cicatricial pemphigoid

Cicatricial Pemphigoid: A Rare and Chronic Autoimmune Disease

Cicatricial pemphigoid, also known as benign mucosal pemphigoid or mucous membrane pemphigoid, is a rare and chronic autoimmune disease characterized by blistering lesions on the mucous membranes and skin. This condition can produce scarring, which can have devastating sequelae.

Key Features:

• Affecting the mucous membranes (including the conjunctiva) and/or the skin
• Blistering lesions that frequently heal with scarring
• Can affect the oral cavity, eyes, and other areas of the body
• May cause blindness, supraglottic stenosis, or airway obstruction due to severe scarring

Symptoms:

• Itching blisters on the mucous membranes and skin
• Erosive skin lesions that can lead to scarring
• Long duration of clinical course, often with significant scarring

Treatment and Referral:

• Patients diagnosed with oral pemphigoid lesions should be referred to a dermatologist and an ophthalmologist for assessment of ocular lesions
• Examination by a gastroenterologist is advised for patients with dysphagia or severe oral lesions to detect possible involvement of the esophagus

References:

• [1] Cicatricial pemphigoid is a rare, chronic autoimmune blistering disorder which can produce scarring. It can affect the skin only, mucous membranes only, or both the skin and mucous membranes. When only mucous membranes are involved, the disease is often referred to as mucous membrane pemphigoid. [11]
• [2] Cicatricial pemphigoid is a subepidermal blistering disease that involves the mucous membranes and the skin. The oral cavity and the eye are most frequently involved. The clinical course is of long duration, and often there is significant scarring that can have devastating sequelae. [13]
• [3] Skin inflammatory (nontumor) - Cicatricial pemphigoid. Affects mucosa of mouth and eyes; lesions heal with scarring May cause blindness, supraglottic stenosis or airway obstruction [14]

Common Signs and Symptoms of Cicatricial Pemphigoid

Cicatricial pemphigoid, also known as mucous membrane pemphigoid (MMP), is a rare autoimmune blistering disorder that can affect the skin and/or mucous membranes. The signs and symptoms of this condition can vary depending on the location and severity of the disease.

Eye-Related Symptoms

• Eye irritation, such as a burning sensation or excessive tearing [1]
• Grittiness, redness, or pain in the eye
• Inward turning of the eyelashes or eyelids
• Shrinking of the space between the eye and eyelid
• Symblepharon: an abnormal adhesion between the eyelid and eyeball

Mouth-Related Symptoms

• Pain in the mouth
• Bleeding when brushing teeth
• Recurrent bloody noses
• Red eyes
• Pain while urinating or with bowel movements

Skin-Related Symptoms

• Tense blisters and erosions, often on the head and neck or at sites of trauma [3]
• Scarring of affected areas
• Subepidermal bullae (blisters) and modest scarring [5]

Other Symptoms

• Hoarseness and difficulty swallowing
• Ulcers in the conjunctiva, mouth, genitals, and anus

Early Signs and Symptoms

In the early stages of MMP with ocular involvement, symptoms are often nonspecific and may delay diagnosis. As the disease progresses, subconjunctival fibrosis and shortening of the inferior fornix can occur [14].

It's essential to note that cicatricial pemphigoid is a rare condition, and not all people will experience all of these symptoms. If you suspect you or someone else may have this condition, consult a healthcare professional for proper diagnosis and treatment.

References: [1] - Search result 4 [3] - Search result 3 [5] - Search result 5 [14] - Search result 14

Cicatricial pemphigoid, also known as mucous membrane pemphigoid (MMP), is a rare autoimmune blistering disorder that can affect the skin and/or mucous membranes. Diagnostic tests for cicatricial pemphigoid are crucial in confirming the diagnosis and ruling out other conditions.

Immunohistochemical Studies

Direct immunofluorescence microscopy on biopsy specimens from affected areas, such as the conjunctiva or gums, is a key diagnostic test for cicatricial pemphigoid [6]. This test can reveal the presence of autoantibodies and complement components in the tissue.

Serological Testing

Immunoserologic tests, including indirect immunofluorescence microscopy on salt-split skin, can help detect circulating autoantibodies in the serum [4]. These tests are essential for diagnosing cicatricial pemphigoid and monitoring disease activity.

Biopsy

A biopsy of affected tissue, such as the conjunctiva or gums, may be performed to examine the tissue under a microscope. This can help confirm the diagnosis of cicatricial pemphigoid [5].

Other Diagnostic Tests

Additional diagnostic tests, including blood tests and imaging studies (e.g., CT scans

Treatment Options for Cicatricial Pemphigoid

Cicatricial pemphigoid, also known as mucous membrane pemphigoid (MMP), is a rare and chronic autoimmune blistering disorder that can result in scarring. The goal of treatment is to suppress extensive blister formation, promote healing, and prevent scarring.

Medications Used to Treat Cicatricial Pemphigoid

Several medications are used to treat cicatricial pemphigoid, including:

• Immunosuppressants: These non-steroid anti-inflammatory medications are designed to dampen the immune system's response and reduce inflammation. Examples include methotrexate, azathioprine, cyclophosphamide, and cyclosporine.
• Biologic therapies: These newer treatments target specific proteins involved in the autoimmune response. Examples include rituximab and tumor necrosis factor-alpha inhibitors.
• Intravenous immune globulin: This treatment involves infusing antibodies into the bloodstream to help reduce inflammation.
• Dapsone: A sulfa-type medication that may be used to treat mild cases of MMP.

Treatment Strategies

A step-up and step-down treatment strategy is often employed, where medications are gradually increased or decreased depending on disease severity and patient response. Monotherapy or combination therapy with multiple medications may be used.

• Methotrexate monotherapy: This has been shown to be effective in achieving complete control or suppression of conjunctival inflammation in 89% of eyes with ocular-cicat

Differential Diagnosis of Cicatricial Pemphigoid

Cicatricial pemphigoid, a rare autoimmune blistering disorder, can be challenging to diagnose due to its varied clinical symptoms. The differential diagnosis for cicatricial pemphigoid includes several conditions that may present with similar symptoms.

• Bullous Pemphigoid: A common autoimmune subepidermal blistering disorder that affects the skin and mucous membranes. It is often seen in older adults, typically between 65 to 75 years of age [1].
• Mucous Membrane Pemphigoid (MMP): A condition that primarily affects the mucous membranes, including the oral and ocular areas. Historically, the term "cicatricial pemphigoid" also referred to MMP [2].
• Lichen Planus: An autoimmune disease that can affect the skin and mucous membranes, causing lesions and blisters. It is often difficult to distinguish from cicatricial pemphigoid, especially when it affects the mucous membranes [3].
• Chronic Herpesvirus Infection: A viral infection that can cause blistering and scarring in the oral and ocular areas, similar to cicatricial pemphigoid.
• Contact Mucositis: An inflammatory condition caused by contact with irritants or allergens, which can lead to blistering and scarring in the mucous membranes.
• Erythema Multiforme: A skin condition characterized by blisters and rashes, often triggered by infections or medications. It can be difficult to distinguish from cicatricial pemphigoid.
• Stevens-Johnson Syndrome: A rare but serious skin condition that can cause blistering and scarring in the mucous membranes and skin.

To accurately diagnose cicatricial pemphigoid, a comprehensive evaluation of clinical symptoms, laboratory tests, and histopathological examination is necessary. The differential diagnosis for cicatricial pemphigoid requires careful consideration of these conditions to ensure proper treatment and management.

References:

[1] Bullous Pemphigoid: A common autoimmune subepidermal blistering disorder. [2] Mucous Membrane Pemphigoid (MMP): A condition that primarily affects the mucous membranes. [3] Lichen Planus: An autoimmune disease that can affect the skin and mucous membranes.