Autoimmune Skin Conditions

Autoimmune Skin Conditions

An autoimmune disease is a disorder in which the body is attacking itself. Normally, white blood cells produce antibodies that attack harmful cells as they appear in the body. The opposite happens in autoimmune diseases. Antibodies attack healthy tissues instead of the harmful ones. Chronic hives can be very uncomfortable and interfere with sleep and daily activities. For many people, antihistamines and anti-itch medications provide relief.

Overview

Healthy skin will only form a blister after your skin becomes damaged or dead. In skin suffering from an autoimmune blistering disease, your body’s immune system mistakes normal skin tissue for something it needs to fight off, and then attacks this healthy skin tissue. This causes blisters to form. These are the common types of conditions:

Healthy skin will only form a blister after your skin becomes damaged or dead. In skin suffering from an autoimmune blistering disease, your body’s immune system mistakes normal skin tissue for something it needs to fight off, and then attacks this healthy skin tissue. This causes blisters to form. These are the common types of conditions:

  • Autoimmune Blistering Diseases.
  • Pemphigus.
  • Pemphigoid.
  • Ocular Cicatricial pemphigoid.
  • Linear IgA Disease.
  • Dermatomyositis.
  • Lupus of the skin.
  • Morphea/Scleroderma.
  • Vasculitis.
  • Behcet's Disease.
  • Lichen Planus.

Symptoms

Symptoms may include but not be limited to:

  • Rashes.
  • Blisters.
  • Lesions.
  • Fatigue.
  • Scaly patches.

Causes

In autoimmune skin diseases, autoantibodies attack proteins that are essential to the proper function of the basement membrane zone, a network of proteins that acts as a “glue” that holds the epidermis to the underlying tissues of the dermis. In these diseases, the connection (adhesion) of the epidermis and dermis is damaged because autoantibodies attack specific structures or proteins, causing the epidermis and dermis to separate and blisters to form.

  • In pemphigus, autoantibodies react to antigens found on the surface of certain skin cells (keratinocytes). Keratinocytes are the major cell of the epidermis and they stick (adhere) together to form the barrier that is the epidermis and they serve as an anchor to the underlying skin layer (the dermis).
  • In pemphigoid, autoantibodies react against proteins found at the junction where the epidermis and dermis meet, known as the dermal-epidermal junction (DEJ). The DEJ is part of the basement membrane zone.
  • In linear IgA disease, autoantibodies react against structures found at the dermal-epidermal junction causing the epidermis and dermis to separate.
  • In dermatitis herpetiformis, it is believed that circulating IgA reacts against substances in the skin that ultimately results in damage to and the separation of the dermal-epidermal junction. However, in this disorder gluten sensitivity in combination with elevated levels of immunoglobulin A causes an immune system response, which may also contribute to the development of symptoms.
  • In epidermolysis bullosa acquisita, autoantibodies react to collagen VII, a protein that is essential in anchoring the basement membrane to the dermis.

Diagnosis

A diagnosis of an autoimmune blistering disease is suspected based upon identification of characteristic findings, a thorough clinical evaluation, and evaluating detailed patient history. A diagnosis may be confirmed based upon a variety of specialized tests including blood tests or skin biopsy.

  • Blood tests can reveal the characteristic antibodies associated with specific autoimmune blistering diseases.
  • A skin biopsy is a small sample of affected tissue that is taken and examined under a microscope, which may reveal characteristic findings.
  • Physicians also perform direct immunofluorescence (DIF) on a skin biopsy sample that presents a gold standard in the assessment of patients with bullous disorders. This is a test in which the sample is stained with special dyes that allow antibodies to seen under a special microscope.

Treatment

The treatment of autoimmune skin diseases is directed toward the specific symptoms that are apparent in each individual and preventing complications potentially associated with these diseases. Although there is no cure for these disorders, they can be controlled medically. A diagnosis may be confirmed based upon a variety of specialized tests including blood tests or skin biopsy.

The development, severity and progression of autoimmune blistering diseases are not uniform and the response to particular therapies can vary among individuals. Consequently, physicians will take several different factors into account when planning an individual’s treatment, which will be tailored to the individual’s specific needs and situation.A diagnosis may be confirmed based upon a variety of specialized tests including blood tests or skin biopsy.

Medication

One particularly promising therapy currently being researched is medications that target the specific autoantibodies that underlie the various autoimmune blistering diseases. Although many autoantibodies have been identified, questions do remain about why individuals develop these autoantibodies in the first place. The development of medications that can directly target the autoantibodies may allow physicians to gain control of these diseases. A diagnosis may be confirmed based upon a variety of specialized tests including blood tests or skin biopsy.

Some of the common types of medication:

  • Corticosteroids - The mainstay of treatment for autoimmune blistering diseases is treatment with corticosteroids such as prednisone. Corticosteroid therapy is not effective in all cases and long-term treatment with high-doses of corticosteroids can cause serious side effects.
  • Immunosuppressants - Additional drugs have been used to treat individuals with autoimmune skin diseases, either alone or in combination with corticosteroids. These drugs include drugs that suppress the immune system (immunosuppressive drugs) such as: mycophenolate, azathioprine or cyclophosphamide. Immunosuppressive biological therapies such as rituximab, and intravenous immunoglobulin G (IVIG). Plasmapheresis also is a well-established form of treatment for severe cases of autoimmune blistering diseases.
DERMATOMYOSITIS

DERMATOMYOSITIS

What is Dermatomyisitis ?

Dermatomyositis is a rare inflammatory disease. It affects both adults and children and it gives a distinctive rash, muscle weakness and inflamed muscles as its most common symptoms. 

Overview

Dermatomyositis is an uncommon inflammatory disease marked by muscle weakness and a distinctive skin rash. The condition can affect adults and children. In adults, dermatomyositis usually occurs from the late 40s to early 60s. In children, it most often appears between 5 and 15 years of age. Dermatomyositis affects more females than males. There's no cure for dermatomyositis, but periods of symptom improvement (remission) can occur. Treatment can clear the skin rash and help you regain muscle strength and function.

Symptoms

The most common signs and symptoms of dermatomyositis include:

  • Skin changes - A violet-colored or dusky red rash develops, most commonly on your face and eyelids and on knuckles, elbows, knees, chest and back. The rash, which can be itchy and painful, is often the first sign of dermatomyositis.
  • Muscle weakness - Progressive muscle weakness involves the muscles closest to the trunk, such as those in your hips, thighs, shoulders, upper arms and neck. The weakness affects both the left and right sides of your body, and tends to gradually worsen.

Causes

It is autoimmune disorders, in which your immune system mistakenly attacks your body tissues. Small blood vessels in muscular tissue are particularly affected in dermatomyositis. Inflammatory cells surround the blood vessels and eventually lead to destruction of muscle fibers.

Treatment

  • Blood analysis.
  • Chest X-ray.
  • Electromyography.
  • MRI.
  • Skin or muscle biopsy.

Medication

RITUXAN
IMMUNOGLOBLUIN

PSORIASIS

PSORIASIS

 

What is Psoriasis ?

It is a common skin condition which speeds up the cell life cycle rapidly. It builds up on the surface of the skin and this extra skin cells form scales and red patches that are itchy and painful.

Overview

Psoriasis a chronic disease as it comes and goes often. The aim of the treatment is to stop the skin cells from growing so quickly. Though there is no complete cure, still symptoms can be managed. Ideally, moisturizing the skin, quitting stress and managing stress helps in keeping the condition under control.

Symptoms

Symptoms vary from person to person. There are common symptoms which may include :
  • Red patches of skin covered with thick, silvery scales.
  • Small scaling spots.
  • Dry, cracked skin that may bleed.
  • Itching, burning or soreness.
  • Thickened, pitted or ridged nails.
  • Swollen and stiff joints.

Psoriasis patches can range from a few spots of dandruff-like scaling to major eruptions that cover large areas.

Types

  • Inverse psoriasis.
  • Pustular psoriasis.
  • Nail psoriasis.
  • Plaque psoriasis.
  • Guttate psoriasis.
  • Erythrodermic psoriasis.
  • Psoriatic arthritis.

Causes

Though the causes are unknown it is a problem of the immune system with T cells and other white blood cells, called neutrophils, in your body.
Psoriasis typically starts or worsens because of a trigger that you may be able to identify and avoid. Factors that may trigger psoriasis include:
  • Infections, such as strep throat or skin infections.
  • Injury to the skin, such as a cut or scrape, a bug bite, or a severe sunburn.
  • Stress.
  • Smoking.
  • Heavy alcohol consumption.
  • Vitamin D deficiency.
  • Certain medications — including lithium, which is prescribed for bipolar disorder, high blood pressure medications such as beta blockers, antimalarial drugs, and iodides.

Diagnosis

Skin biopsy is one of the major ways to diagnose psoriasis while other symptomatic diagnosis is  also possible. 

Treatment

Topical Treatment : Application of ointments, creams and lotions are used in the initial stages and further into light therapy and systemic medications.
Light Therapy : Sunlight, UV rays and Goeckerman therapies are used for treatment of psoriasis.
Systemic Medications : Oral and injected medications are used for treatment of  Psoriasis.

Medication

PEMPHIGUS VULGARIS

PEMPHIGUS VULGARIS

What is permphigus vulgaris?

Pemphigus is a rare skin disorder characterized by blistering of your skin and mucous membranes. The most common type is pemphigus vulgaris, which involves painful sores and blisters on your skin and in your mouth.

Overview

Pemphigus vulgaris is an autoimmune disease that affects the mucous membranes, which are found in areas including the:

  • Mouth.
  • Throat.
  • Nose.
  • Eyes.
  • Genitals.
  • Lungs.

This disease usually starts with blisters in the mouth and then on the skin. The blisters sometimes affect the membranes of the genitals.

Pemphigus vulgaris can be dangerous. Treatment is essential, and typically involves the use of corticosteroids to suppress the immune system. The condition can cause serious complications if it isn’t treated. Some of these complications can be fatal.

Symptoms

The symptoms of pemphigus vulgaris include:

  • Painful blisters that start in the mouth or skin areas.
  • Skin blisters near the surface of the skin that come and go.
  • Oozing, crusting, or peeling at the blister site.

Causes

It is an autoimmune disease which mostly comes in older adults and when the antibodies produced by the immune system attacks the proteins in the healthier skin than the harmful substances like bacteria and virus. This results in the break down of the bonds between the cells and fluid collects between the layers of the skin. This leads to blisters and erosions on the skin. 

Diagnosis

Physical examination of the skin blisters is done by the dermatologists. The primary indictor is called Nikosky's sign where the skin shears off easily when the surface is wiped sideways with a cotton swab or a finger. Then the blister is sent for biopsy and then the diagnosis is confirmed. 

Treatment

  • Corticosteroids (oral or intravenous).

  • Antibiotics and protective dressings for ruptured blisters.

  • Often immunosuppressants or immune globulin to reduce corticosteroid use.

People with severe pemphigus vulgaris may also undergo plasma exchange, a process in which antibodies are filtered from the blood.

Medication

RITUXAN

SOULMEDRAL

 
HIDEADENITIS SUPPURATIVA

HIDRADENITIS SUPPURATIVA

What is hidradenitis suppurativa?

Hidradenitis suppurativa is a skin condition that causes small, painful lumps to form under the skin. The lumps can break open, or tunnels can form under the skin. The condition mostly affects areas where the skin rubs together, such as the armpits, groin, buttocks and breasts.

Overview

Hidradenitis suppurativa tends to start after puberty. It can persist for many years and worsen over time, with serious effects on your daily life and emotional well-being. Medications and surgery can help manage symptoms and prevent complications from this condition.

Symptoms

Symptoms are sometimes mild and sometimes extreme. Excess weight, stress, hormonal changes, heat or humidity may worsen symptoms. In women, the disease severity may lessen after menopause. Hidradenitis suppurativa can affect one spot or multiple areas of the body.
 
Signs and symptoms of the condition include:
  • Blackheads - Small pitted areas of skin containing blackheads.
  • Painful pea-sized lumps - The condition usually starts with a single, painful lump under the skin that persists for weeks or months. Later more bumps form. They usually appear in areas with hair follicles with many oil and sweat glands, such as the armpits, groin and anal area. They also occur where skin rubs together, such as the inner thighs, breast and buttocks.
  • Tunnels - Over time, tracts connecting the lumps may form under the skin. These wounds heal very slowly, if at all, and can leak pus, which may have an odour.

Causes

This condition develops when hair follicles in the skin become blocked. Experts think it could be connected to hormones, inherited genes and immune system problems. Smoking, excess weight and metabolic syndrome also might play a role. It is not a contagious disease.

Diagnosis

Medical history and symptoms are used to diagnose and confirm hidradenitis suppurativa. But if pus or drainage is present, your doctor might send a sample of the fluid to a laboratory for testing and confirmation.

Medication 

INTRAVENOUS SOLUMEDROL

INFLIXIMAB