- A 45-year-old woman presents to the physician's office for a painful and blistering rash. She reports having a 1-month history of nonhealing ulcers in her mouth, which she previously attributed to stress. However, she reports new ulcers in her mouth and new easily-ruptured blisters on her skin. Approximately 6 weeks ago, she reported starting a new drug, captopril, for management of her hypertension. On physical exam, she has dozens of flaccid bullae and erosions on her trunk and extremities; she has 3 ulcers along her buccal mucosa. She is started on systemic steroids and captopril is discontinued.
- Clinical definition
- pemphigus vulgaris (PV) is a potentially fatal autoimmune blistering disease characterized by painful mucocutaneous lesions
- most common in
- those of Mediterranean or Jewish descent
- autoantibodies (IgG) against desmoglein, a component of desmosome
- recall that desmosomes connect keratinocytes in the skin
- type II hypersensitivity reaction
- this disrupts keratinocyte adhesion and causes separation of epidermis
- Associated conditions
- drug use
- myasthenia gravis
- systemic lupus erythematosus
- without treatment, prognosis is poor
- with treatment, prognosis is improved
- nonhealing and nonscarring ulcers persisting for at least 1 month
- progressive development of blisters from mouth to the rest of the body
- Physical exam
- flaccid bullae that are easily ruptured
- superficial erosions of skin (ruptured bullae)
- often on scalp, face, and trunk
- mucosal ulcers
- positive Nikolsky sign
- extension of blister or sloughing of skin with blunt pressure or lateral traction of skin
- auto-antibodies detected in serum with enzyme-linked immunosorbent assay (ELISA)
- anti-desmoglein 3 (mucosal involvement)
- anti-desmoglein 1 (mucocutaneous involvement)
- separation of epidermal cells
- intraepidermal blister
- direct immunofluorescence
- intercellular deposition of IgG or C3 in a net-like (reticular) pattern
- Bullous pemphigoid
- less severe than PV
- does not affect mucous membranes
- negative Nikolsky sign
- wound care
- for all patients, to prevent secondary bacterial infection
- anesthetic nebulizers for mucosal lesions
- potent topical steroids
- systemic steroids
- steroid-sparing immunosuppressants
- may be used concurrently with steroids for flares refractory to steroids
- mycophenolate mofetil
- Skin and soft tissue bacterial infection of open lesions
- Risk of PV in newborn
- due to placental transfer of maternal autoantibodies
AVERAGE 4.0 of 2 RATINGS
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Sharma VK, Khandpur S. Evaluation of cyclophosphamide pulse therapy as an adjuvant to oral corticosteroid in the management of pemphigus vulgaris. Clin Exp Dermatol. 2013 Aug;38(6):659-64. doi: 10.1111/ced.12073. PubMed PMID: 23837939
PMID:23837939 (Link to Abstract)
Bickle K, Roark TR, Hsu S. Autoimmune bullous dermatoses: a review. Am Fam Physician. 2002 May 1;65(9):1861-70. Review. PubMed PMID: 12018809.
PMID:12018809 (Link to Abstract)
Level of Evidence 5 and Other Journal Articles (includes Case Reports, Expert Opinions,
Personal Observations, and Biomechanic Studies)
Bickle K, Roark TR, Hsu S. Autoimmune bullous dermatoses: a review. Am Fam Physician. 2002 May 1;65(9):1861-70. Review.
PMID:12018809 (Link to Abstract)
Chi AC, Neville BW, Krayer JW, Gonsalves WC. Oral manifestations of systemic disease. Am Fam Physician. 2010 Dec 1;82(11):1381-8. Review.
PMID:21121523 (Link to Abstract)
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