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Vesiculo-Bullous Lesions |
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Blistering Conditions in Children/Young Adults ¨ Primary herpetic gingivostomatitis (SEE picture on right) ¨ Recurrent herpes simplex- labialis, oral ulceration ¨ Chickenpox ¨ Herpangina ¨ Hand, foot and mouth disease ¨ Mucocele
Blistering Conditions in Adults/Elderly ¨ Shingles ¨ Erythema Multiforme ¨ Pemphigoid ¨ Pemphigus ¨ Desquamative Gingivitis –assoc. with Lichen Planus, Pemphigoid, Pemphigus ¨ Linear IgA disease ¨ Dermatitis Herpetiformis ¨ Angina Bullosa Haemorrhagica
Erythema Multiforme Acute, often recurrent, hypersensitivity reaction affecting mucocutaneous tissues. Males, uncommon, younger adults(20-40) Aetiology: Cause uncertain, associated with certain predisposing factors: previous infection with herpes simplex or Mycoplasma, administration of a wide range of systemic drugs (incl. sulfonamides, cephalosporins, barbiturates), pregnancy, inflammatory bowel disease. Wide spectrum of severity: minor –one site, mouth alone or skin or other mucosae to major (Stevens-Johnson syndrome) severe, life-threatening, starts with flu-like symptoms, widespread lesions (eyes, skin, genitals, pharynx) invariably involves oral mucosa, bullous and other rashes Degree of mucosal involvement distinguishes between minor and major forms Clinical Features: Oral lesions: in 70% of cases, macules evolve to blisters and erosions; lips that swell, crack, bleed and crust Skin lesions; distal extremities-extensor surfaces, macules can evolve into papules (classic target lesions) – concentric rings of erythema Eye involvement causes lacrimation and photophobia; Genital lesions are painful Diagnosis: mainly clinical; can undertake serology for HSV, Mycoplasma or other micro-organisms; biopsy of peri-lesional tissue. If EM Major – FBC, U+E’s, ESR, LFT’s, cultures from blood, sputum and erosive areas. Management: supportive care important i.e. IV fluids, electrolytes, nutritional support. Early ophthalmologic and dermatological consultation needed Precipitating factors identified and treated 0.2% CHX mouthwash improves OH Anti-microbials may be indicated (HSV, Mycoplasma) Corticosteroids – Minor topical may be sufficient, systemics may still be required Major treated with systemics and/or Azathioprine
Sub-epithelial immunologically-mediated vesiculobullous disorders ‘Mucous Membrane Pemphigoid’ (MMP) –predominantly affects mucous membranes but skin rarely Characterised by damage to basement membrane zone (BMZ) – autoAB’s, gives rise to junctional separation and sub-basilar split 50-60 years, twice as common in females Clinical Features: Wide spectrum of disease – heals with variable amounts of scar formation (rare in mouth) Oral lesions affect mainly the gingivae and palate, and can include:- desquamative gingivitis – erythematous, ulcerated gingival, usually patchy distribution bullae/vesicles – intact blisters rarely seen, sometimes blood-filled. Pressure on blister can cause it to spread (Nikolsky sign) Majority of people with MMP have only oral involvement however untreated Ocular involvement (20%) can lead to blindness mainly due to conjunctival scarring, Genital involvement can be a great source of morbidity Diagnosis: Biopsy of perilesional tissue with histological and immunostaining (reveals presence of BMZ immune deposits) examination essential. (*Oral lesions can be confused clinically with pemphigus, erythema multiforme.) Management: Opthalmological consultation essential; most respond well to topical corticosteroids or tacrolimus – effective if worn in POC to treat desquamative gingivitis; OH important as secondary infection can inhibit healing; Severe pemphigoid treated with dapsone, azathioprine, or systemic corticosteroids
Group of potentially life-threatening chronic autoimmune diseases, characterised by epithelial blistering affecting mucocutaneous surfaces Most common pemphigus variant = Pemphigus vulgaris -usually responsible for oral lesions, and also most severe form Rare, middle-aged/elderly patients, female predisposition Aetiology: Pemphigus vulgaris has strong genetic background - particularly Jewish, South Asian or Mediterranean descent Can be triggered by medications (e.g. captopril, diclofenac, rifampicin) radiation, surgery, emotional stress Serum auto-AB’s against desmosomes in squamous epithelium, loss of cell-to-cell contact (acantholysis), causes intra-epithelial vesiculation
Clinical Features: Causes blisters and scabs on skin, and blisters, erosions and ulcers on mucosae of mouth, pharynx, larynx, oesophagus, nose, conjunctiva, anogenital region Nikolsky sign positive (blister spreads with pressure) Oral lesions: common, early manifestation; seen mainly on soft palate and posterior hard palate, buccal mucosa, gingiva-desquamative; vesiculobullous and rupture easily; form erosions which become yellowish with time; Invariably followed by involvement of skin if no systemic treatment Diagnosis: biopsy mandatory – perilesional tissue with histological and immunostaining examination Serum collected for titres of antibody to epithelial inter-cellular cement, can guide treatment Management: Systemic immunosuppression has reduced mortality to 10% Prednisolone used as first choice, Azathioprine, Cyclosporin, Cyclophosphamide used as adjuncts Hydration important, as is preventing secondary systemic infections
Possible to induce complete and durable remissions whereby systemic therapy can be discontinued without flare up of disease activity (75% of patients after 10 years) |
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Blistering conditions of the oral mucosa can be due to trauma, infection or immune-related diseases. It is rare to see intact vesicles/bullae since they rapidly rupture, leaving an area of ulceration/erosion. These conditions are painful. They can be conveniently divided into those occurring in the young (majority of which are viral infections) or in adults. |
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Pemphigus Vulgaris |