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Dermatology
Pemphigus
Built from Dermatology, 5th Edition
What’s inside
11 sections · 118 slides
Overview
- What is pemphigus
- How this topic is organised
- Key features of pemphigus
Classification, History, and Epidemiology
The forms of pemphigus, how the disease was discovered, and who it affects
- The three major forms of pemphigus
- Classification of pemphigus
- Classification of pemphigus
- How pemphigus was discovered
- Indirect immunofluorescence in pemphigus
- Who gets pemphigus
- Regional patterns in pemphigus subtype
Fogo Selvagem
An endemic form of pemphigus foliaceus in rural Brazil, and a natural experiment in how autoimmunity can be triggered
- What is fogo selvagem
- The insect-bite theory of fogo selvagem
- A natural experiment in autoimmunity
- From harmless antibody to disease-causing antibody
Pathogenesis: Autoantibodies and Desmogleins
How IgG antibodies against desmogleins actually pull skin cells apart
- Pemphigus IgG causes blisters by itself
- Desmogleins: the glue that pemphigus attacks
- Cadherins: calcium-dependent cell glue
- Two kinds of cell-cell junction in the skin
- The desmoglein family and its partners
- Proof that anti-desmoglein antibodies cause disease
- The desmoglein compensation theory
- Which antibody profile causes which disease
- Why anti-Dsg1 alone spares the mucosa
- Why anti-Dsg3 alone favours the mouth
- Desmoglein compensation theory illustrated
- Two more clues that support compensation theory
- How the antibody actually breaks the glue
Autoimmunity in Paraneoplastic Pemphigus
A double attack - antibodies plus T cells - and the reference table of every known pemphigus target antigen
- Two autoimmune attacks in paraneoplastic pemphigus
- What T cells do in paraneoplastic pemphigus
- Why patients start making these antibodies
- Target antigens across the pemphigus family
- Target antigens in pemphigus
- Target antigens in pemphigus (continued)
- Target antigens in pemphigus (continued)
Clinical Features: Pemphigus Vulgaris
Painful mouth erosions, fragile skin blisters, and the bedside signs that reveal fragile skin
- Two subgroups of pemphigus vulgaris
- Oral involvement in pemphigus vulgaris
- Skin lesions of pemphigus vulgaris
- What pemphigus vulgaris looks like on the skin
- Unusual clinical presentations of pemphigus vulgaris
- Unusual clinical presentations of pemphigus vulgaris
- Bedside signs of fragile skin
- Pemphigus vegetans
- Pemphigus vegetans
- Pemphigus vulgaris: flaccid blister and erosion
Pemphigus Foliaceus and Its Variants
A superficial, skin-only pattern of pemphigus, and the localized, endemic, herpetiform, and drug-induced variants built on it
- What pemphigus foliaceus looks like
- Pemphigus foliaceus: superficial erosions
- Pemphigus foliaceus: erosions and thick scale-crusts
- Pemphigus foliaceus of the scalp
- Generalized pemphigus foliaceus
- Pemphigus erythematosus (Senear-Usher syndrome)
- Pemphigus erythematosus
- Herpetiform pemphigus
- Drug-induced pemphigus
Paraneoplastic Pemphigus and IgA Pemphigus
A tumour-associated, multi-organ disease, and a milder, pustular, IgA-driven form of pemphigus
- The tumours behind paraneoplastic pemphigus
- The hallmark of paraneoplastic pemphigus
- Skin findings and complications of paraneoplastic pemphigus
- Paraneoplastic pemphigus: fragile blisters
- What is IgA pemphigus
- What IgA pemphigus looks like
- IgA pemphigus: sunflower-like pustule pattern
- IgA pemphigus: targets and associations
Pathology
What a biopsy shows in each form of pemphigus, and how to take one correctly
- How to biopsy a suspected pemphigus lesion
- Preferred biopsy sites in autoimmune bullous disease
- Pemphigus vulgaris: histology
- Suprabasilar acantholysis in pemphigus vulgaris
- Causes of eosinophilic spongiosis
- Causes of eosinophilic spongiosis
- Pemphigus foliaceus: histology
- Pemphigus foliaceus: histopathologic features
- Pemphigus foliaceus: cleavage within the granular layer
- Pemphigus foliaceus: clefting in the upper spinous layer
- Paraneoplastic pemphigus: histology
- Paraneoplastic pemphigus: histopathologic features
- IgA pemphigus: histology
- IgA pemphigus: histopathologic features
Diagnosis and Differential Diagnosis
How pemphigus antibodies are actually detected, and the look-alike diseases each subtype must be told apart from
- The gold standard: finding the autoantibody itself
- Direct and indirect immunofluorescence techniques
- What DIF and IIF actually show
- Choosing the right IIF substrate
- IIF for pemphigus and bullous pemphigoid - recommended substrates
- Identifying the exact antigen: ELISA and beyond
- How ELISA distinguishes the subtypes
- ELISA target autoantigens across autoimmune bullous diseases
- Differential diagnosis of pemphigus vulgaris
- Differential diagnosis of pemphigus vegetans
- Pemphigus vegetans - differential diagnosis
- Differential diagnosis of pemphigus foliaceus
- Differential diagnosis of paraneoplastic pemphigus
- Disorders that crust the lips like paraneoplastic pemphigus
- Disorders with hemorrhagic crusts of the vermilion lips
- Hemorrhagic crusting of the vermilion lips
- Differential diagnosis of IgA pemphigus
Treatment
From a once-fatal disease to a manageable one - corticosteroids, steroid-sparing agents, and modern B cell-targeted therapy
- Why treatment must target antibody production
- Systemic corticosteroids: the mainstay
- Immunosuppressive steroid-sparing agents
- Tapering and long-term strategy
- Plasmapheresis and high-dose IVIg
- Rituximab: B cell-depleting therapy
- Emerging targeted therapies
- The therapeutic ladder for pemphigus vulgaris
- Therapeutic ladder for pemphigus vulgaris
- Therapeutic ladder for pemphigus vulgaris (continued)
- Treatment of pemphigus foliaceus
- Treatment of paraneoplastic pemphigus
- Treatment of IgA pemphigus
- Summary: telling the pemphigus family apart
- Key take-home points
- References
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- References (continued)
- Dermatology, 5th Edition (2-Volume Set)