The body reacts in a variety of ways to autoimmune disorders, which cause a person’s immune system to attack its own tissues.
Depending on the condition, an autoimmune disorder can affect a variety of organs, joints and muscles, or other bodily tissues. One tissue that’s commonly affected by autoimmune disorders is the skin.
There are many different types of skin-related autoimmune disorders, including scleroderma, psoriasis, dermatomyositis, epidermolysis bullosa, and bullous pemphigoid.
Scleroderma. The skin is just one area that is affected by scleroderma, which is actually a widespread condition that affects all of the body’s connective tissue. Since this autoimmune disorder extends throughout the body, patients can experience not only skin changes, but also symptoms in blood vessels, muscles, and organs. A localized form of scleroderma results in patches of thickened skin, while systemic scleroderma is the form that has the greatest impact on people’s lives.
There are two forms of systemic scleroderma: progressive systemic sclerosis (PSS), sometimes known as just systemic sclerosis (SS), and CREST syndrome. Patients with systemic scleroderma may experience symptoms that affect that the esophagus, intestines, lungs, heart, and kidneys. CREST syndrome is named after its symptoms: calcinosis (calcium accumulation under the skin), Raynaud phenomenon (redness or blueness of fingers and toes), esophageal dysfunction, sclerodactyly (thickening and tightness of the skin that surrounds the fingers and toes), and telangiectasia (red skin blotches causes by dilated blood vessels). In addition to the skin symptoms caused by scleroderma, patients may experience joint pain, shortness of breath, wheezing, constipation or diarrhea, bloating, weight loss, heartburn, or eye itching and burning.
Men and women are both at risk for scleroderma, but the majority of cases occur in women in their thirties and forties. Occupational exposure to silica dust and polyvinyl chloride are considered risk factors for this autoimmune disorder. According to the Scleroderma Foundation, an estimated 300,000 people in the United States live with scleroderma; about 33 percent of them have the systemic type.
Psoriasis. Psoriasis is a chronic autoimmune disorder that manifests as skin redness and irritation. There are five different types of psoriasis: guttate, plaque, inverse, erythrodermic, and pustular. The most common is plaque psoriasis, in which raised, red skin patches are covered by flaky, silver-white patches of dead skin, known as scales. Current research indicates that psoriasis is most likely an inherited disorder — commonly, psoriasis patients have a family member with the same disease or another autoimmune disorder.
Psoriasis symptoms can come and go through a person’s life. Episodes of this autoimmune disorder may be triggered by infections, skin injuries, sun exposure, medications, alcohol, or even stress. People whose immune systems are already compromised, such as those with HIV or undergoing chemotherapy, are at risk for more severe attacks of psoriasis.
The National Institutes of Health reports that approximately 7.5 million Americans are living with psoriasis. Usually, signs of this autoimmune disorder appear between the ages of 15 and 35, although people of all ages can be affected. An estimated 30 percent of people with psoriasis also have arthritis — a condition known as psoriatic arthritis.
Dermatomyositis. This autoimmune disorder is primarily muscular in nature, but because dermatomyositis also affects the skin, it is sometimes categorized with skin-related autoimmune conditions. Dermatomyositis goes hand-in-hand with polymyositis, an autoimmune disease that causes muscle weakness, soreness, and stiffness. Patients with these conditions also may experience difficulty swallowing and shortness of breath. Dermatomyositis and polymyositis share these symptoms, but dermatomyositis is distinguished by a skin rash, normally on the upper body, as well as thickening and tightening of the skin in many areas. Dermatomyositis patients may also have purple colored eyelids.
Childhood dermatomyositis is differentiated from the adult form, with symptoms including fever, fatigue, rash, and weakness. In children, the disorder normally shows up between the ages of 5 and 15, and in adults, people 40 to 60 are most at risk. The condition is more prevalent among women.
Epidermolysis bullosa.There are many forms of epidermolysis bullosa, but only one, epidermolysis bullosa acquisita, is considered autoimmune in nature. All forms of epidermolysis bullosa cause fluid-filled skin blisters to develop in response to injuries that don’t normally warrant that type of reaction. For example, gentle rubbing of the skin or even an increase in room temperature can cause blisters to form.
Diagnosing the correct form of epidermolysis bullosa can be challenging. However, one distinguishing characteristic of epidermolysis bullosa acquisita is that it normally doesn’t develop until later in life — after age 50 — while non-autoimmune forms of epidermolysis bullosa typically show up at birth or soon after. The condition can still be difficult to diagnose, because it's hard to differentiate from mucous membrane pemphigoid, another autoimmune disorder characterized by blistering.
Bullous pemphigoid. This chronic autoimmune disorder involves skin blisters that range in severity. In some cases, the patient may experience only mild redness or irritation of the skin, while other, more severe cases involve multiple blisters that can break open and form ulcers. Bullous pemphigoid patients normally develop blisters on their arms, legs, or torso, and in about one-third of cases, blisters form in the mouth. Some but not all people with this condition also experience itching and bleeding gums.
Cases of bullous pemphigoid have been reported in all age groups, but the disorder most commonly affects the elderly. Men and women are equally at risk for bullous pemphigoid. It is difficult to pin down the incidence of this disease because symptoms come and go, with many patients seeing the condition completely disappear after six years. One estimate is that roughly 5 or 10 new cases of bullous pemphigoid are seen in a typical large hospital each year.
If you have symptoms of any of these autoimmune disorders of the skin, see your doctor. She can help you determine what is causing your symptoms and start you on the appropriate treatment.