Immunobullous Diseases

1. 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

2. 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

3. Progression of ocular cicatricial pemphigoid
Diffuse hyperaemia
Subepithelial fibrosis and shrinkage Symblepharon
Pseudomembrane formation

4. Complications of ocular cicatricial pemphigoid
Ankyloblepharon
Corneal keratinization
Metaplastic lashes Cicatricial entropion
Total obliteration of fornices Secondary bacterial keratitis

5. 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 haemorrhagic
and necrotic lesions

6. Ocular complications of Stevens-Johnson syndrome
Transient conjunctivitis and
lid crusting without sequelae
Severe membranous or
pseudomembranous conjunctivitis
Focal fibrotic patches and
occasionally symblepharon
Metaplastic lashes

7. 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

8. 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)