Pemphigoid: Types, Causes, Risk Factors, Signs, Symptoms, Investigations, Treatment
Pemphigoid is a rare type of autoimmune disorder commonly affecting the elderly people; however, it can affect individuals of any age too. Pemphigoid is characterized by skin blistering and rashes on the arms, legs, and abdomen caused due to malfunction of the immune system. There may also be blistering of the mucous membranes of the nose, eyes, mouth and vagina. This can also occur in pregnancy. As of now, there is no cure for pemphigoid, but the prognosis of pemphigoid is good with treatment. Many patients benefit with treatment and in many cases the disease will go away after some years of treatment. However, there are some patients where even with diligent treatment, there is recurrence of pemphigoid.
Treatment comprises of managing this disease and its symptoms with medicines, such as corticosteroids, immunosuppressants and certain antibiotics.
Types of Pemphigoid
There are different types of pemphigoid and they all occur as a result of malfunction in the immune system. Patient develops rashes and fluid-filled blisters on his/her skin. Depending on the location and the time of the development of the rashes and blisters, the different types of pemphigoid are:
- Bullous Pemphigoid: In this type of pemphigoid, the blistering on the skin commonly occurs around the joints of the arms and legs where maximum movement occurs and also in the region of lower abdomen.
- Cicatricial Pemphigoid: In this type of pemphigoid, blisters are formed on the mucous membranes lining the mouth, throat, nose, eyes and genitals.
- Pemphigoid Gestationis: This type of pemphigoid is also known as Herpes Gestationis (no relation to herpes virus). The blistering occurs during or soon after pregnancy. Rashes and blisters usually appear in the second or third trimester of the pregnancy or can occur up to six weeks after the delivery. Blisters often form on abdomen, arms and legs.
Causes and Risk Factors of Pemphigoid
Pemphigoid is an autoimmune disorder where our body's immune system, instead of protecting us from infection, mistakenly starts attacking its own healthy tissues. In pemphigoid, the immune system produces antibodies which attack the subcutaneous tissue, which is present beneath the skin's outer layer. This results in separation of the layers of skin and leading to blistering and rashes which are very painful. The exact cause behind our immune system behaving this way is not known.
There are some triggering factors for this condition, such as certain medications, ultraviolet light therapy, radiation therapy etc. Individuals who already are suffering from other autoimmune disorders are more prone to develop pemphigoid. This condition also largely affects the elderly people.
Signs and Symptoms of Pemphigoid
The main symptom of pemphigoid is the water filled blisters which develop on the abdomen, arms, legs and mucous membranes. The blisters have the following characteristics:
- Patient may also experience itching and hives.
- The blisters are commonly preceded by a red rash.
- The blisters are large in size and are filled with clear fluid.
- Sometimes, the fluid within the blisters can be blood tinged.
- The blisters have thick walls and they do not rupture easily.
- The skin surrounding the blisters is often normal or it can be slightly red or dark in color.
- If the blisters rupture then they can be very painful.
Investigations for Pemphigoid
Physical examination of the blisters is often sufficient for a diagnosis. For definitive diagnosis, tests such as biopsy, where small skin samples of the affected regions is removed and sent to lab for testing, is done. These biopsied samples are examined for immune system antibodies, which are present in pemphigoid. Blood tests can also be done, as these antibodies can also be found in the blood.
Treatment for Pemphigoid
There is no permanent cure for pemphigoid, but treatment can greatly relieve the symptoms.
- Treatment comprises of oral or topical corticosteroids which will help in reducing the inflammation, healing of the blisters and also gives relief from itching. Long term use of corticosteroids has some serious side effects, so careful monitoring is required. For this reason your doctor may stop corticosteroids once the blistering subsides.
- Immunosuppressants are another treatment choice. These are medicines, which suppress the immune system. These are usually given in combination with corticosteroids. Immunosuppressants are effective, but they increase the risk of the patient having infections.
- Antibiotics, like tetracycline, can also be prescribed to decrease inflammation and infection.