Bullous pemphigoid

Bullous pemphigoid is an autoimmune skin disorder producing chronic, pruritic bullous eruptions in elderly patients. Usually on the arms, legs or trunk. Without treatment, bullous pemphigoid may persist, with periods of remission and flare-ups, for many years.

Symptoms and signs

• Characteristic tense bullae develop on normal-appearing or erythematous skin, most often in flexural areas
• Redness
• Multiple blisters
• Itching
• Hive-like lesions
• Rashes
• Bleeding gums
• Irritation
• Mouth sores
• Nikolsky's sign, in which lateral pressure on skin adjacent to a blister causes epidermal detachment, is negative.

Causes

In bullous pemphigoid, antibodies are directed against the basement membrane zone of the epidermis, causing separation between the epidermis and dermis

Diagnosis and tests

• Skin biopsy 

• Serum antibody titers for hemidesmosomal BP antigens BP230 (BPAg1) and BP180 (BPAg2)

Prognosis

Prognosis is good, and the disease usually subsides within months to years; however, the disease is potentially fatal, especially in the elderly and debilitated patients, with death being caused by infection and sepsis or the effects of the drugs

Treatment

• Corticosteroids oral or topical form helps relieve inflammation
• Immunosuppressants like azathioprine, mycophenolate mofetil, cyclosporine, rituiximab
• Corticosteroid-sparing agents- dapsone and tetracycline medications, as well as immunosuppressant drugs in more severe cases.