Bullous pemphigoid

Background

• Chronic autoimmune blistering disease
• Typically older than 60 years of age
• Blisters occur deep, within the epidermal basement membrane,
• Bullae evolve over weeks to months

Clinical Presentation

• Bullae commonly occur in the axillae, abdomen, inner thighs, flexural forearms, and lower legs
• May be intensely pruritic
• Nikolsky sign negative
• No mucous membrane involvemet

Differential Diagnosis

• Cicatricial Pemphigoid
• Dermatitis Herpetiformis
• Drug-Induced Bullous Disorders
• Erythema Multiforme

Management

• Antihistamine for pruritis
• Anti-inflammatory agents (eg, corticosteroids, tetracyclines, dapsone)
• Immunosuppressants (eg, azathioprine, methotrexate, mycophenolate mofetil, cyclophosphamide).
• European article: evidence that strong topical corticosteroid treatment may achieve disease control while avoiding systemic adverse effects from systemic corticosteroids
• Derm referral for dx via biopsy

Sources

• Medscape: http://emedicine.medscape.com/article/1062391-overview - last accessed Jan 2015
• Joly P, Roujeau JC, Benichou J, et al. A comparison of oral and topical corticosteroids in patients with bullous pemphigoid. N Engl J Med. Jan 31 2002;346(5):321-7. [Medline].
• Terra JB, Potze WJ, Jonkman MF. Whole body application of a potent topical corticosteroid for bullous pemphigoid. J Eur Acad Dermatol Venereol. Apr 3 2013;[Medline].
• Gual A, Iranzo P, Mascaró Jr JM. Treatment of bullous pemphigoid with low-dose oral cyclophosphamide: a case series of 20 patients. J Eur Acad Dermatol Venereol. Apr 13 2013;[Medline].

See Also

• Fever and Rash
• Visual diagnosis (main)