Pemphigoid Flashcards
what is the most common type of pemphigoid to affect the oral mucosa
mucous membrane pemphigoid
definition of pemphigoid
Group of immune mediated blistering diseases characterised by production of autoantibodies to components of hemi desmosomes and epithelial basement membrane
epidemiology of mucous membrane pemphigoid
women affected more than men
seen more commonly in older population, ages 50+
discuss the pathogenesis of mucous membrane pemphigoid
body produces autoantibodies to its own proteins, notable to BP180 and BP230 which are found in hemidesmosomes attaching epithelial cells to the basement membrane.
Autoantibodies, mainly IgG or IgA, bind to these proteins forming an immune complex which triggers the complement system which triggers recruitment of inflammatory cells to the area (causing formation of a blister)
discuss the extra oral manifestations of mucous membrane pemphigoid
ocular involvement - inflammation causing redness and irritation, severe cases can see scarring that can affect vision
skin involvement - rare, localised erythmatous plaques near affected mucosa
describe the intra oral manifestations of mucous membrane pemphigoid
bullae are sub epithelial so may be seen intact, upon rupture leave painful areas of erythema, erosion and ulceration
lesions can affect any site in oral cavity
may present as desquamative gingivitis
severe cases may see stricture (restricted movement) due to scarring
nikolskys sign
separation of superficial epithelial layers from the basal layers when lateral pressure is applied
discuss the histological findings in mucous membrane pemphigoid
hemidesmosomes are attacked meaning sub epithelial split is seen at epithelial-connective tissue junction. Full thickness separation of epithelium and space is infiltrated by immune cells (mainly eosinophils, neutrophils and lymphocytes)
what test is most useful for diagnosis of mucous membrane pemphigoid and what will it show
direct immunofluorescence
characteristic sign is linear binding of IgG and/ or C3 along basement membrane
other than direct immunofluorescence, what other tests may be done to aid diagnosis of mucous membrane pemphigoid
indirect immunofluorescence - detect circulating auto antibodies in well established disease
ELISA test: detecting circulating autoantibodies in serum
biopsy for histopathological analysis
discuss potential treatment for milder cases of mucous membrane pemphigoid
may be treatable with topical corticosteroids alone - various formulations e.g mouthwash, inhalers
Dapsone (antibiotic) may also be prescribed for mild disease
discuss potential treatment for mucous membrane pemphigoid that is not controlled by primary measures
systemic corticosteroid e.g prednisolone
often in conjunction with an immunosuppresive agent e.g azathioprine so steroid dose can be reduced without losing disease control
