A 77-year-old woman is sent for consultation from a nursing home for evaluation of skin blisters and erosions. She is unable to provide meaningful history, but according to the nursing records, the condition has been present several weeks and is being treated with a topical antibiotic ointment. She is currently taking multiple oral medications for control of hypertension, diabetes, and hyperlipidemia. Examination reveals multiple tense blisters and denuded patches.
Bullous PemphigoidThe disease classification pemphigoids includes autoimmune bullous diseases characterized by subepidermal blistering. Bullous pemphigoid is the most frequently seen condition within this group and is also the most common autoimmune-based blistering disease overall.1,2,3 The average age of onset is...
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The disease classification pemphigoids includes autoimmune bullous diseases characterized by subepidermal blistering. Bullous pemphigoid is the most frequently seen condition within this group and is also the most common autoimmune-based blistering disease overall.1,2,3 The average age of onset is 65 years.
Bullous pemphigoid is manifested as tense blisters arising on normal-appearing skin or on an erythematous base. These blisters may be pruritic and most commonly involve flexural surfaces. Diagnosis is made via histology and immunofluorescence showing subepidermal blistering, an eosinophilic predominance of inflammatory infiltrate, immunoglobulin G deposits along the dermoepidermal junction, and immunoglobulin G circulating autoantibodies.2,4
Treatment of bullous pemphigoid aims to dampen autoantibody production and lessen inflammation and bullae formation. Topical steroids may be effective for localized disease, whereas more extensive disease often requires oral prednisone with or without oral immunosuppressant agents. Some cases may respond to doxycycline.5 Rituximab, an anti-CD20 antibody, shows promise for control of refractory pemphigoid.6 Without treatment the disease is often self-limiting but may last for several months to years.2,4
Ken Anderson is a physician assistant student at Arcadia University.
1. Schiavo L, Ruocco E, Brancaccio G, Caccavale S, Rucocco V, Wolf R. Bullous pemphigoid: etiology, pathogenesis, and inducing factors: facts and controversies. Clin Dermatol. 2013;31(4):391-399.
2. Khandpur S, Verma P. Bullous pemphigoid. Indian J Dermatol. 2011;77(4):450-455.
3. Asbrink E, Hovmark A. Clinical variations in bullous pemphigoid with respect to early symptoms. Acta Derm Venereol. 1981;61(5):417-421.
4. Kirtschig G, Khumalo NP. Management of bullous pemphigoid: recommendations for immunomodulatory treatments. Am J Clin Dermatol. 2004;5(5):319-326.
5. Grantham HJ, Stocken DD, Reynolds NJ. Doxycycline: a first-line treatment for bullous pemphigoid? Lancet. 2017;389(10079):1586-1588.
6. Lamberts A, Euverman HI, Terra JB, Jonkman MF, Horváth B. Effectiveness and safety of rituximab in recalcitrant pemphigoid diseases. Front Immunol. 2018;9:248.