People, Says 12-year Review

I read this article in the Science Daily, and the title and following quote intrigued me.

Women living in affluent areas were 29 per cent more likely than people living in disadvantaged areas to suffer from basal cell carcinoma and nearly two and a half times more likely to suffer from malignant melanoma.

Men displayed a similar pattern. They were 41 per cent more likely to suffer from basal cell carcinoma if they lived in an affluent area and two and a half times more likely to suffer from malignant melanoma.

The article referred to a study published last month.

Hoey et al. Skin cancer trends in Northern Ireland and consequences for provision of dermatology services. British Journal of Dermatology. 156, pp1301-1307. June 2007.

I found these statements particularly interesting as I am currently reviewing data for a local Moh's surgeon, and, as I ran through the data, I wondered about the socioeconomic effects on treating and diagnosing skin cancer. There are a number of day laborers working in industry in this area who are contract employees and likely do not have particularly good health insurance. I'm guessing that, even though they are high-risk for sun damaged skin, going to the dermatologist for yearly skin checks is not high on their priority lists. I wanted to look at the socioeconomic status versus likelihood for multiple lesions or recurrence rates. But this population is so hard to access because they simply do not visit the dermatologist. And, if they do, it is at a late stage, and they are less likely to follow up for regular skin checks, so recurrence rates are difficult to record.

So any study using data from patients who voluntarily went to the dermatologist is naturally skewed towards more affluent patients. Even in Ireland, where the majority of citizens are covered by public or private health insurance, this does not imply equivalent care. There are still both public and private health care options, and I cannot help but assume that public health care options are harder and more expensive to access. Working in a public hospital in the U.S., one realizes how difficult it is to even get an appointment with a specialist such as a dermatologist.

Anyways, my point is that statements like this are very misleading. After reviewing the article, it appears that the researchers divided their already-diagnosed-with-skin-cancer sample population based on their economic districts. Of course there will be fewer people from the poorer neighborhoods. Of course more affluent people have more time and better access to healthcare, causing their increased diagnosis of skin cancer. We need a study comparing incidence rates of skin cancer amongst varying socioeconomic classes. That would be a much better representation of the effect of affluence on skin cancer rates.