TibotSkin ProblemImmunological Skin DisorderCicatricial Pemphigoid

Cicatricial Pemphigoid

Mucous membrane pemphigoid, known as cicatricial pemphigoid, refers to a group of rare chronic autoimmune blistering diseases that predominately affect the mucous membranes, including the conjunctiva, and occasionally the skin.

Pemphigoid is a rare autoimmune disorder showing IgG and C3 deposition at the basement membrane zone that can develop at any age, including in kids, but that 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.

bullous (cicatricial pemphigoid)

Pemphigoid can also cause blistering on the mucous membranes. Mucous membranes produce mucus that helps protect inside of the body. Pemphigoid can be found on the mucous membranes in the eyes, nose, mouth, and genitals. It can also occur during pregnancy in some women.

Pemphigoid is a non-curable disease, but there are various supportive treatment options.

All types of pemphigoid are caused by the immune system attacking healthy tissue. They appear as rashes and fluid-filled blisters. The types of pemphigoid differ in terms of where on the body the blistering occurs and when it occurs.

Bullous pemphigoid: In cases of bullous pemphigoid — the common of the three types — the skin blistering happens commonly on the arms and legs where movement occurs. This includes the areas around the joints and on the lower abdomen.

Cicatricial pemphigoid: Cicatricial pemphigoid, also called mucous membrane pemphigoid, refers to blisters that form on the mucous membranes. This includes the: mouth, eyes, nose, throat, genitals.

The common sites affected are the mouth and eyes. The rash and blistering may begin in one of these areas and spread to the others if left untreated. If it’s left untreated in the eyes, it may cause scarring, which in turn may lead to blindness.

Pemphigoid gestationis: When blistering occurs during or shortly after pregnancy, it’s called pemphigoid gestationis. It was formerly called herpes gestationis, although it’s 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.

Female-to-male ratio of approximately 2:1. Most patients with mucous membrane pemphigoid are elderly, with a mean age of 62-66 years.

 

 

The common symptom of pemphigoid is blistering that occurs on the arms, legs, abdomen, and mucous membranes. Hives and itching are also common.

The blisters have certain characteristics, regardless of where on the body they form:

  • A red rash develops before the blisters,
  • The blisters are large and filled with fluid that’s usually clear, but may contain some blood,
  • The blisters are thick and don’t rupture easily,
  • The skin around the blisters may appear normal, or slightly red or dark,
  • Ruptured blisters are usually sensitive and painful.

Mucous membrane pemphigoid is an autoimmune blistering disease associated with autoantibodies directed against basement membrane zone target antigens. This means that immune system mistakes and begins to attack healthy tissues. In the case of pemphigoid, the immune system creates antibodies to attack the tissue just below the outer layer of skin. This causes the layers of skin to separate and results in painful blistering. It’s unknown why the immune system reacts this way in people living with pemphigoid.

In many cases, there’s no specific trigger for pemphigoid. In some instances, it may be caused by:

  • certain medications,
  • radiation therapy,
  • ultraviolet light therapy.

People with other autoimmune disorders are found to be at a higher risk for developing pemphigoid. It’s also more common in the elderly than in any other age group and seems to occur slightly more in women than men.

Pemphigoid cannot be cured, but treatments are usually very successful at relieving symptoms. The goal of treatment in mucous membrane pemphigoid (MMP) (cicatricial pemphigoid) is to suppress extensive blister formation, promote healing, and prevent scarring. Corticosteroids, either in pill or topical form, will likely be the first treatment doctor prescribes. These medications reduce inflammation and can help to heal the blisters and relieve itching. However, they can also cause significant side effects, especially from long-term use, so the doctor will taper off of the corticosteroids after the blistering clears up.

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

Surgical intervention may be required to improve functioning or to prevent further morbidity.

The management of mucous membrane pemphigoid (MMP) requires a coordinated team approach. Specific consultations are dictated by the phenotype of the disease and the target organ or organs involved.

The patient management team typically includes a dermatologist with expertise in this area; an internist to assist with monitoring therapy, adverse effects of medications, and the patient’s overall health; an ophthalmologist for ocular disease; an otolaryngologist for upper airway evaluation and management; and a dentist for oral disease.

Additional specialists, such as a gynecologist (vulvar disease), a gastroenterologist (esophageal involvement), and an endocrinologist (prophylaxis of osteoporosis in patients receiving long-term systemic corticosteroids), may be indicated.

So, when the suspected symptoms arise, must consult with a doctor to diagnosed the case.

  • Oxford hand Book of medical Dermatology
  • Clinical Dermatology
  • Andrew’s Diseases of the skin.
TibotSkin ProblemImmunological Skin DisorderCicatricial Pemphigoid

Cicatricial Pemphigoid

TUI - Tibot Urgency Index

Mucous membrane pemphigoid, known as cicatricial pemphigoid, refers to a group of rare chronic autoimmune blistering diseases that predominately affect the mucous membranes, including the conjunctiva, and occasionally the skin.

Pemphigoid is a rare autoimmune disorder showing IgG and C3 deposition at the basement membrane zone that can develop at any age, including in kids, but that 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.

bullous (cicatricial pemphigoid)

Pemphigoid can also cause blistering on the mucous membranes. Mucous membranes produce mucus that helps protect inside of the body. Pemphigoid can be found on the mucous membranes in the eyes, nose, mouth, and genitals. It can also occur during pregnancy in some women.

Pemphigoid is a non-curable disease, but there are various supportive treatment options.

All types of pemphigoid are caused by the immune system attacking healthy tissue. They appear as rashes and fluid-filled blisters. The types of pemphigoid differ in terms of where on the body the blistering occurs and when it occurs.

Bullous pemphigoid: In cases of bullous pemphigoid — the common of the three types — the skin blistering happens commonly on the arms and legs where movement occurs. This includes the areas around the joints and on the lower abdomen.

Cicatricial pemphigoid: Cicatricial pemphigoid, also called mucous membrane pemphigoid, refers to blisters that form on the mucous membranes. This includes the: mouth, eyes, nose, throat, genitals.

The common sites affected are the mouth and eyes. The rash and blistering may begin in one of these areas and spread to the others if left untreated. If it’s left untreated in the eyes, it may cause scarring, which in turn may lead to blindness.

Pemphigoid gestationis: When blistering occurs during or shortly after pregnancy, it’s called pemphigoid gestationis. It was formerly called herpes gestationis, although it’s 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.

Female-to-male ratio of approximately 2:1. Most patients with mucous membrane pemphigoid are elderly, with a mean age of 62-66 years.

 

 

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Dr. Lora Smith

MBBS (Dhaka), DGO (DU) Ex SR. Gynaecologist & Obstetrician

09 606 111 222

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