Cicatricial Pemphigoid

Cicatricial pemphigoid, also known as scarring pemphigoid, is a type of autoimmune blistering disorder that affects the skin and mucous membranes. It is characterized by the formation of blisters and erosions that eventually lead to scarring and fibrosis of the affected tissues.

The main cause of cicatricial pemphigoid is an abnormal immune response, in which the body’s immune system attacks and damages its own skin and mucous membranes. The exact trigger of this immune response is not yet fully understood, but it may be related to genetic factors, environmental triggers, or a combination of both.

Causes

Cicatricial pemphigoid, also known as scarring pemphigoid, is a rare autoimmune disease that affects the skin and mucous membranes. The exact cause of cicatricial pemphigoid is not known, but it is believed to be a combination of genetic and environmental factors.

The following are some of the main causes of cicatricial pemphigoid:

  1. Genetics: There is a strong genetic component to cicatricial pemphigoid, as people with a family history of autoimmune diseases are more likely to develop the condition.
  2. Environmental triggers: Certain environmental triggers such as infections, exposure to certain medications, and ultraviolet light exposure may trigger the onset of cicatricial pemphigoid.
  3. Autoimmunity: Cicatricial pemphigoid is considered an autoimmune disease, meaning that the body’s immune system mistakenly attacks its own tissues. This can result in inflammation and scarring of the skin and mucous membranes.
  4. Age: Cicatricial pemphigoid is most commonly diagnosed in people over the age of 60.
  5. Hormonal changes: Hormonal changes in women during pregnancy or menopause may trigger the onset of cicatricial pemphigoid.

It is important to note that the exact cause of cicatricial pemphigoid is still unknown, and further research is needed to fully understand the underlying mechanisms of this disease.

Symptoms

The main symptoms of cicatricial pemphigoid include:

  1. Blisters: The patient may experience fluid-filled blisters on the skin and mucous membranes, which may be painful and break easily.
  2. Rashes: A red, raised rash may appear on the skin, which can become itchy and painful.
  3. Scarring: As the blisters heal, they may leave behind scars, which can cause skin thickening, discoloration, and tightening.
  4. Oral sores: Cicatricial pemphigoid can also affect the mouth, causing painful sores and difficulty eating and speaking.
  5. Eye involvement: The disease can also affect the eyes, causing conjunctivitis, corneal ulcers, and vision loss.
  6. Joint pain: Some patients with cicatricial pemphigoid may experience joint pain and inflammation.
  7. Systemic symptoms: Some patients may experience systemic symptoms such as fever, fatigue, and weight loss.

It is important to seek medical attention if you experience any of these symptoms, as early treatment can help prevent scarring and other complications.

Diagnosis

It is diagnosed based on clinical presentation, history, physical examination, and laboratory testing. The following are the main diagnosis and tests used to diagnose cicatricial pemphigoid:

  1. Clinical presentation: The presence of blisters and erosions on the mucous membranes and skin are the hallmark of cicatricial pemphigoid. The blisters usually occur on the oral mucosa, genitalia, eyes, and anus.
  2. History: The patient’s history of blistering, itching, and burning sensations are important in diagnosing cicatricial pemphigoid.
  3. Physical examination: A thorough physical examination is performed to assess the location, size, and appearance of the blisters and erosions.
  4. Direct immunofluorescence (DIF) test: This test involves taking a small sample of skin and applying a fluorescent dye to it. The presence of antibodies in the skin sample is then detected using a fluorescence microscope. A positive result in DIF test confirms the diagnosis of cicatricial pemphigoid.
  5. Indirect immunofluorescence (IIF) test: This test involves incubating a patient’s serum with human skin or basement membrane zone. The presence of antibodies in the serum sample is then detected using a fluorescence microscope. A positive result in IIF test confirms the diagnosis of cicatricial pemphigoid.
  6. ELISA: Enzyme-linked immunosorbent assay (ELISA) is used to detect the presence of specific antibodies in the serum. A positive result in ELISA test confirms the diagnosis of cicatricial pemphigoid.
  7. Biopsy: A biopsy of the affected skin or mucous membrane may be performed to confirm the diagnosis and to rule out other possible causes of blistering.

In conclusion, the combination of clinical presentation, history, physical examination, and laboratory tests such as DIF, IIF, ELISA, and biopsy is used to diagnose cicatricial pemphigoid.

Treatment

The main goal of treatment for cicatricial pemphigoid is to control the immune system and reduce inflammation.

The latest treatment options for cicatricial pemphigoid include:

  1. Topical corticosteroids: Topical corticosteroids are used to reduce inflammation and relieve itching. They are applied directly to the affected area and are available in different forms, including creams, gels, and ointments.
  2. Systemic corticosteroids: Systemic corticosteroids are taken orally or intravenously and are used to control the immune system and reduce inflammation. They are typically used in severe cases of cicatricial pemphigoid.
  3. Immunosuppressive drugs: Immunosuppressive drugs, such as cyclophosphamide and azathioprine, are used to suppress the immune system and reduce inflammation. They are used in severe cases of cicatricial pemphigoid that do not respond to other treatments.
  4. Rituximab: Rituximab is a monoclonal antibody that targets the immune system and is used to reduce inflammation in cicatricial pemphigoid. It is used in severe cases that do not respond to other treatments.
  5. Plasma exchange: Plasma exchange is a procedure in which the plasma (the liquid part of the blood) is removed and replaced with fresh plasma or a plasma substitute. This procedure is used to remove harmful antibodies from the blood and reduce inflammation in cicatricial pemphigoid.

It is important to work closely with a dermatologist to determine the best treatment option for cicatricial pemphigoid, as individual cases can vary greatly.

  1. https://www.ncbi.nlm.nih.gov/books/NBK11733/
  2. https://www.ncbi.nlm.nih.gov/books/NBK208/
  3. https://www.ncbi.nlm.nih.gov/books/NBK212/
  4. https://www.ncbi.nlm.nih.gov/books/NBK92761/
  5. https://www.ncbi.nlm.nih.gov/books/NBK11733/
  6. https://www.nccih.nih.gov/health/skin-conditions-at-a-glance
  7. https://www.aad.org/public/diseases/a-z
  8. https://medlineplus.gov/skinconditions.html
  9. https://www.aad.org/about/burden-of-skin-disease
  10. https://www.usa.gov/federal-agencies/national-institute-of-arthritis-musculoskeletal-and-skin-diseases
  11. https://www.cdc.gov/niosh/topics/skin/default.html
  12. https://www.skincancer.org/
  13. https://www.jaad.org/
  14. https://www.psoriasis.org/about-psoriasis/
  15. https://books.google.com/books?
  16. https://www.niams.nih.gov/health-topics/skin-diseases
  17. https://cms.centerwatch.com/directories/1067-fda-approved-drugs/topic/292-skin-infections-disorders
  18. https://www.fda.gov/files/drugs/published/Acute-Bacterial-Skin-and-Skin-Structure-Infections—Developing-Drugs-for-Treatment.pdf
  19. https://dermnetnz.org/topics
  20. https://www.aaaai.org/conditions-treatments/allergies/skin-allergy
  21. https://www.sciencedirect.com/topics/medicine-and-dentistry/occupational-skin-disease
  22. https://aafa.org/allergies/allergy-symptoms/skin-allergies/
  23. https://www.nibib.nih.gov/
  24. https://rxharun.com/rxharun/rxharun/article-types/skin-care-beauty/skin-diseases-types-symptoms-treatment/
  25. https://www.nei.nih.gov/
  26. https://en.wikipedia.org/wiki/List_of_skin_conditions
  27. https://en.wikipedia.org/?title=List_of_skin_diseases&redirect=no
  28. https://en.wikipedia.org/wiki/Skin_condition
  29. https://oxfordtreatment.com/
  30. https://www.nidcd.nih.gov/health/
  31. https://consumer.ftc.gov/articles/w
  32. https://www.nccih.nih.gov/health
  33. https://catalog.ninds.nih.gov/
  34. https://www.aarda.org/diseaselist/
  35. https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets
  36. https://www.nibib.nih.gov/
  37. https://www.nia.nih.gov/health/topics
  38. https://www.nichd.nih.gov/
  39. https://www.nimh.nih.gov/health/topics
  40. https://www.nichd.nih.gov/
  41. https://www.niehs.nih.gov
  42. https://www.nimhd.nih.gov/
  43. https://www.nhlbi.nih.gov/health-topics
  44. https://obssr.od.nih.gov/
  45. https://www.nichd.nih.gov/health/topics
  46. https://rarediseases.info.nih.gov/diseases
  47. https://beta.rarediseases.info.nih.gov/diseases
  48. https://orwh.od.nih.gov/

References