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Cicatricial Pemphigoid
What is cicatricial pemphigoid?
Cicatricial pemphigoid is a chronic vesiculobullous disease with
a tendency to affect the mucous membranes of the eyes and mouth. Scarring
often results. Other mucosal surfaces may be involved as well, including the
esophagus, upper airways, anus, and external genitalia. Chronic inflammation of
the oral mucosa may lead to loss of teeth.
With what can cicatricial pemphigoid be confused?
Cicatricial pemphigoid can be confused clinically with other
bullous disease, such as bullous pemphigoid,
linear IgA bullous disease,
epidermolysis bullosa acquisita, and
dermatitis herpetiformis. Oral
lesions may be difficult to distinguish from oral
lichen planus or pemphigus vulgaris.
How is cicatricial pemphigoid diagnosed?
The mouth is most often involved, with erosions, ulcerations,
vesicles, and bullae. Ocular involvement is common, as well. Scarring of
the cornea and conjunctiva can result in blindness. Skin lesions are
present in about 25% of cases, but is rarely the initial site of involvement.
When skin lesions are present, the face, scalp, neck and upper trunk are the
most frequently involved sites. Multiple autoantibodies may lead to
the clinical phenotype of cicatricial pemphigoid. Direct
immunofluorescence is helpful is necessary to determine which epitopes are
involved. Multiple site on the C-terminal domain BP180 protein are
implicated in some instances (in contrast to the NC16 domain involved in
bullous pemphigoid). IgG antibodies to
epiligrin (laminin 5) is involved in 10% of cases. Direct immunofluorescence
usually reveals linear IgG at the basement membrane zone.
On histopathology, a subepidermal blister is present, often with
scarring evident in the dermis. Early lesions have a neutrophilic
infiltrate, while older lesions tend toward eosinophils, but less so than is
seen in bullous pemphigoid. Circulating
antibodies to these antigens are often present, and correlate with disease
activity.
How is cicatricial pemphigoid treated?
Cicatricial pemphigoid is difficult to treat. Potent topical
corticosteroids are used to control local
disease. Systemic medications may be required for severe disease, including:
dapsone,
cyclophosphamide, and
azathioprine. Systemic
steroids alone are usually insufficient for
severe disease and should be combined with another agent, such as
cyclophosphamide.
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What is the prognosis for cicatricial pemphigoid?
Cicatricial pemphigoid is usually a chronic disease.
Though scarring and disability often occur, death is rare. Ocular involvement
and vision impairment often result. Weight loss may occur due to
dysphagia.
Date created 11/18/2007
Last updated
01/03/2009
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