Vesiculobullous disease

By
Dr. Hadi Zahid Rao
Classification
I. Intraepithelial vesiculobullous diseases
Acantholytic lesions
Pemphigus vulgaris
Paraneoplastic pemphigus & other variants
Darier’s disease
Non-acantholytic lesions
Viral infections of oral mucosa
Classification continued
II. Subepithelial vesiculobullous diseases
Erythema multiforme
Pemphigoid
Dermatitis herpetiformis & Linear IgA disease
Epidermolysis bullosa
Angina bullosa haemorrhagica
Bullous lichen planus
Pemphigus vulgaris
 Autoimmune
 Presents in middle age (40-60 yrs)
 More common in females
 Intraepithelial
Clinical presentation
 Bullous eruptions on skin and mucous
membrane
 Ruptured readily forming crusted denuded

areas on skin
 Irregular, jagged, painful ulcers on mucosa
 Desquamative gingivitis
 Nikolsky’s sign may be positive
Histological presentation
 Autoantibodies to desmogleins 1 & 3
 Loss of intracellular adherence of suprabasal

spinous cells ( acantholysis)

 Cleft-like spaces
 Intraepithelial vesicles/ bullae
 Polyhedral stratum spinosum cells appears as

small, rounded and contains hyperchromatic

nuclei (Tzanck cells)
Diagnosis
 Clinical presentation
 Biopsy
 Immunofluorescence
Treatment
 Immunosuppressive
 80-100mg/day predisolone + azathioprine

(1-1.5mg/kg daily)
Pemphigoid
 Autoimmune
 Elderly females usually affected
 Subepithelial
Clinical presentation
 Vesicles/ bullae on mucosa and skin
 Relatively tough, tense remains intact
 If ruptured---slow healing erosions, scarring

(esp. eye)
 Desquamative gingivitis
Histological presentation
 Autoantibodies to C3
 Separation of full thickness of epithelium

from lamina propria

 Subepithelial bulla with thick roof
 Infiltration of neutrophils & eosinophils
Diagnosis
 Clinical presentation
 Biopsy
 Immunofluorescence
Treatment
 Mild-moderate – Dapsone ( first line)
 Severe – Potent immunosuppressive therapy
 Steroids + azathioprine
 Cyclophosphamide/ mycophenylate mofetil