Difference between revisions of "Bullous pemphigoid"

(Background)
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[[File:bullous pemphigoid.JPG|thumbnail]]
 
[[File:bullous pemphigoid.JPG|thumbnail]]
 
*Bullae commonly occur in the axillae, abdomen, inner thighs, flexural forearms, and lower legs<ref>http://emedicine.medscape.com/article/1062391-overview</ref>
 
*Bullae commonly occur in the axillae, abdomen, inner thighs, flexural forearms, and lower legs<ref>http://emedicine.medscape.com/article/1062391-overview</ref>
*May be intensely pruritic
+
*May be intensely [[pruritus|pruritic]]
**Start with urticarial lesions
+
**Start with [[urticaria|urticarial]] lesions
 
**Then tense blisters/bullae up to 10 cm
 
**Then tense blisters/bullae up to 10 cm
 
*'''Nikolsky sign negative'''
 
*'''Nikolsky sign negative'''
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**Oral Corticosteroids such as Prednisone 50mg PO daily
 
**Oral Corticosteroids such as Prednisone 50mg PO daily
 
**[[Tetracycline]] 1.5-2 g/day with Nicotinamide 1.5-2 g/day  
 
**[[Tetracycline]] 1.5-2 g/day with Nicotinamide 1.5-2 g/day  
**Topical high potency steroids such as Clobetasol
+
**High potency [[topical steroids]] such as [[clobetasol]]
*Immunosuppressants (eg, azathioprine, methotrexate, mycophenolate mofetil, cyclophosphamide).  
+
*Immunosuppressants (eg, [[azathioprine]], [[methotrexate]], [[mycophenolate mofetil]], [[cyclophosphamide]]).  
 
*Most will require therapy for 6-60 months, mortality asso w disease usually secondary to medications
 
*Most will require therapy for 6-60 months, mortality asso w disease usually secondary to medications
  

Revision as of 23:19, 13 November 2016

Background

  • 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

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

  • 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