Mucous membrane pemphigoid

Autoimmune blistering disease

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Medical condition

Mucous membrane pemphigoid

Other names| Cicatricial pemphigoid; MMP, Benign mucosal pemphigoid, Benign mucous membrane pemphigoid,[1] Ocular pemphigoid,[2] and Scarring pemphigoid[1])
Specialty| Dermatology

Mucous membrane pemphigoid is a rare chronic autoimmune subepithelial blistering disease characterized by erosive lesions of the mucous membranes and skin.[3] It is one of the pemphigoid diseases that can result in scarring.[4]

## Signs and symptoms

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The autoimmune reaction most commonly affects the oral mucosa in the mouth, causing lesions in the gums (gingiva), known as desquamative gingivitis. More severe cases can also affect areas of mucous membrane elsewhere in the body, such as the sinuses, genitals, anus, and cornea.[5] 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.[6] [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 mucous membrane pemphigoid, but not in bullous pemphigoid.

## Pathophysiology

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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

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Diagnostic techniques:

* antibodies (IgG) precipitate complement (C3) in the lamina lucida of the basement membrane.
* Circulating auto-antibodies to BP-1 antigen (located in hemidesmosome). 50% have BP-2.
* Positive Nikolsky sign.
* IgG, C3 deposition at BM creating smooth line in immunofluorescent analysis.

## Management

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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.[7]

### Conservative

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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.[7]

### Medications

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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.[7]

### Other treatments

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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.[7]

## See also

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* Gestational pemphigoid
* List of cutaneous conditions
* List of target antigens in pemphigoid
* List of immunofluorescence findings for autoimmune bullous conditions

## References

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1. ^ a b c 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. ^ Holtsche, MM; Zillikens, D; Schmidt, E (January 2018). "[Mucous membrane pemphigoid]". Der Hautarzt; Zeitschrift für Dermatologie, Venerologie, und verwandte Gebiete. 69 (1): 67–83\. doi:10.1007/s00105-017-4089-y. PMID 29242962. S2CID 195668609.67-83&rft.date=2018-01&rft_id=https://api.semanticscholar.org/CorpusID:195668609#id-name=S2CID&rft_id=info:pmid/29242962&rft_id=info:doi/10.1007/s00105-017-4089-y&rft.aulast=Holtsche&rft.aufirst=MM&rft.au=Zillikens,+D&rft.au=Schmidt,+E&rfr_id=info:sid/en.wikipedia.org:Mucous+membrane+pemphigoid" class="Z3988">
4. ^ Freedberg (2003). Fitzpatrick's dermatology in general medicine (6th. ed.). McGraw-Hill, Medical Pub. Division. p. 581\. ISBN 0-07-138076-0.
5. ^ "Mucous Membrane Pemphigoid". British Skin Foundation.
6. ^ Chen, Peggy. "Brunsting-Perry cicatricial pemphigoid". dermnetz.org. Retrieved 21 July 2019.
7. ^ a b c d 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 17804127.358-366&rft.date=2008-06-30&rft_id=info:doi/10.1016/j.bjoms.2007.07.200&rft_id=info:pmid/17804127&rft.aulast=Scully&rft.aufirst=Crispian&rft.au=Lo+Muzio,+Lorenzo&rfr_id=info:sid/en.wikipedia.org:Mucous+membrane+pemphigoid" class="Z3988">

## External links

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Classification|

* ICD-10: L12.1
* ICD-9-CM: 694.6
* OMIM: 164185
* MeSH: D010390
* DiseasesDB: 30757

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External resources|

* Patient UK: Mucous membrane pemphigoid

* v
* t
* e

Diseases of the skin and appendages by morphology

Growths|

| Epidermal|

* Wart
* Callus
* Seborrheic keratosis
* Acrochordon
* Molluscum contagiosum
* Actinic keratosis
* Squamous-cell carcinoma
* Basal-cell carcinoma
* Merkel-cell carcinoma
* Nevus sebaceous
* Trichoepithelioma

|

Pigmented|

* Freckles
* Lentigo
* Melasma
* Nevus
* Melanoma
* Postinflammatory hyperpigmentation

Dermal and
subcutaneous|

* Epidermal inclusion cyst
* Hemangioma
* Dermatofibroma (benign fibrous histiocytoma)
* Keloid
* Lipoma
* Neurofibroma
* Xanthoma
* Kaposi's sarcoma
* Infantile digital fibromatosis
* Granular cell tumor
* Leiomyoma
* Lymphangioma circumscriptum
* Myxoid cyst

Rashes|

| With
epidermal
involvement|

| Eczematous|

* Contact dermatitis
* Atopic dermatitis
* Seborrheic dermatitis
* Stasis dermatitis
* Lichen simplex chronicus
* Darier's disease
* Glucagonoma syndrome
* Langerhans cell histiocytosis
* Lichen sclerosus
* Pemphigus foliaceus
* Wiskott–Aldrich syndrome
* Zinc deficiency

|

Scaling|

* Psoriasis
* Tinea (Corporis
* Cruris
* Pedis
* Manuum
* Faciei)
* Pityriasis rosea
* Secondary syphilis
* Mycosis fungoides
* Systemic lupus erythematosus
* Pityriasis rubra pilaris
* Parapsoriasis
* Ichthyosis

Blistering|

* Herpes simplex
* Herpes zoster
* Varicella
* Bullous impetigo
* Acute contact dermatitis
* Pemphigus vulgaris
* Bullous pemphigoid
* Dermatitis herpetiformis
* Porphyria cutanea tarda
* Epidermolysis bullosa simplex

Papular|

* Scabies
* Insect bite reactions
* Lichen planus
* Miliaria
* Keratosis pilaris
* Lichen spinulosus
* Transient acantholytic dermatosis
* Lichen nitidus
* Pityriasis lichenoides et varioliformis acuta

Pustular|

* Acne vulgaris
* Rosacea
* Folliculitis
* Impetigo
* Candidiasis
* Gonococcemia
* Dermatophyte
* Coccidioidomycosis
* Subcorneal pustular dermatosis

Hypopigmented|

* Tinea versicolor
* Vitiligo
* Pityriasis alba
* Tuberous sclerosis
* Idiopathic guttate hypomelanosis
* Leprosy
* Hypopigmented mycosis fungoides

Without
epidermal
involvement|

| Red|

| Blanchable
Erythema|

| Generalized|

* Drug eruptions
* Viral exanthems
* Toxic erythema
* Systemic lupus erythematosus

|

Localized|

* Cellulitis
* Abscess
* Boil
* Erythema nodosum
* Carcinoid syndrome
* Fixed drug eruption

Specialized|

* Urticaria
* Erythema (Multiforme
* Migrans
* Gyratum repens
* Annulare centrifugum
* Ab igne)

Nonblanchable
Purpura|

| Macular|

* Thrombocytopenic purpura
* Actinic/solar purpura

|

Papular|

* Disseminated intravascular coagulation
* Vasculitis

Indurated|

* Scleroderma/morphea
* Granuloma annulare
* Lichen sclerosis et atrophicus
* Necrobiosis lipoidica

Miscellaneous
disorders|

| Ulcers|

|

Hair|

* Telogen effluvium
* Androgenic alopecia
* Alopecia areata
* Systemic lupus erythematosus
* Tinea capitis
* Loose anagen syndrome
* Lichen planopilaris
* Folliculitis decalvans
* Acne keloidalis nuchae

Nail|

* Onychomycosis
* Psoriasis
* Paronychia
* Ingrown nail

Mucous
membrane|

* Aphthous stomatitis
* Oral candidiasis
* Lichen planus
* Leukoplakia
* Pemphigus vulgaris
* Mucous membrane pemphigoid
* Cicatricial pemphigoid
* Herpesvirus
* Coxsackievirus
* Syphilis
* Systemic histoplasmosis
* Squamous-cell carcinoma

* v
* t
* e

Vesiculobullous disease

Acantholysis
(epidermis)|

| Pemphigus|

* Pemphigus vulgaris: Pemphigus vegetans
* of Hallopeau
* of Neumann

* Pemphigus foliaceus: Pemphigus erythematosus
* Endemic pemphigus

* Paraneoplastic pemphigus

* IgA pemphigus
* Subcorneal pustular
* Intraepidermal neutrophilic

Other|

* Transient acantholytic dermatosis

Pemphigoid
(dermis)|

| IgG:|

* Bullous pemphigoid
* Cicatricial pemphigoid
* Localised
* Gestational pemphigoid
* Pemphigoid nodularis
* Epidermolysis bullosa acquisita

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IgA:|

* Linear IgA bullous dermatosis
* Childhood
* Adult

Other bullous|

* Dermatitis herpetiformis

In diseases
classified elsewhere|

* Porphyria cutanea tarda
* Bullous lupus erythematosus
* PUVA-induced acrobullous dermatosis

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