Other variants of bullous pemphigoid include gestational pemphigoid and cicatricial pemphigoid. Gestational pemphigoid occurs during pregnancy and leads to bullae or urticarial lesions on the abdomen, trunk, and extremities, with mucous membrane sparing. Fetal loss is not increased; however, infants are often born prematurely and small for gestational age. Cicatricial pemphigoid rarely involves the skin and tends to affect mucous membranes, with ocular and oropharyngeal involvement, and it can lead to scarring and significant morbidity from blindness and airway obstruction.[3]
Treatment options for bullous pemphigoid include high-potency topical steroids, oral steroids (prednisone 0.5-1 mg/kg/d), antibiotics, steroid-sparing immunosuppressants (eg, azathioprine, mycophenolate mofetil, and methotrexate), plasmapheresis, and immunoglobulin infusions. Calcium and vitamin D supplements with a bisphosphonate should be given when treating with systemic steroids.[2] For acutely ill patients or those with extensive skin involvement, fluid replacement, thermoregulation, and infection management may be necessary.
Most patients with bullous pemphigoid are elderly; they may have multiple comorbidities and may be taking multiple medications. Treatment should be carefully tailored to minimize side effects yet control disease adequately.
A systematic review by Khumalo and colleagues[6] found that potent topical steroids were safe and effective in patients with bullous pemphigoid, with less systemic side effects than oral steroid treatment. Topical clobetasol propionate 0.05% (40 g/d) has been shown to be superior to oral prednisolone (0.5 mg/kg/d) in terms of overall survival, disease control, and adverse event profile for patients with extensive bullous pemphigoid. It is equally effective for patients with moderate bullous pemphigoid, as shown in a randomized controlled trial.[7] For this reason, topical clobetasol may be used as first-line treatment for bullous pemphigoid.
Side effects of steroid treatment can include diabetes mellitus, peptic ulcer disease, glaucoma, cataract formation, and agranulocytosis. The prognosis of patients with bullous pemphigoid is generally good. Disease is usually self-limited over 5-6 years.
The patient in this case was treated with high-potency topical steroids and experienced a good result. The medication was tapered over several weeks, and she regularly followed up with her healthcare provider.
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Cite this: Tuyyab Hassan. An 84-Year-Old With a Blistering Rash That Is Spreading - Medscape - Jun 28, 2016.
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