11 immunobullous diseases
TRANSCRIPT
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1. Cicatricial pemphigoid
2. Stevens-Johnson syndrome
3. Toxic epidermal necrolysis (Lyell disease)
4. Epidermolysis bullosa
5. Pemphigus vulgaris
6. Linear IgA bullous dermatosis
IMMUNOBULLOUS DISEASES
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Cicatricial pemphigoid• Chronic and progressive• Typically affects elderly women• Increased prevalence of HLA-B12
Oral mucosal lesions in most cases Skin lesions are less common
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Progression of ocular cicatricial pemphigoid
Diffuse hyperaemia
Subepithelial fibrosis and shrinkage Symblepharon
Pseudomembrane formation
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Complications of ocular cicatricial pemphigoid
Ankyloblepharon
Corneal keratinization
Metaplastic lashes Cicatricial entropion
Total obliteration of fornices Secondary bacterial keratitis
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Stevens-Johnson syndrome• Acute, and self-limiting• Hypersensitivity to drugs or infection• Typically affects young men
Lesions of oral mucosa and lips
Maculopapules which may develop into target lesions
Vesciculobullous haemorrhagicand necrotic lesions
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Ocular complications of Stevens-Johnson syndrome
Transient conjunctivitis and lid crusting without sequelae
Severe membranous or pseudomembranous conjunctivitis
Focal fibrotic patches andoccasionally symblepharon
Metaplastic lashes
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Other immunobullous diseases (1)Toxic epidermal necrolysis
(Lyell disease)
• ‘Scalded’ skin lesions
• Conjunctivitis - common and similar to Stevens-Johnson syndrome
Epidermolysis bullosa
• Skin bullae and necrosis induced by minor trauma• Conjunctivitis - common and may result in scarring
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Pemphigus vulgaris
• Flaccid, weeping bullae
• Conjunctivitis - uncommon and mild
Linear IgA bullous dermatosis
• Tense bullae of varying size
• Conjunctivitis - common and may result in scarring
Other immunobullous diseases (2)