Bullous pemphigoid: Difference between revisions
Kghaffarian (talk | contribs) (changed from no mucosal involvement to "involves mucosa") |
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*May be intensely pruritic | *May be intensely pruritic | ||
*Nikolsky sign negative | *Nikolsky sign negative | ||
*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 | ||
Revision as of 13:23, 28 May 2015
Background
- Chronic autoimmune blistering disease
- Typically older than 60 years of age
- Blisters occur deep, within the epidermal basement membrane,
- Bullae evolve over weeks to months
Clinical Presentation
- Bullae commonly occur in the axillae, abdomen, inner thighs, flexural forearms, and lower legs[1]
- May be intensely pruritic
- 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 pruritis
- 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
- Topical high potency steroids such as Clobetasol
- Immunosuppressants (eg, azathioprine, methotrexate, mycophenolate mofetil, cyclophosphamide).
- Most will require therapy for 6-60 months, mortality asso w disease usually secondary to medications
Topical corticosteroids may offer similar treatment effect as oral steroids with less systemic effects.[2][3][4]
- Derm referral for dx via biopsy
Sources
- ↑ 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].