FA C U LT Y

DEVELOPMENT
PODCAST FOR
PEMPHIGUS
VULGARIS

Rashi Agrawal
w/ Dr. Loretta Davis
and Dr. Victoria Madray
 • Brief Overview of the Skin
• Differential of Blistering Conditions
LEARNING
• Pemphigus Vulgaris and possible
OBJECTIVES treatments
• Psycho-social aspects
Skin
• Body’s largest organ and covers the
entire external surface
• Made up of three layers with varying
anatomy and functions: epidermis,
dermis, and hypodermis
• The epidermis is further made up of 4,
or 5 in the case of thick skin, layers

• Provides important protection and

functions for the body
• Made up of multiple cell types, such as
keratinocytes, melanocytes,
Langerhans’ cells, and Merkel’s cells
 Click icon to add picture
Skin Cells
• Keratinocytes
• Melanocytes
• Langerhans’ cells
• Merkel’s cells
Blistering Conditions
Blistering Conditions: Closer look into Generalized
FLACCID BULLAE
• Stevens-Johnson Syndrome/Toxic
Epidermal Necrolysis
• Staphylococcal Scalded Skin Syndrome
• Pemphigus vulgaris
• Pemphigus foliaceous
• Paraneoplastic pemphigus
TENSE BULLAE
• Bullous pemphigoid
• IgA bullous dermatosis
• Dermatitis herpetiformis
• Pemphigoid gestationis
• Vesicles:
• Disseminated HSV/VZV
• Sweet Syndrome
Pemphigus Vulgaris
• Pemphigus, broadly, is defined blistering disorders characterized by
acantholysis that results in the formation of intraepithelial blisters in mucous
membranes and skin
• PV diagnosis made with clinical presentation of mucosal erosions (oral and
genital) fragile vesicles and bullae with widespread erosions on the skin
• IgG autoantibodies against Desmoglein 3 or both desmoglein 1 and 3
• Incidence rates between 0.1 and 0.5 per 100,000 people per year have been
reported
• Individuals with Jewish ancestry (particularly Ashkenazi Jews) and inhabitants of
India, Southeast Europe, and the Middle East have the greatest risk for
pemphigus vulgaris
Pemphigus Vulgaris Pathophysiology
• PV is an autoimmune condition due to IgG autoantibodies binding to antigens in the skin,
specifically desmoglein 3 (mucosal lesions) and 1 (mucocutaneous). The binding between the
antibodies and desmoglein is know to result in the formation of the blisters; however, there
are multiple theories behind the exact mechanism of how this interaction leads to
acantholysis.
• Steric Hindrance: the induction of signal transduction events that trigger cell separation and the
inhibition of adhesive molecule function
• Desmoglein non-assembly depletion: autoantibodies bind each other leading to crosslinking and the
inability for desmosomes to maintain cell-cell adhesion
• Apoptolysis: trigger for enzymatic cascades that lead to structural collapse and cellular shrinkage
Pemphigus Vulgaris Biopsy: H&E and DIF
Treatment for Pemphigus Vulgaris
• The mainstay treatment regimen is systemic glucocorticoids (typically prednisone) in
conjunction with an immunosuppressant drug. Dual therapy is often needed to first, attain
disease control then secondly, control the disease and prevent flares.
• Common adjuvant immunosuppressive drugs: Mycophenolate mofetil or azathioprine
• Rituximab is increasingly being more commonly used due to multiple trials demonstrating its
efficacy. Rituximab is a monoclonal antibody targeting CD20, which is found on B cells.
• Symptom management: blister and wound care; preventing secondary infections; topical
corticosteroids for persistent lesions
Psycho-Social Aspects
• While there is no scarring generally associated with the lesions, there can still be a risk of
post-inflammatory hyperpigmentation or scarring as a result of secondary infections.
• Many people with a skin condition:
• Experience decreased sense of body image
• Have lower self-esteem
• Avoid situations where the skin is exposed
• Feel anxious about people judging them
• Withdraw from social interactions
• Have sexual and relationship issues
• Feel shame and disgust about their appearance