Tuesday, October 13, 2009

The Skinny on Autoimmune Skin

Image found here.

Unfortunately, I have had several opportunities to learn more about autoimmune related skin conditions recently, and I've been just itching (sorry) to share some of my newfound knowledge. A discussion about skin needs to begin with a brief anatomy review. Human skin is comprised of three layers. The excellent chart below is from the University of Maryland Medical Center.

epidermisThe epidermis is the thin outer layer of the skin. The epidermis itself is made up of three sub-layers:
  • stratum corneum (horny layer)
    This layer contains continually shedding, dead keratinocytes (the primary cell type of the epidermis). The keratin, a protein formed from the dead cells, protects the skin from harmful substances.
  • keratinocytes (squamous cells)
    This layer contains living keratinocytes (squamous cells), which help provide the skin with what it needs to protect the rest of the body.
  • basal layer
    The basal layer is the inner layer of the epidermis, containing basal cells. Basal cells continually divide, forming new keratinocytes and replacing the old ones that are shed from the skin's surface.
The epidermis also contains melanocytes, which are cells that produce melanin(skin pigment).
dermisThe dermis is the middle layer of the skin. The dermis is made up of the following:
  • blood vessels
  • lymph vessels
  • hair follicles
  • sweat glands
The dermis is held together by a protein called collagen, made by fibroblasts (skin cells that give the skin its strength and resilience). This layer also contains pain and touch receptors.
subcutisThe subcutis is the deepest layer of skin and is also known as the subcutaneous layer. The subcutis, consisting of a network of collagen and fat cells, helps conserve the body's heat while protecting other organs from injury by acting as a "shock absorber."

There's a lot going on in that thin layer of skin. What seems to be a just a fleshy layer of body wrapping paper is actually the body's largest organ, with a total surface area of about 20 feet. The skin protects the body from infection, helps regulate body temperature, and facilitates our senses of touch, heat, and cold. In its layers are glands that secrete body oils and sweat, and hair follicles.

About two months ago, I developed a healthy crop of some unhealthy looking spots on my skin. I called my doctor's office to schedule an appointment. What do the spots look like? asked the appointment secretary.

Um. I wanted to use medical terminology specific to skin splotches, but for the life of me, I couldn't remember any of those strange words. It's useful to know some of this terminology when reading dermatology literature. Here's an abbreviated list. You can read a much more detailed description in Skin Lesion Nomenclature from the University of Wisconsin found here.

A skin lesion, or patch of abnormal tissue, can be identified in two ways - by a description of the actual lesion, and by the way the lesions are arranged on the skin. Here are just a few of the most commonly used terms.
Lesion descriptive terms:

Macule: a small area of the skin that has a changed color and is perfectly flat.
Papule: a small solid raised lesion
Vesicle: a small raised and clear fluid filled lesion.
Pustule: a vesicle filled with pus.

Lesion patterns:

Annular: in the shape of a ring
Discrete: lesions that remain separate from each other
Clustered: lesions that occur in a group
Confluent: lesions that overlap or run together
Some skin conditions are autoimmune diseases that arise specifically in the skin. You can read more about these conditions, such as alopecia and vitiligo, here. Other skin problems can be caused by autoimmune diseases that affect the body as a whole, such as Sjogren's syndrome, sarcoidosis, and Lupus. You can read my previous post about sarcoidosis and it's skin rash, erythema nodosum, here.

SLE, or systemic lupus erythematosis can cause the skin to develop a malar rash - a red rash over the cheeks and nose often described as a butterfly rash - or discoid rash with raised, reddened disc shaped patches. This Lupus Foundation of America article is a great resource to learn more about SLE, cutaneous lupus erythematosis, and subacute cutaneous erythematosis, all forms of Lupus.

Sjogren's syndrome also can significantly affect skin. According to the Sjogren's Syndrome Handbook edited by Steven Carsons, M.D. and Elaine K. Harris, the most common manifestation of skin problems associated with Sjs is dryness of the skin. The authors recommend strategies such as the use of unscented body creams that do not contain alcohol, humidifying the home environment, limit the use of moisture-robbing soaps, and avoid lengthy showers and baths.

Aside from dryness, Sjs can cause other cutaneous (skin) issues. This info was taken from a very in-depth article about Sjogren's syndrome on the Medifocus Health site: *update 5/16/10 - Medifocus is now not allowing free access to this guidebook. It is available for purchase online* Grr. 

Estimates of the percentage of Sjogren's syndrome patients who experience skin problems range from 10-40%.Cutaneous vasculitis (inflammation of the blood vessels in the skin) is one of the most characteristic extraglandular manifestations of Sjogren's syndrome and is thought to be due to lymphocytic infiltration into the walls of the blood vessels. Typically, small blood vessels are affected more than large ones.

The most common forms of cutaneous vasculitis seen in Sjogren's syndrome patients (typically patients who have anti-Ro and anti-La antibodies) are:
  • Palpable or nonpalpable purpura (purple spots on the skin after blood "leaks" underneath; similar to a bruise) which can cause raised, red skin lesions
  • Urticarial lesions (hives)
  • Erythematosus micropapules - red spots
Patients with cutaneous vasculitis also may develop non-vasculitic lesions including:
  • Petechiae - pinpoint dots
  • Photosensitive cutaneous lesions - lesions which are sensitive to light (also seen in patients with systemic lupus erythematosus)
  • Livedo reticularis - marbled appearance of the skin
  • Lichen planus - small itchy pink or purple spots on arm and/or legs
  • Thrombocytopenic purpura - purple areas on the skin related to a decrease in blood platelets
  • Vitiligo - white patches on skin due to loss of pigmentation
Cutaneous vasculitis (as well as other forms of vasculitis - see below) is considered to be a significant prognostic indicator with the development of lymphoma and mortality. A large 2004 study of 558 Sjogren's syndrome patients diagnosed with cutaneous involvement reported that 58% of the patients had cutaneous vasculitis and showed a higher incidence of:
  • Peripheral neuropathy - damage to the nerves that supply the arms and legs characterized by burning, tingling, numbness and pain
  • Raynaud's phenomenon - a circulatory disorder caused by insufficient blood supply to the hands and feet
  • Renal (kidney) involvement
  • Presence of autoimmune markers such as antinuclear antibodies (ANA) and rheumatoid factor
  • B-cell lymphoma
Other skin problems that may be experienced by patients with Sjogren's syndrome include:
  • Dry skin - affects up to 55% of patients with Sjogren's syndrome (xerosis)
  • Itchy skin
  • Burning skin - may be experienced in up to 20% of patients with Sjogren's syndrome
  • Skin rashes - may be experienced in up to 10% of patients with Sjogren's syndrome
  • Raynaud's phenomenon - may appear before the development of symptoms of dry mouth and dry eyes by many years. It is estimated to occur in up to 30% of patients with Sjogren's syndrome and is usually of minor clinical significance.
Treatment of the above conditions can vary and depend on the severity of the condition. Successful management may include the use of topical agents, such as ointments and moisturizers, avoiding UV rays, and use of medications as prescribed by your physician. You can read more about treatment of skin problems related to Sjogren's syndrome here and here.


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Shelley said...

I have discovered your blog and I am grateful, mostly b/c I have had a very hard time putting into words what is going on with me. This particular post is of great interest to me b/c I have significant cutaneous vasculitis and I have peripheral neuropathy. I am 39 years old and received my formal diagnosis of SS at age 36. Anyway, as you can imagine, I am struggling with all the information regarding the lymphoma and more serious complications revolving around my type of sjogrens. Thank you for spelling out some things for me as I navigate this condition. If there is anything further you have read about regarding this please let me know.

Anonymous said...

I have for years had a maddening itch to my left arm that comes and goes and once it flares up can be insanely itchy for weeks.

I can't really see anything, but it runs down the outside of my arm along the muscles from my shoulder to my elbow.

I thought it might have something to do with sundamage since that is the area of arm that is exposed to sun when I drive.

But the itching is often when I'm wearing clothes over the area and only sometimes do I see one or two raised tiny bumps. It feels like "itching powder" and is virtually invisible.

I don't know why it's only right there that it is such a problem.

I figured it had something to do with SS.

Thanks for listing this blog to help out.

Unknown said...

Thank you for all this information about sjs... I was diagnosed at age 47 , 2 yrs ago although I was searching what was wrong with me for 10_12 yrs prior . now this skin conditions that seem to be coming out in the past yr. All make sense to me finally because of all your great information. Please keep me updated with any new news ty J schmidt

Louise On The Left (Coast0 said...

Can anyone tell me if remaining on a "hormone replacement therapy" helped relieve any skin dryness or itching issues for them?

I am a 52 yr old woman dx'd with Primary Sjogren's Syndrome for over 15 yrs. with Cutaneous vasculitis issues and an inexplicable series of bouts of invisible itching up and down my arms from time to time.

When I get it, nothing will relieve the itching. It's like I'm being stung by hundreds of mosquitoes, but only on the outer part of my arms from my shoulder to below the elbow. My rheumy thinks it's something to do with osteo arthritis in my spinal areas, but, I don't know. It comes and goes without rhyme or reason.

But, ever since I have gone into menopause he suggests I stay on a hormonal patch, but it's pricey and I worry about cancer issues. Has anyone done a hormonal treatment plan just to help with skin issues with SJogrens?

If so I'd love to hear your thoughts about that.



Summer said...

I have been reading through your blog all evening. It's amazing to have most of the things I have stumbled upon in the last year to be spelled out for me. At 28 years old I never expected to be disabled by the rapid fatigue or loss of feeling, a deep burn other days, in my hands and feet. My doctor has been giving me the run around for the past year. Thankfully, I have went for a second opinion. Your site just reaffirmed everything; I KNEW it wasn't all in my mind.

Itchyyyyy said...

I was diagnosed with ss at age 27. I am now 49. Taking methotrexate has helped with a lot of my symptoms. I used to get vasculitis but haven't had any bad bouts since I've been on methotrexate. However, I do get an awful systemic itch with no rash for weeks on end. It feels like bugs are crawling just under my skin. Has anyone else had this? Changing lotions, soaps, etc does not help. Thoughts anyone?

Anonymous said...

I am finding all this terrifying. Is there any hope to reverse sjogrens or have remission. I have a one year flare and every system in my body hurts or doesn't work

ECL said...

No doctor has ever mentioned my hormone patches and SS. I use Vivelle Dot as hormone replacement for surgical menopause, though am now old enough for true menopause, 61. I too have SS and skin problems, rashes and purple legs when standing. I will show this to my doctor and hopefully give them some useful information. Thank you

Unknown said...

Hello I have only just looked into this as i am having a problem with this itching sensation. This itching seems only to happen when I have a hand rolling tobacco' a roll up then I start to itch all over by body from head to my ankles . This is oy very recently this is starting happening I'm 54 but started smoking at the age of 10 and only smoke less than 10 a day and for the last 6 years I only smoke tobacco with no additives or preservatives .
Hope this info helps and maybe someone can add to my observations of myself . Yours kindly Daren