Pemphigus

Pemphigus vulgaris (PV) is an autoimmune disease causing blisters and erosions of the skin and mucous membranes associated with intraepithelial acantholysis.

1. Pemphigus Dr Shrikant Phatak Consultant & Head of ENT Deptt Choithram Hospital Indore

2. Pemphigus A wide number of dermatological conditions can affect the oral cavity. Pemphigus is one of them. Pemphigus vulgaris (PV) is an autoimmune disease causing blisters and erosions of the skin and mucous membranes associated with intraepithelial acantholysis. When only mucosal involvement is present it may be difficult to diagnose.

3. Pemphigus The oral cavity may be the only site of involvement for a year or so, and this may lead to delayed diagnosis and inappropriate treatment of a potentially fatal disorder Such patients may first present to ENT Surgeon with the symptoms of burning sensation, painful oral ulceration dysphagia, and hoarseness of voice . Hence ENT surgeon must be aware of the clinical manifestations of pemphigus vulgaris.

4. Pemphigus . Oral blisters have a thin roof, which readily rupture in the face of trauma, leading to multiple chronic painful ulcers, bleeding ulcers, and erosions that heal with difficulty . A ntibodies against specific proteins in the  skin and mucous membranes called desmogleins are responsible for Acantholysis.  

5. Pemphigus The lesions most commonly start in areas of frictional trauma such as the buccal mucosa along the occlusal plane , followed by palate, gingiva, pharynx, larynx, oesophagus,

6. Pemphigus P ainful ulcers with irregular, ragged margins seen on the lower lip and labial mucosa, and bilateral buccal mucosa extending posteriorly up to the retro molar trigon Lower lip showing ruptured blister with erosion , the dorsum of the tongue has a thick, diffuse, white coating , while the buccal mucosae showed erosions with pseudomembrane formation [

7. Pemphigus The classic lesion of pemphigus is a thin-walled bulla arising on otherwise normal skin or mucosa , which rapidly breaks and continues to extend peripherally, eventually leaving large denuded areas.

8. Pemphigus Nikolsky's sign is pathognomic for pemphigus and is elicited by applying lateral or tangential pressure to the mucosa or skin in the peri-lesional area resulting in shearing away of the epidermis in the normal areas.

9. Pemphigus Gingiva is the least commonly affected site Desquamative gingivitis is the manifestation of the disease when gingiva is affected.

10. Pemphigus Histopathological features include intraepithelial acantholysis and presence of Tzanck cells.

11. Pemphigus Corticosteroid on a long term basis in high doses play the main role in treatment. Azathioprim ,Methotrexate Rituximab also play a role depending on individual case.

12. Pemphigus When laryngeal mucosa is involved on healing may cause Stenotic narrowing of the airway obscuring the landmarks causing Vice change & may require Tracheostomy for stenosis. Interarytenoid Epiglottis

13. Disclaimer The information contained in the presentation is based on the personal experience and cases collected at Choithram Hospital Indore over the last 20 years. It is intended for the use of Medical students. ENT postgraduates Dermatology students. The views expressed are purely on personal opinion. viewers can make their own opinion. For any confusion please contact sole author. Everybody is allowed to copy or download the material best suited to him. I am not responsible for any controversies arising out of the presentation. For any suggestions or corrections you may please contact [email protected]

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