Bullous pemphigoid is an skin condition which is more common than pemphigus. It affects the people in later life (usually over 60 years old) and the mucosal involvement is very rare. Auto antibodies against a 230 kDa or 180 kDa hemidesmosomal protein ('bullous pemphigoid antigens 1 and 2') play an aetiological role in Bullous pemphigoid.
Bullous pemphigoid is considered to be a chronic variant of erythema multiforme. In the absence of established etiology, there is some confusion and controversy about this clinical condition, which is thought to be of auto-immunologic origin. Skin biopsy shows a deeper blister (than in pemphigus) owing to a subepidermal split through the basement membrane. Direct and indirect IMF studies show linear staining of IgG along the basement membrane.
Large tense bullae appear anywhere on the skin but often involve limbs, hands and feet. They may be centred on an erythematous or urticated background and they can be haemorrhagic. Pemphigoid can be very itchy. Mucosal ulceration is uncommon but a variant of pemphigoid exists which predominantly affects mucosal surfaces with scarring (cicatricial pemphigoid).
It usually runs a benign course but may be fatal in elderly, debiliated people. Some patients may have a single attack persistent for weeks while others have recurrent attacks. Mortality rate is low, especially after the advent of corticosteroids.
Subepidermal pressure bullae without any acantholysis are characteristic. With regeneration of the epidermis at the floor of the bullae, intra-epidermal location may be come across. Usually, the bulla contains a network of fibrin with only a few inflammatory cells. Upper dermis shows oedema with little inflammatory infiltrate.
This is with high-dose oral prednisolone (30-60 mg daily) and steroid-sparing agents such as azathioprine. In general, disease control is easier than with pemphigus. Often, treatment can be withdrawn after 2-3 years. However, pemphigoid treatment often causes side-effects, especially as most patients are elderly. Occasionally localized disease can be controlled with potent topical steroids or oral dapsone.
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