Bullous pemphigoid: Difference between revisions

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==Background==
==Background==
*Chronic autoimmune blistering disease  
*Chronic autoimmune blistering disease  
*Typically older than 60 years of age
*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==
===Risk factors===
*Age > 60
*Female
*Malignancy
*[[Furosemide]]
 
==Clinical Features==
[[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]]
*Nikolsky sign negative
**Start with [[urticaria|urticarial]] lesions
*No mucous membrane involvemet
**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==
==Differential Diagnosis==
*Cicatricial Pemphigoid
{{Bullous rashes DDX}}
*Dermatitis Herpetiformis
*Drug-Induced Bullous Disorders
*[[Erythema Multiforme]]


==Management==
==Management==
*'''[[Antihistamine]]''' for pruritis
*'''[[Antihistamine]]''' for pruritus
*'''Anti-inflammatory''' agents options
*'''Anti-inflammatory''' agents options
**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
**[[Dapsone]] 50mg daily
*Immunosuppressants (eg, azathioprine, methotrexate, mycophenolate mofetil, cyclophosphamide).
**High potency [[topical steroids]] such as [[clobetasol]]
 
*''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>
''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>
*Immunosuppressants (eg, [[azathioprine]], [[methotrexate]], [[mycophenolate mofetil]], [[cyclophosphamide]]).
*Derm referral for dx via biopsy
*Most will require therapy for 6-60 months, mortality associated with disease usually secondary to medications
*Derm referral for diagnosis via biopsy


==Sources==
==References==
<references/>
<references/>


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*[[Visual diagnosis (main)]]
*[[Visual diagnosis (main)]]


[[Category:Derm]]
[[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