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Pemphigoid
Pemphigoid is a group of uncommon and rare autoimmune blistering skin diseases. As its name
indicates, pemphigoid is similar in general appearance to pemphigus, but, unlike pemphigus,
pemphigoid does not feature acantholysis.
Pemphigoid is more common than pemphigus, and is slightly more common in women than in men. It
is also more common in people over 60 years of age than it is in younger people.
Bullous pemphigoid (BP) Rarely affect the mouth
Bullous pemphigoid is an acute or chronic autoimmune skin disease, involving the formation
of blisters, more appropriately known as bullae, at the space between the skin
layers epidermis and dermis. It is classified as a type II hypersensitivity reaction.
pathophysiology
The bullae are formed by an immune reaction, initiated by the formation of IgG autoantibodies
targeting Dystonin, also called Bullous Pemphigoid Antigen 1, and/or type XVII collagen, also called
Bullous Pemphigoid Antigen 2.] which is a component of hemidesmosomes. A different form of
dystonin is associated with neuropathy]. Following antibody targeting, a cascade
ofimmunomodulators results in a variable surge of immune cells,
including neutrophils, lymphocytes and eosinophils coming to the affected area. Unclear events
subsequently result in a separation along the dermoepidermal junction and eventually stretch bullae.
Relation with oral micosa
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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.
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.
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
(Pemphigus) the epithelium tends to disintegrate rather than form a bulla.
Nikolsky's sign is present in case of pemphigus only but not in the case of pemphigoid.
Diagnostic techniques:
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antibodies (IgG) precipitates complement (C3) in the lamina lucida of the basement membrane.
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Circulating auto-antibodies to BP-1 antigen (located in hemidesmosome). 50% have BP-2.
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Negative Nikolsky sign.
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IgG, C3 deposition at BM creating smooth line in immunofluorescent analysis.
Cicatricial pemphigoid
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