Auto immune disorders Pemphigus Auto immune disease involving

Auto immune disorders

Pemphigus • Auto immune disease involving skin &mucosa & charecterised by intra epidermal bullae formation, caused by antibodies to intercellular cementing substance. • 4 types • 1. P. vulgaris – most common • 2. P. vegetans • 3. P. foliaceus • 4. P. erythematous

Clinical feauters • Thin walled bullae breaks especially but extend peripherally leaving large areas of denuded skin. • Characteristic sign-Asboe Hansen phenomena/ bulla expansion phenomena Application of pressure to an intact bulla causes enlargement of bulla by extention into a normal surface. Nikolskys sign is positive –pressure to an normal area will result in formation of a new lesion, results from the upper layer of the skin pulling away from the basal layer.

Oral manifestation • Shallow ulcers on the buccal mucosa along the occlusal line. • Palate and gingiva is also involved.

Diagnosis • History • Viral infections & EM-acute in nature. • Aphthous ulcers-lesion heal and recur, pemphigus lesions persists for months together. • Lab investigations(1) biopsy –look for suprabasilar acantholysis (2) tzank test –positive (3) direct &indirect immunoflorescence antibody test.

Treatment • Topical corticosteroids –triamcinolone acetate • Systemic corticosteroids + immunoisuppressive drugs like cyclosporine