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Dermatology
Dermatitis Herpetiformis and Linear IgA Bullous Dermatosis
Built from Dermatology, 5th Edition
What’s inside
15 sections · 100 slides
Overview
- Scope of this topic
- How this topic is organised
- Four findings that support a diagnosis of DH
Dermatitis Herpetiformis: Introduction and History
A skin disease that is really the visible sign of gluten sensitivity
- What is dermatitis herpetiformis
- Course of dermatitis herpetiformis
- History of dermatitis herpetiformis
- Dermatitis herpetiformis: historic landmarks
- Dermatitis herpetiformis: historic landmarks (continued)
Dermatitis Herpetiformis: Epidemiology
Who gets DH, and how often
- Who gets dermatitis herpetiformis
- How common is dermatitis herpetiformis
Dermatitis Herpetiformis: Pathogenesis
How eating gluten ends up causing a blistering rash on the skin
- Key observations behind the DH mechanism
- Genetic predisposition in DH
- Gluten and the gut in DH
- From gluten to an immune attack: the first steps
- Proposed pathogenesis of dermatitis herpetiformis and celiac disease
- How a helper T cell response drives gut and antibody changes
- How gluten exposure leads to a skin blister
- Epitope spreading explains DH's timing
- Circulating antibodies in DH
- Evidence from animal models
- Granular IgA deposition: the hallmark of DH
- Iodide and DH flares
Dermatitis Herpetiformis: Associated Disorders
Other autoimmune diseases, and a cancer risk, linked to DH
- Thyroid disease and lymphoma risk in DH
- Other autoimmune disorders linked to DH
- Autoimmune disorders associated with dermatitis herpetiformis
Dermatitis Herpetiformis: Clinical Features
What DH looks like on the skin, and where it appears
- Distribution and lesion types in DH
- Less common presentations of DH
- Dermatitis herpetiformis on the elbow and knee
- Dermatitis herpetiformis on the back, buttocks, and fingertips
- Multiple pruritic papules with hemorrhagic crusts in DH
- Hemorrhagic macules on the thumb in DH
Dermatitis Herpetiformis: Pathology and IgA Deposits
What a biopsy shows, and why the biopsy site matters
- Routine histology in DH
- Dermatitis herpetiformis: histopathologic features
- Where to biopsy for the IgA stain in DH
- Direct immunofluorescence in dermatitis herpetiformis
- How often each IgA deposit pattern is seen in DH
Dermatitis Herpetiformis: Diagnosis
Blood tests, the diagnostic work-up, and diseases that mimic DH
- Laboratory evaluation in DH
- Approach to the patient with suspected dermatitis herpetiformis
- Differential diagnosis of DH
- DH, LABD, and bullous pemphigoid at a glance
- Characteristics that differentiate DH, LABD, and bullous pemphigoid
Dermatitis Herpetiformis: Treatment
Dapsone, its side effects, and the gluten-free diet
- Dapsone for DH: how it works and how fast
- Dapsone dosing in DH
- Facial disease and dapsone side effects overview
- Dapsone side effects: hemolysis
- Side effects of dapsone
- Side effects of dapsone
- Serious dapsone reactions to watch for
- Dapsone hypersensitivity syndrome and neuropathy
- Alternatives to dapsone in DH
- Monitoring patients on dapsone or sulfapyridine
- The gluten-free diet in DH
- Patient support for DH
Linear IgA Bullous Dermatosis: Introduction and History
A separate IgA disease, named for a straight line of antibody on the skin
- What is linear IgA bullous dermatosis
- The childhood form: chronic bullous disease of childhood
- Two structural types of LABD
- Two structural subtypes of LABD
- History of linear IgA bullous dermatosis
- Linking the childhood and adult forms at the molecular level
Linear IgA Bullous Dermatosis: Epidemiology and Pathogenesis
How common LABD is, and what its antibodies actually target
- Epidemiology of LABD
- BP180: the shared target of LABD and bullous pemphigoid
- BP180 fragments recognised by LABD antibodies
- Cleavage fragments of BPAG2/BP180 recognised in bullous pemphigoid and LABD
- The sublamina densa type of LABD
- Gut and other disease associations of LABD
- Malignancy and infection triggers of LABD
- Drug-induced LABD
- Drugs reported to cause LABD
- Drug-induced linear IgA bullous dermatosis
- Drug-induced linear IgA bullous dermatosis from vancomycin
Linear IgA Bullous Dermatosis: Clinical Features
A variable rash that can mimic either DH or bullous pemphigoid
- General clinical picture of LABD
- Chronic bullous disease of childhood
- LABD lesions resembling bullous pemphigoid
- Herpetiform, annular LABD lesions on an inflamed base
- Striking annular vesiculobullous lesions of LABD on the thigh
- Circumferential and linear vesicles and bullae in LABD
- Annular and herpetiform vesiculobullae on the face in childhood LABD
- Annular vesiculobullous lesions on the hands in LABD
- Annular and polycyclic plaques of the trunk in LABD
- Mucosal and drug-induced presentations of LABD
Linear IgA Bullous Dermatosis: Pathology and Diagnosis
Biopsy findings, and how LABD is separated from DH and bullous pemphigoid
- Pathology of LABD
- Linear IgA bullous dermatosis: histopathologic features
- Subtyping LABD by electron microscopy
- Direct immunofluorescence in linear IgA bullous dermatosis
- Separating LABD from DH and bullous pemphigoid
- Circulating antibodies and salt-split skin testing in LABD
Linear IgA Bullous Dermatosis: Treatment
Dapsone as first-line therapy, steroid-sparing options, and natural course
- First-line treatment of LABD
- Dosing in children and other treatment options
- Natural course of LABD
Summary
Bringing DH and LABD back together
- DH versus LABD: the core contrast
- Key takeaways
- References
- References (continued)
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- References (continued)
- Dermatology, 5th Edition (2-Volume Set)