Pemphigoid is a group of blistering skin conditions caused by the deposition of immune proteins in the basement membrane. It can be classified based on the type of immunoglobulin involved and is often diagnosed through skin biopsy and immunofluorescence. Treatment involves corticosteroids and other medications.
Pemphigoid is a term used to refer to a group of skin conditions that manifest primarily as blisters. Although similar in presentation to pemphigus, these blistering skin diseases occur without acantholysis, or the destruction of intercellular connections and cohesion of adjacent skin keratinocytes. This condition tends to occur more frequently in women and the elderly.
Diseases in this group are skin blisters or bullous diseases caused by the deposition of immune proteins called antibodies, or immunoglobulins, in the basement membrane of the epithelial layers. As such, they can be classified based on which immunoglobulin is involved. Pemphigoid gestationis (PG), bullous pemphigoid (BP), and mucous membrane or cicatricial pemphigoid (MMP/CP) all involve immunoglobulin G (IgG). Another classification called immunoglobulin A (IgA) pemphigoid involves IgA.
As the name suggests, PG, or herpes gestationis, is associated with pregnancy, usually appearing in the second or third trimester or soon after delivery. MMP/CP and BP generally affect people 60 years of age and older. While MMP/CP does not involve the skin, PA rarely affects the mouth.
In PA, skin involvement includes the inner surfaces of the thighs, flexor aspects of the forearms, groin, axillae, and lower legs, and may be associated with hive-like lesions and extreme pruritus or pruritus. Some people with this condition may get oral blisters, which appear after skin blisters. The lesions are tight blisters, or bullae, filled with a clear fluid and found on normal or reddish skin. These bubbles generally have an average diameter of 0.8 inches (2 cm), but can sometimes reach 1.6 to 3.1 inches (4 to 8 cm).
BP blisters only reach the subepidermis and not the dermis, so they are not prone to rupture or scarring. However, blister rupture leads to ulceration. This makes the skin prone to secondary bacterial infections that could reach the dermis, leading to scarring.
Pemphigoid is often a diagnosis of exclusion, given after inflammatory and infectious causes of acute or chronic blisters have been ruled out. Diagnosis involves proper examination of skin lesions, a skin biopsy, and an immunohistological method called direct immunofluorescence. On microscopic examination of biopsied skin, pemphigoid would show a vacuolated basal cell layer extending only subepidermally, without acantholysis. On direct immunofluorescence of the biopsy sample, desmoglein antibodies would be positive. An enzyme immunosorbent assay (ELISA) has been developed to detect antibodies to desmoglein present in blood.
Treatment of this chronic bladder disease involves the administration of topical or oral corticosteroids to reduce the production of antibodies. Examples include prednisolone and prednisone. Other treatment options include dapsone, erythromycin, cyclosporine, cyclophosphamide, azathioprine, and mycophenolate mofetil.
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