Pemphigus is a group of rare autoimmune diseases that cause blistering of the skin and mucous membranes such as in mouth, nose, throat, eyes, and genitals.

There are several types of pemphigus. The type of disease depends on what layer in the skin the blisters form and where they are located on the body. The two main types are:

  • Pemphigus vulgaris: It is the most common subtype of pemphigus in the United States and worldwide. It is characterized by blisters and erosions on the skin and mucous membranes, most commonly inside the mouth.
  • Pemphigus foliaceus: It is characterized by blistering lesions which usually begin on face and scalp and later on the chest and other parts of the body. The sores are superficial and often itchy, and usually are not painful.

Pemphigus is an autoimmune blistering disease, which means that the immune system starts attacking its cells in the epidermis, or top layer of the skin, and the mucous membranes.  It is unclear what triggers the disease, although it appears that some people have a genetic susceptibility. Environmental agents may trigger the development of pemphigus in people who are likely to be affected by the disease because of their genes. In rare cases, it may be triggered by certain medications. In those cases, the disease usually disappears when the medication is stopped.

The primary aim of treatment is to decrease blister formation, prevent infections and promote healing of blisters and erosions. Treatment for pemphigus involves using one or more drugs. High doses of oral corticosteroids are the mainstay of medical treatment for controlling the disease. Immunosuppressive drugs are often added to the treatment regimen to minimize steroid use. Severe cases of pemphigus may need intravenous immunoglobulin (IVIG) treatment to reduce the amount of antibodies in the blood.

Treatment for pemphigus and pemphigoid involves using one or more drugs. The mainstay of current treatment is the administration of high-dose oral corticosteroids, such as prednisone or prednisolone. However, using corticosteroids over an extended period or in high doses may cause serious side effects, including type 2 diabetes, osteoporosis, an increased risk of infection, salt and water retention, cataracts and swelling of the face and upper back.

To keep the levels of corticosteroid use to a minimum, immunosuppressive agents are often used as adjuvants. These drugs may have serious side effects, including increased risk of infection, anemia, inflammation of the liver, nausea, vomiting or allergic reactions.

IVIG is an effective therapy in the treatment of patients with pemphigus. It seems to work by rapidly and selectively lowering serum levels of pemphigus antibodies.[1]The magnitude of the decrease in antibody levels varies among patients – it is dramatic in some, minimal in others. The decrease in pemphigus antibodies associated with IVIG treatment is highly selective.

The mechanism of action of IVIG is very similar to that of plasmapheresis. Both procedures rapidly remove circulating pemphigus antibodies from the circulation and do so at about equal rates. However, IVIG has major advantages over plasmapheresis in that the pemphigus antibodies are removed selectively.  Plasmapheresis, as usually performed, removes all circulating immunoglobulins, the good with the bad. Furthermore, IVIG appears to be safer[1] as it does not damage red blood cells and is less damaging to venous access.

Reference

1. Engineer, L, Bhol, KC, Ahmed, AR: Analysis of current data on the use of intravenous immunoglobulin in management of pemphigus vulagaris. J M Acad Dermatol 43:1049-1057, 2000.

The IVIG dose is usually 2 gm of IVIG/kg per cycle, administered over 5 days, every 2 to 4 weeks for several months.

There is a general agreement that IVIG is indicated for the control of pemphigus unresponsive to conventional therapy, or when a serious complication to standard therapy occurs. Other suggested indications include inability to withdraw steroids without a flare in disease activity, absolute or relative contraindications to the use of systemic steroids, and progressive disease despite appropriate but safe conventional therapy [1]. An important element in the decision of treating pemphigus with IVIG are the policies of Medicare or of the insurance plan that will pay for the treatment. The indications vary with each plan, and Medicare’s indication vary from region to region in the US.

Reference

1. Engineer, L, Bhol, KC, Ahmed, AR: Analysis of current data on the use of intravenous immunoglobulin in management of pemphigus vulagaris. J M Acad Dermatol 43:1049-1057, 2000.

Pemphigoid is a rare autoimmune disorder that can develop at any age, but that most often affects the elderly. Pemphigoid is caused by a malfunction of the immune system and results in skin rashes and blistering on the legs, arms, and abdomen. Pemphigoid can also cause blistering on the mucous membranes of the eyes, nose, mouth, and vagina, and can occur during pregnancy in some women. There is no cure for pemphigoid, but there are many treatment options.

All types of pemphigoid are caused by an immune system malfunction. They create rashes and fluid-filled blisters. The types of pemphigoid differ in terms of where on the body the blistering occurs and when it occurs. The main types are:

Bullous Pemphigoid

In cases of bullous pemphigoid, the skin blistering occurs most commonly on the arms and legs where movement occurs, that is, around the joints and on the lower abdomen. 

Cicatricial Pemphigoid

Cicatricial pemphigoid refers to blisters that form on the mucous membranes. This includes the mouth, eyes, nose, throat, and genitals. The rash and blistering may begin in one of these areas and spread to the others if left untreated.

Pemphigoid Gestationis

When blistering occurs during or shortly after pregnancy, it is called pemphigoid gestationis. It is also called herpes gestationis, although it is not related to the herpes virus. The blistering typically develops during the second or third trimester, but may occur at any time during pregnancy or up to six weeks after delivery. Blisters tend to form on the arms, legs and abdomen.

Pemphigoid is an autoimmune disease. This means that your immune system, which normally protects you from infection, begins to attack your healthy tissues. In the case of pemphigoid, your immune system creates antibodies to attack the tissue just below your outer layer of skin. This causes the layers of skin to separate and results in painful blistering. Why the immune system reacts this way in patients with pemphigoid is not fully understood.

In many cases, there is no specific trigger for pemphigoid. In some instances, however, it may be caused by certain medications, radiation therapy, or ultraviolet light therapy. People with other autoimmune disorders may be at a higher risk for developing pemphigoid. It is much more common in the elderly than in any other group.

Pemphigoid cannot be cured, but treatments are usually very successful at relieving symptoms. Corticosteroids, either in pill or topical form, will likely be the first treatment your doctor prescribes. These medications reduce inflammation and can help to heal the blisters and relieve itching. However, they can also cause serious side effects, especially from long-term use, so your doctor will take you off of the corticosteroids after the blistering clears up.

Another treatment option is to take medication that suppresses your immune system, often in conjunction with the corticosteroids. Immunosuppressants help, but they also put you at risk for other infections. Certain antibiotics, such as tetracycline, may also be prescribed to reduce inflammation and infection.

Severe cases may need intravenous immunoglobulin (IVIG) treatment to reduce the amount of antibodies in the blood.

Treatment for pemphigus and pemphigoid involves using one or more drugs. The mainstay of current treatment is the administration of high-dose oral corticosteroids, such as prednisone or prednisolone. However, using corticosteroids over an extended period or in high doses may cause serious side effects, including type 2 diabetes, osteoporosis, an increased risk of infection, salt and water retention, cataracts, and swelling of the face and upper back.

To keep the levels of corticosteroid use to a minimum, immunosuppressive agents are often used as adjuvants. These drugs may have serious side effects, including increased risk of infection, anemia, inflammation of the liver, nausea, vomiting, or allergic reactions.

IVIG is an effective therapy in the treatment of patients with pemphigus. It seems to work by rapidly and selectively lowering serum levels of pemphigus antibodies.[1]The magnitude of the decrease in antibody levels varies among patients – it is dramatic in some, minimal in others. The decrease in pemphigus antibodies associated with IVIG treatment is highly selective.

The mechanism of action of IVIG is very similar to that of plasmapheresis. Both procedures rapidly remove circulating pemphigus antibodies from the circulation and do so at about equal rates. However, IVIG has the major advantage over plasmapheresis in that the pemphigus antibodies are removed selectively. Plasmapheresis as usually performed removes all circulating immunoglobulins, the good with the bad. Furthermore, IVIG appears to be safer[1] as it does not damage red blood cells and is less damaging to venous access.

References:

1. Jean-Claude Bystryn M.D., Jennifer L Rudolph M.D. and Ronald O Perelman et Al. IVIg Treatment of Pemphigus: How it Works and How to Use it. Journal of Investigative Dermatology (2005) 125, 1093–1098; doi:10.1111/j.0022-202X.2004.23930.x

The IVIG dose is usually 2 gm of IVIG/kg per cycle, administered over 5 days, every 2 to 4 weeks for several months.

There is a general agreement that IVIG is indicated for the control of pemphigus unresponsive to conventional therapy, or when a serious complication to standard therapy occurs. Other suggested indications include inability to withdraw steroids without a flare in disease activity, absolute or relative contraindications to the use of systemic steroids, and progressive disease despite appropriate but safe conventional therapy[2]. An important element in the decision of treating pemphigus with IVIG are the policies of Medicare or of the insurance plan that will pay for the treatment. The indications vary with each plan, and Medicare’s indication vary from region to region in the US.

References:

2. Ahmed, A.R., MD, DSc; Dahl, M.V., MD et. Al. Consensus statement on the use of intravenous immunoglobulin therapy in the treatment of autoimmune mococutaneous blistering disease. Arch Dermatol. 2003;139:1051-1059.

Dermatomyositis is an uncommon muscle disease that is accompanied by a skin rash. It affects adults and children alike. In adults, dermatomyositis usually occurs from the late 40s to early 60s; in children, the disease most often appears between 5 and 15 years of age. Dermatomyositis affects more females than males.

The most common symptom of dermatomyositis is a purple or dark red rash that may occur anywhere on the skin, but most typically appears on the eyelids and in places on the skin where a muscle covers a joint, such as the elbows, knees, knuckles and toes. Additionally, there may be muscle pain, weakness, atrophy (reduction in size), and dysfunction. Symptoms can vary in intensity and frequency among individuals.

The exact cause of dermatomyositis is unknown, but the disease is similar to other autoimmune disorders, in which your immune system attacks itself. Research is taking place to identify other factors that may play a part in its development, some of which are an infection, underlying skin cancer(more likely in elderly) and genetic predisposition.

There is no cure for dermatomyositis. The primary aim of treatment is to control the skin condition and the muscle strenght and function. Treatment options include antimalarial medications, corticosteroids to reduce inflammation, immunosuppressant medications, intravenous immunoglobulins (IVIG) to reduce the immune response, pain relieving medications and steroid-sparing agents. Other important measures in the management of dermatomyositis include physical therapy, speech therapy, and dietary counseling.

The mainstay of dermatomyositis treatment usually involves oral corticosteroids to slow down the rate of disease progression. Immunosuppressive medications may also be used in conjunction with corticosteroids. These medicines all have significant side effects and often a less than adequate response is achieved with this conventional therapy.

IVIG is an effective additional therapy for patients with dermatomyositis who fail to respond to conventional therapy or who experience unacceptable side effects. A summary of clinical trials shows an overall response rate of 80% at about 2 months, with maximal response at 4 months. Most patients require ongoing IVIG therapy in addition to conventional treatments given at lower and better-tolerated dosages.

IVIG is recommended to be given monthly at a dose of 1-2 g/kg administered over 5 days. Usually the effects of IVIG can last up to a month after each administration. IVIG is given as an infusion into a vein over a period of time, usually from 4 to 6 hours.

Skin reactions to IVIG are uncommon. Of all the reported rashes, a a blistering type of eczema is the most common. [1] It often begins at about 8 to 10 days after exposure to IVIG. The rash characteristically begins as small itchy blisters on the palms and/or soles that then extends to the rest of the body. The affected individual may become red all over and itchy. Switching the type of IVIG may eliminate the reaction.

The skin lesions often resolve within a period of 1 to 4 weeks. The use of steroids controls symptoms and may speed recovery.

Reference

1. Vecchietti, G, et al., Severe eczematous skin reaction after high-dose intravenous immunoglobulin infusion: report of 4 cases and review of the literature. Arch Dermatol, 2006. 142(2): p. 213-7.

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