Vesiculobullous Diseases: Pemphigus Vulgaris

Question 1

Pemphigus Vulgaris

Clinical Feature

Answer 1

autoimmune blistering disease characterized by flaccid, non-pruritic intraepidermal bullae/vesicles on
an erythematous or normal skin base
• may present with erosions and secondary bacterial infection

Question 2

Pemphigus Vulgaris

Relevant Signs

Answer 2

Nikolsky’s sign: epidermal detachment with shear stress

• Asboe-Hansen sign: pressure applied to bulla causes it to extend laterally

Question 3

Pemphigus Vulgaris. sites:

Answer 3

mouth (90%), scalp, face, chest, axillae, groin, umbilicus

Question 4

Pemphigus Vulgaris Pathophysiology

Answer 4

• IgG against epidermal desmoglein-1 and -3 lead to loss of intercellular adhesion in the epidermis

Question 5

Pemphigus Vulgaris

Epidemiology,. Ethnics

Answer 5

40-60 yr old, M=F, higher prevalence in Jewish, Mediterranean, Asian populations

Question 6

Pemphigus Vulgaris

Associated pathologies

Answer 6

• paraneoplastic pemphigus may be associated with thymoma, myasthenia gravis, malignancy, and use of
D-penicillamine

Question 7

Pemphigus Vulgaris Investigations

Answer 7

immunofluorescence: shows IgG and C3 deposition intraepidermally
• circulating serum anti-desmoglein IgG antibodies

Question 8

Pemphigus Vulgaris Prognosis. Fatality

Answer 8

lesions heal with hyperpigmentation but do not scar

• may be fatal unless treated with immunosuppressive agents

Question 9

Pemphigus Vulgaris Management

Answer 9

prednisone 1-2 mg/kg until no new blisters, then 1-1.5 mg/kg until clear, then taper ± steroid-sparing
agents (e.g. azathioprine, cyclophosphamide, cyclosporine, IVIg, mycophenolate mofetil, rituximab)