Cicatricial pemphigoid

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Cicatricial pemphigoid
Other namesMucous membrane pemphigoid, MMP, Benign mucosal pemphigoid, Benign mucous membrane pemphigoid, [1] Ocular pemphigoid, [2] , and Scarring pemphigoid [1] )
Specialty Dermatology   OOjs UI icon edit-ltr-progressive.svg

Cicatricial pemphigoid is a rare chronic autoimmune subepithelial blistering disease characterized by erosive lesions of the mucous membranes and skin that can result in scarring. [3] :581 It is one of the pemphigoid diseases.

Contents

Signs and symptoms

The autoimmune reaction most commonly affects the oral mucosa in the mouth, causing lesions in the gums (gingiva), known as desquamative gingivitis but it can also affect areas of mucous membrane elsewhere in the body, such as the sinuses, genitals, anus, and cornea. When the cornea of the eye is affected, repeated scarring may result in blindness.

Brunsting–Perry cicatricial pemphigoid is a rare variant of mucous membrane pemphigoid involving the scalp and the neck without mucosal involvement. It is proposed by some authors that this be called a variant of epidermolysis bullosa acquisita. [4] [1]

Nikolsky's sign (gentle lateral pressure) on unaffected mucosa or skin raises a bulla. If no lesions are present on examination it may be useful way of demonstrating reduced epithelial adhesion. In contrast, in Pemphigus, the epithelium tends to disintegrate rather than form a bulla.

Nikolsky's sign is present in pemphigus and cicatricial pemphigoid, but not in bullous pemphigoid.

Pathophysiology

In mucous membrane pemphigoid, the autoimmune reaction occurs in the skin, specifically at the level of the basement membrane, which connects the lower skin layer (dermis) to the upper skin layer (epidermis) and keeps it attached to the body.

When the condition is active, the basement membrane is dissolved by the antibodies produced, and areas of skin lift away at the base, causing hard blisters which scar if they burst. In other words, this is a desquamating/blistering disease in which the epithelium "unzips" from the underlying connective tissue, allowing fluid to gather that subsequently manifest as bullae, or blisters.

Diagnosis

Diagnostic techniques:

Management

The management depends upon the severity of the condition. For example, where there are lesions in the mouth alone, systemic drugs are less likely to be used. Where the condition is not limited to the mouth, or where there is poor response to Topical treatments, systemic drugs are more likely to be used. [5]

Conservative

Simple measures that can be taken include avoidance of hard, sharp or rough foods, and taking care when eating. Good oral hygiene is also usually advised, and professional oral hygiene measures such as dental scaling . [5]

Medications

Topical and intralesional (injected into the affected areas) corticosteroid drugs may be used, such as fluocinonide, clobetasol propionate or triamcinolone acetonide. Oral candidiasis may develop with long term topical steroid use, and sometimes antimycotics such as miconazole gel or chlorhexidine mouthwash are used to prevent this. Topical ciclosporin is sometimes used.

Dapsone is sometimes used as a steroid sparing agent. The dose is often increased very slowly in order to minimize side effects. Systemic steroids, such as prednisone or prednisolone may be needed in severe cases. Many other drugs have been used to treat mucous membrane pemphoid, including azathioprine, cyclophosphamide, methotrexate, thalidomide, mycophenolate mofetil, leflunomide, sulphasalazine, sulphapuridine, sulphamethoxypiridazine, tetracyclines (e.g. minocycline, doxycycline) and nicotinamide. [5]

Other treatments

Plasmapheresis appears to help some cases. Sometimes surgical procedures are required to repair scars, prevent complications such as blindness, upper airway stenosis or esophageal stricture. [5]

See also

Related Research Articles

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Oral candidiasis a fungal infection

Oral candidiasis, also known as oral thrush among other names, is candidiasis that occurs in the mouth. That is, oral candidiasis is a mycosis of Candida species on the mucous membranes of the mouth.

Lichen planus lichen disease that is located in skin, located in tongue or located in oral mucosa, which presents itself in the form of papules, lesions or rashes

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Pemphigus rare group of blistering autoimmune diseases that affect the skin and mucous membranes

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Aphthous stomatitis stomatitis characterized by the repeated formation of benign and non-contagious mouth ulcers

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Bullous pemphigoid Autoimmune disease of skin and connective tissue characterized by large blisters

Bullous pemphigoid is an autoimmune pruritic skin disease preferentially in older people, aged over 60, that may involve the formation of blisters (bullae) in the space between the epidermal and dermal skin layers. The disorder is a type of pemphigoid. It is classified as a type II hypersensitivity reaction, with the formation of anti-hemidesmosome antibodies.

The oral mucosa is the mucous membrane lining the inside of the mouth. It comprises stratified squamous epithelium, termed "oral epithelium", and an underlying connective tissue termed lamina propria. The oral cavity has sometimes been described as a mirror that reflects the health of the individual. Changes indicative of disease are seen as alterations in the oral mucosa lining the mouth, which can reveal systemic conditions, such as diabetes or vitamin deficiency, or the local effects of chronic tobacco or alcohol use. The oral mucosa tends to heal faster and with less scar formation compared to the skin. The underlying mechanism remains unknown, but research suggests that extracellular vesicles might be involved.

Nikolsky's sign is a clinical dermatological sign, named after Pyotr Nikolsky (1858–1940), a Russian physician who trained and worked in the Russian Empire. The sign is present when slight rubbing of the skin results in exfoliation of the outermost layer. A typical test would be to place the eraser of a pencil on the roof of a lesion and spin the pencil in a rolling motion between the thumb and forefinger. If the lesion is opened, then the Nikolsky's sign is present/positive.

Pemphigus vulgaris chronic blistering skin disease

Pemphigus vulgaris is a rare chronic blistering skin disease and the most common form of pemphigus. Pemphigus was derived from the Greek word pemphix, meaning blister. It is classified as a type II hypersensitivity reaction in which antibodies are formed against desmosomes, components of the skin that function to keep certain layers of skin bound to each other. As desmosomes are attacked, the layers of skin separate and the clinical picture resembles a blister. These blisters are due to acantholysis, or breaking apart of intercellular connections through an autoantibody-mediated response. Over time the condition inevitably progresses without treatment: lesions increase in size and distribution throughout the body, behaving physiologically like a severe burn.

Hailey–Hailey disease, or familial benign chronic pemphigus or familial benign pemphigus, was originally described by the Hailey brothers in 1939. It is a genetic disorder that causes blisters to form on the skin.

Pemphigoid

Pemphigoid is a group of rare autoimmune blistering diseases of the skin, and mucous membranes. As its name indicates, pemphigoid is similar in general appearance to pemphigus, but, unlike pemphigus, pemphigoid does not feature acantholysis, a loss of connections between skin cells.

Desquamative gingivitis is an erythematous (red), desquamatous (shedding) and ulcerated appearance of the gums. It is a descriptive term and can be caused by several different disorders.

Gestational pemphigoid dermatosis of pregnancy

Gestational pemphigoid (GP) is an autoimmune blistering skin disease of pregnancy, typically occurring in the second or third trimester. It was originally called herpes gestationis because of the blistering appearance, although it is not associated with the herpes virus. It is one of the pemphigoid (pemphigus-like) diseases.

Discoid lupus erythematosus

Discoid lupus erythematosus is the most common type of chronic cutaneous lupus (CCLE), an autoimmune skin condition on the lupus erythematosus spectrum of illnesses. It presents with red, inflamed, coin-shaped patches of skin with a scaling and crusty appearance, most often on the scalp, cheeks, and ears. Hair loss may occur if the lesions are on the scalp. The lesions can then develop severe scarring, and the centre areas may appear lighter in color with a rim darker than the normal skin. These lesions can last for years without treatment.

Pemphigus foliaceus is an autoimmune blistering disease of the skin. Pemphigus foliaceus causes a characteristic inflammatory attack at the subcorneal layer of epidermis, which results in skin lesions that are scaly or crusted erosions with an erythematous (red) base. Mucosal involvement is absent even with widespread disease.

Paraneoplastic pemphigus (PNP) is an autoimmune disorder stemming from an underlying tumor. It is hypothesized that antigens associated with the tumor trigger an immune response resulting in blistering of the skin and mucous membranes.

Ernst H. Beutner German-born microbiologist

Ernst H. Beutner was a German-born microbiologist who discovered the role of autoimmunity in pemphigus and pemphigoid using self-designed immunofluorescent methods. For this achievement, he is often regarded as the "Founder of Immunodermatology". He was the author or co-author of over 10 papers, which were each cited over 100 times.

Autoimmune skin disease in dogs are a group of diseases that occur in dogs that are caused by the body's immune system, where the body's white blood cells or body's antibodies attack its own tissues or extracellular protein of the skin.

References

  1. 1 2 3 Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN   978-1-4160-2999-1.
  2. "Pemphigoid, Benign Mucous Membrane". MeSH. 2019. Retrieved 22 July 2019.
  3. Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). McGraw-Hill. ISBN   0-07-138076-0.
  4. Chen, Peggy. "Brunsting-Perry cicatricial pemphigoid". dermnetz.org. Retrieved 21 July 2019.
  5. 1 2 3 4 Scully, Crispian; Lo Muzio, Lorenzo (30 June 2008). "Oral mucosal diseases: Mucous membrane pemphigoid". British Journal of Oral and Maxillofacial Surgery. 46 (5): 358–366. doi:10.1016/j.bjoms.2007.07.200. PMID   17804127.
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