Vesicobullous Conditions Affecting The Oral Mucosa KATHLEEN BERRY

Definition Vesicle – a small fluid lesion/blister which can rupture to form an ulcer. Bulla – a larger blister. Ulcer – a break in the mucous membrane. Vesicobullous conditions can be classified into intraepithelial or subepithelial vesicles.

Oral mucosa is made up of specialised stratified squamous epithelium with an underlying connective tissue layer (lamina propria) Intraepithelial vesicles : lesion is formed within the epithelium as there is loss of cell-cell adhesion by breakdown of specialised attachments (desmosomes). This is acantholysis. Subepithelial vesicles : lesions are formed between the epithelium and the underlying lamina propria (corium).

Intraepithelial

Pemphigus Chronic mucocutaneous disease Aetiology – antibody mediated autoimmune Binding of IgG antibody to pemphigus antigen leads to epithelial cell separation – acantholysis Several types : Pemphigus vulgaris, vegetans , erythematosus, foliaceous, paraneoplastic pemphigus and drug related pemphigus. Most common is Pemphigus vulgaris.

Pemphigus Vulgaris Most common >65 years Site – mouth and skin Potentially fatal Clinical Features: Bullae readily burst to form large irregular ulcers. Common sites : buccal mucosa, palate, tongue

Diagnosis and treatment Nicolsky’s sign +ve Biopsy Immunofluorescent techniques. (Direct – uses biopsy sample to detect autoantibodies. Indirect – uses patient’s serum to detect circulating antibodies). Treatment steroids – topical and systemic Immunosuppressants eg. Azathioprine, methotrexate (allow lower doses of steroid to be used).

Primary herpetic gingivostomatitis Aetiology – Herpes simplex virus type 1 rarely type 2 Severity usually increases with increasing age. Prodromal period of 2-12 days Self limiting usually heals within 2 weeks

Primary herpetic gingivostomatitis Clinical features: Fever , malaise Enlarged cervical lymph nodes Multiple vesicles which breakdown to form shallow , painful ulcers Gingivitis

Diagnosis and treatment. History and clinical examination Treatment : soft diet, topical/systemic analgesics , fluids. If patient is immunocompromised – antiviral Acyclovir

Herpes Labialis (cold sore) Reactivation of primary infection- latent in trigeminal ganglion. Predisposing factors eg. Trauma, stress, sun light Usual site is lip but can affect intraoral mucosa of palate , gingivae.

Herpes Zoster (Varicella zoster) Primary infection – chicken pox Reactivates as shingles. Confined to distribution of a nerve – oromucosal lesions follow trigeminal nerve branches Following healing may result in post herpetic pain

Shingles Pre eruption pain Painful vesicles rupture to form ulcers Treatment includes fluids, pain relief, antivirals?

Coxsackie Virus Herpangina. Hand Foot and Mouth disease. Coxsackie A or B Mainly children Fever , malaise Multiple, small vesicles posterior oral cavity and pharynx Dysphagia Self limiting 1-2 weeks Coxsackie A16 Usually children Fever , malaise Multiple vesicles on hands, feet and oral mucosa – mainly palate Self limiting 1-2 weeks

Subepithelial vesicobullous disorders.

Benign Mucous Membrane Pemphigoid Aetiology – chronic , autoimmune disorder Autoantibodies directed against basement membrane to form subepithelial vesicles. F > M >60 years Affects any mucosa (can lead to blindness if conjunctiva affected) Periods of inactivity 2 variants are seen affecting oral mucosa.

Mucous membrane pemphigoid Variant 1. Intact vesicles , bullae (maybe blood filled) Ulcerations may lead to scarring.- if this affects larynx results in hoarseness, dysphagia Variant 2. Presents with desquamative gingivitis involving only the gingivae around the teeth. Gingivae are erythematous and hyperaemic

Diagnosis and treatment. History and clinical exam. Biopsy Immunofluoresence Management – analgesics, topical/systemic corticosteroids, immunosuppressants

Erythema Multiforme. Acute , inflammatory disease of skin and mucous membranes. Aetiology : hypersensitivity reaction immune mediated idiopathic predisposing factors- infection (particularly herpes), drugs (antibiotics, anticonvulsants, allopurinol and NSAIDs). Most common in young adult males Severe form is Stevens Johnson syndrome.

Erythema Multiforme Vesicles and bullae which rupture to form ulcers and erosions. Common sites: lips, buccal mucosa , palate , tongue, face. Target lesions of erythema on skin. Crusting of lips. Fever , malaise.

Diagnosis and management. History and clinical exam. Biopsy Self limiting but will need supportive therapy –fluids, soft diet , analgesics Topical/ systemic corticosteroids.

Epidermolysis Bullosa Aetiology-genetic or acquired. Appears shortly after birth Affects skin and mucous membranes Symptoms mild-severe Trauma to mucosa results in bullae formation Healing leads to scarring which may make eating, swallowing , speaking difficult.

Other subepithelial vesicobullous disorders affecting oral mucosa. Linear IgA disease Dermatitis Herpetiformis Bullous Lichen Planus Bullous systemic lupus erythematosus.

Summary Most vesicobullous disorders present initially with oral lesions. With their associated morbidity and mortality early diagnosis is vital and should be referred – (apart from those known to be viral).

References: Oral Medicine (C. Scully and R.A. Cawson). Oral Medicine (S. Gandolfo, C. Scully and M. Carrozzo). An overview of vesicobullous conditions affecting the oral mucosa. (Emma Hayes, Stephen J Challacombe).