VESICULO BULLOUS DISEASE - 3 DR. S. KARTHIGA KANNAN. MDS Erythema multiforme Pemphigus By DR. S. KARTHIGA KANNAN. MDS PROFESSOR Oral Medicine & Radiology

LEARNING OBJECTIVES ☛ To know about those allergic and immunological vesiculo bullous lesions in orofacial area ☛ To know about their clinical features ☛ To know about their management.

Erythema Multiforme Introduction It is an acute inflammatory- vesiculo bullous disease of skin and mucous membranes. As it is characterized by variety of skin lesions showing various shades of redness, it is named as Erythema multiforme. Etiology and Pathogenesis Immune mediated or hypersensitivity reaction to various triggering or precipitating factors. Antigen antibody complexes that are targeted for microvasculature of dermis or submucosa. Cell mediated immune damage.

Erythema multiforme Precipitating factors I. Infections (a) Mycoplasma Pneumonia. (b) Herpes simplex virus (HSV I & II) (c) Histoplasmosis (d) Infectious mononucleosis II. Drugs (a) Sulphonamides and combinations (b) Trimethoprim (d) Nitrofurantoin (e) Penicillin III. Miscellaneous (a) Crohn’s disease (b) Sarcoidosis (c) Stress or emotional factors (d) Radiotherapy

Clinical Features • Age Predilection: Children and Young adults. • Sex predilection: Males > Females • Onset: Sudden explosive onset • Lesion occur as macules and papules of 0.5 to 2cm in diameter, ➝vesicle or bulla ➝ extensive ulceration. The pathognomonic skin lesion is the target or iris lesion, which consists of a central bulla or pale clearing area surrounded by edema and bands of erythema. Site predilection: Cutaneous areas involved are hands, feet, face and neck but only severe cases involves the trunk. • Stevens – Johnson Syndrome Severe form of Erythema multiforme involving skin, mouth, eyes and genitalia.

Oral Manifestation • EM occurs in oral mucosa or accompanied with skin lesions. • Site - lips, buccal mucosa, palate and tongue are common sites, gingiva is usually unaffected. • Erossion and bloody crustations of lips are characteristic. Thin walled vesicles and bulla ruptures into acute multiple, large irregular shallow ulcers. Which often bleeds on palpations. Eating and swallowing becomes difficult. Usually lesion heals within 2-6 weeks

Differential diagnosis Management Primary herpetic gingivo stomatisis Pemphigus vulgaris. Topical - Anesthetics - Corticosteroids - Antiseptic mouth washes. • Soft or liquid diet • Intravenous rehydration if necessary • Anti pyretic & Analgesics if necessary Systemic Corticosteroids Prednisone 30-50mg/day for one week and dose should be tapered and stopped.  

E R Y T H M A U L I F O Before Treatment After Treatment

Pemphigus It is an auto immune disease affecting skin and mucosa. It is a vesiculo bullous disease of importance to dentist as the oral lesion often precedes the skin lesions. Etiopathogenesis The exact etiology is unknown, But there is production of abnormal IgG auto-antibodies against the epidermal cell surface glycoprotein which is a component of desmosomes / intercellular cementing substance. Binding of IgG antibody to pemphigus antigen leads to epithelial cell separation by triggering either complement activity or the plasminogen plasmin system.

Clinical Features • Racial predilection: Ashkenazi Jews • Age incidence: 50-60 yrs of life. • No sex predilection.   Clinical types (a) Pemphigus vulgaris (b) Pemphigus vegetans (c) Pemphigus erythematosus (d) Pemphigus foliaceus • Only the first two types affect oral mucosa. pemphigus vegetans is a variant of P. vulgaris.“Vulgaris is Latin word for Common” . P. Vulgaris the most common type which has been described here. The thin walled bulla arising on normal skin or mucosa rapidly breaks into ulcers. Extend peripherally resulting in large area of denuded skin and superficial, ragged erosions and ulcerations in oral mucosa. A characteristic feature of pemphigus vulgaris is that a bulla can be induced on normal appearing skin by firm lateral pressure. This is called as Nikolsky sign.

Oral manifestations Nearly 50% of patients have oral lesions prior to skin lesion, some time as much as a year or more. This may be because of the lesser amount of intercellular cement substances and lesser number of desmosomes in oral mucosa than the skin. Intra oral sites commonly involved are buccal mucosa, palate, gingival, lip and ventral surface of tongue. In gingival, there may be generalized erythema and desquamation and severe burning sensation often referred as Desquamative gingivitis In the lips there may be chronic erosion, ulcerations covered with bloody crustations.

Investigations Tzanck smear or test The cytological study of the smear from a freshly opened bulla of pemphigus, demonstrate the round free floating epithelial cells which have undergone acantholysis often referred as Tzanck cells.   Biopsy Incisional biopsy from the perilesional or advancing edge of the lesion will demonstrate supra basilar split or bulla (Intraepithelial bulla). Immunofluorescent antibody test Both direct and indirect immunofluorescent technique will be positive in pemphigus.

Management If the treatment is started when the lesion is confined to oral mucosa, the prognosis is good. Systemic corticosteroids – Predinisolone 40-60 mg per day and then gradually tapered. • Steroid sparing agents like Cyclosporine or azathioprine administered to reduce the dose of steroids. • Combination of topical as well as systemic steroids for pemphigus involving only the oral mucosa. Other modalities ☞Parenteral gold therapy. ☞ Etretinate ☞ Dapsone ☞ Plasmapheresis.

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