Cicatricial pemphigoid
| Cicatricial pemphigoid | |
|---|---|
| Classification and external resources | |
| Specialty | dermatology |
| ICD-10 | L12.1 |
| ICD-9-CM | 694.6 |
| OMIM | 164185 |
| DiseasesDB | 30757 |
| Patient UK | Cicatricial pemphigoid |
| MeSH | D010390 |
Cicatricial pemphigoid (also known as "Mucous Membrane Pemphigoid", "MMP", "Benign mucosal pemphigoid," "Benign mucous membrane pemphigoid,"[1] "Ocular pemphigus,"[1] and "Scarring pemphigoid"[1]) is a rare chronic autoimmune subepithelial blistering disease characterized by erosive skin lesions of the mucous membranes and skin that results in scarring of at least some sites of involvement.[2]:581
Cicatricial pemphigoid has been referred to by a variety of designations based largely on its site of involvements, with examples of such terminology including "desquamative gingivitis," "ocular pemphigus," and "benign mucous membrane pemphigoid." However, currently "...such designations are thought to be confusing or somewhat misleading (e.g., pemphigus in this context is a misnomer, and this disorder is hardly benign given the extent of morbidity it can cause)." [2]:581
Signs and symptoms
The autoimmune reaction most commonly affects the mouth, causing lesions in the gingiva or gums, but it can also affect areas of mucous membrane elsewhere in the body, such as the sinuses, genitals and anus. When the cornea of the eye is affected, repeated scarring may result in blindness.
Localized cicatricial pemphigoid (also known as "Brunsting–Perry cicatricial pemphigoid"[1]) refers to a localised variant of cutaneous cicatricial pemphigoid involving the head and the neck without mucosal involvement.[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 case of Pemphigus and Cicatricial Pemphigoid, but not in the case of 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:
- antibodies (IgG) precipitates 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
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.[3]
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 periodontal scaling.[3]
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.[3]
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.[3]
See also
- Bullous pemphigoid
- Gestational pemphigoid
- List of cutaneous conditions
- List of target antigens in pemphigoid
- List of immunofluorescence findings for autoimmune bullous conditions
References
- 1 2 3 4 5 Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.
- 1 2 Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). McGraw-Hill. ISBN 0-07-138076-0.
- 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.