Bullous pemphigoid

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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 Presentation

  • Bullae commonly occur in the axillae, abdomen, inner thighs, flexural forearms, and lower legs[1]
  • May be intensely pruritic
  • 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

Afebrile

Management

  • Antihistamine for pruritis
  • 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
    • Topical high potency steroids such as Clobetasol
  • Immunosuppressants (eg, azathioprine, methotrexate, mycophenolate mofetil, cyclophosphamide).
  • Most will require therapy for 6-60 months, mortality asso w disease usually secondary to medications

Topical corticosteroids may offer similar treatment effect as oral steroids with less systemic effects.[2][3][4]

  • Derm referral for dx via biopsy

Sources

  1. http://emedicine.medscape.com/article/1062391-overview
  2. 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].
  3. 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].
  4. 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