11 immunobullous diseases

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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

Transcript of 11 immunobullous diseases

Page 1: 11 immunobullous diseases

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)