Bullous pemphigoid
Background
- Chronic autoimmune blistering disease
- Blisters occur deep, within the epidermal basement membrane
- Bullae evolve over weeks to months
Risk factors
- Age > 60
- Female
- Malignancy
- Furosemide
Clinical Features
- Bullae commonly occur in the axillae, abdomen, inner thighs, flexural forearms, and lower legs[1]
- May be intensely pruritic
- Start with urticarial lesions
- Then tense blisters/bullae up to 10 cm
- Nikolsky sign negative
- Reported after UV/radiation therapy, drugs: furosemide, NSAIDs, captopril, antibiotics, vaccinations
- Involves mucosa in 10-25%, may limit PO intake
Differential Diagnosis
Vesiculobullous rashes
Febrile
- Diffuse distribution
- Varicella
- Smallpox
- Disseminated gonococcal disease
- DIC
- Purpural fulminans
- Localized distribution
Afebrile
- Diffuse distribution
- Bullous pemphigoid
- Drug-Induced bullous disorders
- Pemphigus vulgaris
- Phytophotodermatitis
- Erythema multiforme major
- Bullous impetigo
- Localized distribution
Management
- Antihistamine for pruritus
- Anti-inflammatory agents options
- Oral corticosteroids such as prednisone 50mg PO daily
- Tetracycline 1.5-2 g/day with nicotinamide 1.5-2 g/day
- Dapsone 50mg daily
- High potency topical steroids such as clobetasol
- Topical corticosteroids may offer similar treatment effect as oral steroids with less systemic effects.[2][3][4]
- Immunosuppressants (eg, azathioprine, methotrexate, mycophenolate mofetil, cyclophosphamide).
- Most will require therapy for 6-60 months, mortality associated with disease usually secondary to medications
- Derm referral for diagnosis via biopsy
References
- http://emedicine.medscape.com/article/1062391-overview
- 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].
- 2. 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
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