Patient Case: Pemphigus Vulgaris
A 48-year-old woman presents to the dermatology clinic with a two-month history of painful blisters and erosions on her skin and in her mouth. She first noticed painful sores in her mouth, making it difficult to eat. A few weeks later, she developed flaccid blisters on her chest, back, and arms, which rupture easily, leaving raw, tender skin. She has no fever or recent infections and denies taking any new medications.
On examination, there are multiple superficial blisters and erosions on her trunk and extremities, with some areas showing crusting and oozing. There are also painful erosions on her oral mucosa. A Nikolsky sign is positive (skin shears off with slight pressure).
A clinical diagnosis of pemphigus vulgaris is made. A skin biopsy with direct immunofluorescence confirms intercellular IgG deposition against desmoglein. The patient is started on high-dose systemic corticosteroids and rituximab for immunosuppression.
- What is the most likely diagnosis in this patient?
a) Bullous pemphigoid
b) Pemphigus vulgaris
c) Stevens-Johnson Syndrome
d) Contact dermatitis - Which clinical feature is most characteristic of pemphigus vulgaris?
a) Tense bullae on flexural surfaces
b) Deep ulcerations with eschar formation
c) Flaccid blisters that rupture easily with mucosal involvement
d) Pruritic wheals that resolve spontaneously - Which diagnostic test is most useful in confirming pemphigus vulgaris?
a) Direct immunofluorescence of a skin biopsy
b) Bacterial culture of blister fluid
c) Skin scraping with potassium hydroxide (KOH) preparation
d) Complete blood count (CBC) - What is the first-line treatment for pemphigus vulgaris?
a) Topical corticosteroids and antihistamines
b) High-dose systemic corticosteroids and immunosuppressants
c) Oral antibiotics and wound care
d) Antiviral therapy with acyclovir
