Bullous pemphigoid

Background

Normal dermal anatomy.
  • Chronic autoimmune blistering disease
  • Blisters occur deep, within the epidermal basement membrane
  • Bullae evolve over weeks to months

Risk factors

Clinical Features

Bullous pemphigoid.JPG
  • 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

Afebrile

Management

  • Antihistamine for pruritus
  • Anti-inflammatory agents options
  • Topical corticosteroids may offer similar treatment effect as oral steroids with less systemic effects.[2][3][4]
  • Immunosuppressants (eg, azathioprine, methotrexate, mycophenolate mofetil, cyclophosphamide).
  • IVIG is effective in bullous pemphigoid and can be given every 4 weeks, making it a feasible option for those with poor social support and/or limited access to healthcare or housing
  • Most will require therapy for 6-60 months, mortality associated with disease usually secondary to medications
  • Derm referral for diagnosis via biopsy

References

  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