Difference between revisions of "Bullous pemphigoid"

(edits)
(Management)
 
(21 intermediate revisions by 7 users not shown)
Line 1: Line 1:
 
==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 (eg, corticosteroids, tetracyclines, dapsone)
+
*'''Anti-inflammatory''' agents options
*Immunosuppressants (eg, azathioprine, methotrexate, mycophenolate mofetil, cyclophosphamide).  
+
**Oral [[corticosteroids]] such as [[prednisone]] 50mg PO daily
*European article: evidence that strong topical corticosteroid treatment may achieve disease control while avoiding systemic adverse effects from systemic corticosteroids<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>
+
**[[Tetracycline]] 1.5-2 g/day with nicotinamide 1.5-2 g/day
*Derm referral for dx via biopsy
+
**[[Dapsone]] 50mg daily
 +
**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>
 +
*Immunosuppressants (eg, [[azathioprine]], [[methotrexate]], [[mycophenolate mofetil]], [[cyclophosphamide]]).
 +
*Most will require therapy for 6-60 months, mortality associated with disease usually secondary to medications
 +
*Derm referral for diagnosis via biopsy
  
==Sources==
+
==References==
*Medscape: http://emedicine.medscape.com/article/1062391-overview - last accessed Jan 2015
+
<references/>
*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].
 
*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].
 
*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==
 
==See Also==
Line 34: Line 42:
 
*[[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