Bullous pemphigoid: Difference between revisions
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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 | ===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]] | ||
**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''' | ||
*Reported after UV/radiation therapy, drugs: furosemide, NSAIDs, captopril, antibiotics, vaccinations | *Reported after UV/radiation therapy, drugs: furosemide, NSAIDs, captopril, antibiotics, vaccinations | ||
*Involves mucosa in 10-25%, may limit PO intake | *Involves mucosa in 10-25%, may limit PO intake | ||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
| Line 23: | Line 24: | ||
==Management== | ==Management== | ||
*'''Antihistamine''' for | *'''[[Antihistamine]]''' for pruritus | ||
*'''Anti-inflammatory''' agents options | *'''Anti-inflammatory''' agents options | ||
**Oral | **Oral [[corticosteroids]] such as [[prednisone]] 50mg PO daily | ||
**[[Tetracycline]] 1.5-2 g/day with | **[[Tetracycline]] 1.5-2 g/day with nicotinamide 1.5-2 g/day | ||
** | **[[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]]). | |||
''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> | *Most will require therapy for 6-60 months, mortality associated with disease usually secondary to medications | ||
*Derm referral for | *Derm referral for diagnosis via biopsy | ||
== | ==References== | ||
<references/> | <references/> | ||
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
- Age > 60
- Female
- Malignancy
- Furosemide
Clinical Features
- 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
- Diffuse distribution
- Varicella (chickenpox)
- Smallpox
- Monkeypox
- Disseminated gonococcal disease
- DIC
- Purpural fulminans
- Localized distribution
Afebrile
- Diffuse distribution
- Bullous pemphigoid
- Drug-Induced bullous disorders
- Pemphigus vulgaris
- Phytophotodermatitis
- Erythema multiforme major
- Bullous impetigo
- Localized distribution
- Contact dermatitis
- Herpes zoster (shingles)
- Dyshidrotic eczema
- Burn
- Dermatitis herpetiformis
- Erythema multiforme minor
- Poison Oak, Ivy, Sumac dermatitis
- Bullosis diabeticorum
- Bullous impetigo
- Folliculitis
Management
- Antihistamine for pruritus
- Anti-inflammatory agents options
- Oral corticosteroids such as prednisone 50mg PO daily
- Tetracycline 1.5-2 g/day with nicotinamide 1.5-2 g/day
- Dapsone 50mg daily
- High potency topical steroids such as clobetasol
- Topical corticosteroids may offer similar treatment effect as oral steroids with less systemic effects.[2][3][4]
- 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
References
- ↑ http://emedicine.medscape.com/article/1062391-overview
- ↑ 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].
- ↑ 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].