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Dermatology
Pemphigoid Group
Built from Dermatology, 5th Edition
What’s inside
5 sections · 151 slides
Overview of the pemphigoid group
Three autoimmune blistering diseases that share one junction and diverge in where they strike
- What this chapter covers
- The shared target: the dermal-epidermal junction
- Mapping the target proteins
- Major autoantigens of subepidermal autoimmune-mediated blistering diseases.
- Major autoantigens of subepidermal autoimmune-mediated blistering diseases. (continued)
Bullous Pemphigoid
The most common autoimmune blistering disease of the skin
- Key features of bullous pemphigoid
- History of bullous pemphigoid
- Epidemiology of bullous pemphigoid
- The two target antigens: BP180 and BP230
- The NC16A domain: where most antibodies bind
- T cells drive the antibody response
- From antibody binding to blister: the mechanism
- Potential mechanisms of blister formation
- IgG can damage the junction without complement
- The role of IgE autoantibodies
- Evidence from animal models
- Clinical spectrum of bullous pemphigoid
- The non-bullous (pre-blister) phase
- The bullous phase
- Classic bullous pemphigoid presentation
- Residual changes and mucosal involvement
- Bullous pemphigoid -- urticarial presentation
- Bullous pemphigoid -- eczematous presentation
- Bullous pemphigoid -- itch-only presentation
- Unusual clinical variants of BP
- Bullous pemphigoid -- uncommon clinical variants
- Unusual clinical variants of bullous pemphigoid.
- Bullous pemphigoid in children
- Childhood bullous pemphigoid
- Associated malignancy and autoimmune disease
- Triggers and disease associations
- Bullous pemphigoid localized to a psoriatic plaque
- Neurologic and psychiatric associations
- Drug-induced bullous pemphigoid
- Drug-induced bullous pemphigoid
- Bullous pemphigoid -- further bullous examples
- Bullous pemphigoid -- fresh and older blisters together
- Bullous pemphigoid -- urticarial and bullous overlap
- Bullous pemphigoid -- a second eczematous example
- Bullous pemphigoid -- progression over time
- Bullous pemphigoid -- excoriated and confluent disease
- Oral involvement (desquamative gingivitis) in BP
- Childhood BP -- acral predominance
- Diagnosing bullous pemphigoid
- Light microscopy findings
- Urticarial-phase histopathology
- Bullous-phase histopathology
- Direct immunofluorescence (DIF) microscopy
- Two ways to refine a positive DIF result
- BP -- direct and indirect immunofluorescence
- Indirect immunofluorescence (IIF) microscopy
- Indirect immunofluorescence on salt-split skin
- ELISA blood tests for BP180 and BP230
- BP180 and BP230 reactivity on lab testing
- Bullous pemphigoid -- other lab studies
- BP -- immunoelectron microscopy of BP230
- Differential diagnosis: nonspecific forms
- Four clinical clues that favour BP
- Distinguishing BP from its closest mimics
- Bullous pemphigoid vs epidermolysis bullosa acquisita
- Prognosis of bullous pemphigoid
- Monitoring disease activity
- Treatment principles
- Oral corticosteroids for extensive disease
- Ultrapotent topical corticosteroids
- The treatment ladder, at a glance
- Therapeutic ladder for bullous pemphigoid.
- Steroid-sparing immunosuppressive drugs
- Options for localized or mild disease
- Newer biologic therapies
- Evidence from controlled trials
- Survey of selected controlled trials for the treatment of patients with bullous pemphigoid.
- Survey of selected controlled trials for the treatment of patients with bullous pemphigoid. (continued)
- Survey of selected controlled trials for the treatment of patients with bullous pemphigoid. (continued)
- Survey of selected controlled trials for the treatment of patients with bullous pemphigoid. (continued)
- Duration of treatment and safety measures
Mucous Membrane Pemphigoid
A scarring disease of the mouth, eyes, and other mucosal surfaces
- Key features of mucous membrane pemphigoid
- History of mucous membrane pemphigoid
- Epidemiology of mucous membrane pemphigoid
- How MMP damages tissue
- The four autoantibody subgroups of MMP
- Subgroup 1: anti-laminin 332 disease
- Subgroups 2-4: ocular, anti-BP, and heterogeneous
- Which mucosae does MMP affect
- Oral involvement in MMP
- Mucous membrane pemphigoid -- oral lesions
- Conjunctival (eye) involvement in MMP
- Ocular involvement -- progression over time
- How scarring damages the eye
- Symblepharon in mucous membrane pemphigoid
- Nose, throat, and airway involvement
- Genital and anal involvement
- Cutaneous (skin) lesions in MMP
- The Brunsting-Perry variant
- MMP Brunsting-Perry variant
- MMP -- oral disease as the presenting sign
- MMP -- gingival erythema and erosion
- MMP -- ectropion and exudative conjunctivitis
- MMP -- a second symblepharon example
- MMP -- scarring alopecia of the scalp
- MMP -- chronic scarring ulceration of the chest
- Diagnosing MMP: why delay is common
- Light and electron microscopy in MMP
- Direct immunofluorescence (DIF) in MMP
- Immunoelectron microscopy in MMP
- Indirect immunofluorescence (IIF) in MMP
- ELISA and immunochemical testing in MMP
- Differential diagnosis of MMP
- Prognosis of mucous membrane pemphigoid
- Local (topical) therapy for MMP
- Systemic therapy for MMP
- Additional and emerging MMP therapies
- Surgical therapy for MMP
Epidermolysis Bullosa Acquisita
The rarest of the three, targeting a deeper anchor -- type VII collagen
- Key features of epidermolysis bullosa acquisita
- History of EBA
- Epidemiology of EBA
- Type VII collagen: the target structure
- How EBA autoantibodies cause damage
- Two broad clinical patterns of EBA
- The mechanobullous (classic) form
- EBA -- mechanobullous presentation
- EBA in a patient with systemic lupus erythematosus
- The inflammatory, BP-like form
- EBA -- inflammatory presentation
- Rarer inflammatory subtypes and mucosal disease
- EBA -- oral involvement
- EBA -- inflammatory presentation in multiple myeloma
- Systemic disease associations of EBA
- Light and electron microscopy in EBA
- Direct immunofluorescence (DIF) in EBA
- Indirect immunofluorescence (IIF) in EBA
- Immunoelectron microscopy: the gold standard
- ELISA and other immunochemical tests in EBA
- Differential diagnosis of EBA
- EBA vs bullous systemic lupus erythematosus
- Treating EBA
Putting it all together
Three diseases, one shared junction, told apart by where they strike and how the antibodies bind
- The pemphigoid group at a glance
- How lab testing separates the three diseases
- Key take-away points
- References
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- References (continued)
- Dermatology, 5th Edition (2-Volume Set)