An 87-year-old man presents for evaluation of an itchy rash affecting his trunk and extremities. The eruption has continued to intensify despite an intramuscular steroid injection. The patient recently started combination memantine and donepezil hydrochloride for treatment of early stage dementia; discontinuation of this medication had no effect on the dermatitis. Examination reveals multiple indurated, erythematous plaques.
Submit your diagnosis to see full explanation.
Bullous pemphigoid is the most common bullous autoimmune disease and typically affects elderly individuals. The condition is characterized by tense bullae that arise on erythematous or urticarial plaques. Atypical variants include pemphigoid nodularis, pemphigoid vegetans, papular pemphigoid, erythrodermic pemphigoid, and nonbullous pemphigoid. The nonbullous subtype commonly presents with severe pruritus and other nonbullous manifestations such as urticarial plaques, erythematous patches, and erythroderma.1 True incidence is uncertain and somewhat controversial; according to a study published in the British Journal of Dermatology, about 20% of all pemphigoid patients lacked the presence of bullae at the time of diagnosis.2
Cases of nonbullous pemphigoid often go undiagnosed for many months or even years.1 Diagnosis is confirmed by immunofluorescence performed on a skin biopsy specimen as well as by immunoserology.3 Treatment is similar to that used for bullous pemphigoid with oral prednisone being the mainstay of therapy. Some cases of nonbullous pemphigoid may respond to oral doxycycline.4
- Lamberts A, Meijer JM, Jonkman MF. Nonbullous pemphigoid: a systemic review. J Am Acad Dermatol. 2018;78(5):989-995.
- della Torre R, Combescure C, Cortés B, et al. Clinical presentation and diagnostic delay in bullous pemphigoid: a prospective nationwide cohort. Br J Dermatol. 2012;167(5):1111-1117.
- Meijer JM, Diercks GFH, de Lang EWG, Pas HH, Jonkman MF. Assessment of diagnostic strategy for early recognition of bullous and nonbullous variants of pemphigoid. JAMA Dermatol. 2019;155(2):158–165.
- Safa G, Darrieux L. Nonbullous pemphigoid treated with doxycycline monotherapy: report of 4 cases. J Am Acad Dermatol. 2011;64(6):116-118.