Difference between revisions of "Bullous pemphigoid"

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==Background==
 
==Background==
 
*Chronic autoimmune blistering disease  
 
*Chronic autoimmune blistering disease  
*Typically older than 60 years of age
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*Blisters occur deep, within the epidermal basement membrane
*Blisters occur deep, within the epidermal basement membrane,
 
 
*Bullae evolve over weeks to months
 
*Bullae evolve over weeks to months
  
==Clinical Presentation==
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===Risk factors===
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*Age > 60
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*Female
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*Malignancy
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*[[Furosemide]]
 +
 
 +
==Clinical Features==
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[[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
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*May be intensely [[pruritus|pruritic]]
*Nikolsky sign negative
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**Start with [[urticaria|urticarial]] lesions
*No mucous membrane involvemet
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**Then tense blisters/bullae up to 10 cm
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*'''Nikolsky sign negative'''
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*Reported after UV/radiation therapy, drugs: furosemide, NSAIDs, captopril, antibiotics, vaccinations
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*Involves mucosa in 10-25%, may limit PO intake
  
 
==Differential Diagnosis==
 
==Differential Diagnosis==
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==Management==
 
==Management==
*'''Antihistamine''' for pruritis
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*'''[[Antihistamine]]''' for pruritus
 
*'''Anti-inflammatory''' agents options
 
*'''Anti-inflammatory''' agents options
**Oral Corticosteroids such as Prednisone 50mg PO daily
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**Oral [[corticosteroids]] such as [[prednisone]] 50mg PO daily
**[[Tetracycline]] 1.5-2 g/day with Nicotinamide 1.5-2 g/day  
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**[[Tetracycline]] 1.5-2 g/day with nicotinamide 1.5-2 g/day  
**Topical high potency steroids such as Clobetasol
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**[[Dapsone]] 50mg daily
*Immunosuppressants (eg, azathioprine, methotrexate, mycophenolate mofetil, cyclophosphamide).
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**High potency [[topical steroids]] such as [[clobetasol]]
*Most will require therapy for 6-60 months, mortality asso w disease usually secondary to medications
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*''Topical corticosteroids may offer similar treatment effect as oral steroids with less systemic effects.''<ref>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].</ref><ref>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]. </ref><ref> 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].</ref>
 
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*Immunosuppressants (eg, [[azathioprine]], [[methotrexate]], [[mycophenolate mofetil]], [[cyclophosphamide]]).
''Topical corticosteroids may offer similar treatment effect as oral steroids with less systemic effects.''<ref>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].</ref><ref>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]. </ref><ref> 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].</ref>
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*Most will require therapy for 6-60 months, mortality associated with disease usually secondary to medications
*Derm referral for dx via biopsy
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*Derm referral for diagnosis via biopsy
  
==Sources==
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==References==
 
<references/>
 
<references/>
  
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*[[Visual diagnosis (main)]]
 
*[[Visual diagnosis (main)]]
  
[[Category:Derm]]
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[[Category:Dermatology]]

Latest revision as of 20:19, 27 September 2019

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

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