Derm Dx: Can You Diagnose These Itchy, Blistering Lesions?

Bullous pemphigoid is an autoimmune disease that primarily affects elderly individuals.1,2 Males and females are affected equally with no racial predilection. Bullae containing serous or hemorrhagic fluid present as tense oval or round lesions that can arise on normal-appearing, urticarial,...

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Bullous pemphigoid is an autoimmune disease that primarily affects elderly individuals.1,2 Males and females are affected equally with no racial predilection. Bullae containing serous or hemorrhagic fluid present as tense oval or round lesions that can arise on normal-appearing, urticarial, or erythematous areas of skin. Itching is variable but may be severe. Diagnosis is confirmed with histopathologic analysis and immunofluorescence studies that demonstrate subepidermal blistering, an inflammatory infiltrate with a predominance of eosinophils, and IgG-circulating autoantibodies.2,3

Bullous pemphigoid may be associated with infectious diseases including hepatitis and HIV as well as PD-1 inhibitors.4 According to a study published in JAMA Dermatology, onset of bullous pemphigoid may be linked to administration of dipeptidyl peptidase 4-inhibitors.5

Without treatment, the disease may persist for several months to years. High potency topical steroids may control limited cases of the disease. When this form of treatment is impractical or ineffectual, oral prednisone is the mainstay of therapy, although mild-to-moderate cases may be managed with oral doxycycline.6 Rituximab has proven valuable for control of refractory disease.7

Lauren Ax, MSPAS, PA-C, is a physician assistant on staff at the DermDox Center for Dermatology in Hazleton, Pennsylvania, and Stephen Schleicher, MD, is director of the DermDox Center for Dermatology, as well as an associate professor of medicine at Commonwealth Medical College and a clinical instructor of dermatology at Arcadia University and Kings College.

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References

1. Lo Schiavo A, 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 Venereol Leprol. 2011;77(4):450-455.

3. Kirtschig G, Khumalo NP. Management of bullous pemphigoid: recommendations for immunomodulatory treatments. Am J Clin Dermatol. 2004;5(5):319-326.

4. Lopez AT, Khanna T, Antonov N;,Audrey-Bayan C, Geskin L. A review of bullous pemphigoid associated with PD-1 and PD-L1 inhibitors. Int J Dermatol.  2018; 57(6):664-669.

5. Lee SG, Lee HJ, Yoon MS, Kim DH. Association of dipeptidyl peptidase 4 inhibitor use with risk of bullous pemphigoid in patients with diabetes. JAMA Dermatol. 2019;155(2):172-177.  

6. Williams HC, Wonjnarowska F, Kirtschig G, et al.Doxycycline versus prednisolone as an initial treatment strategy for bullous pemphigoid: a pragmatic, non-inferiority, randomised controlled trial. Lancet. 2017;389(10079):1630-1638.

7. Lamberts A, Euverman HI, Terra JB, Jonkman MF, Horváth B. Effectiveness and safety of rituximab in recalcitrant pemphigoid diseases. Front Immunol. 2018;9:248.