A case of chronic mouth ulcers is presented. Pemphigus is an autoimmune blistering disease that affects the skin and mucous membranes. It is caused by IgG autoantibodies that disrupt intracellular attachments between keratinocytes. Pemphigus vulgaris is the most common type and presents with oral ulcers that precede skin involvement. Diagnosis is made through skin biopsy and direct immunofluorescence. Treatment involves systemic corticosteroids and immunosuppressants to reduce pathogenic antibody levels. Prognosis has improved with corticosteroid use but mortality can still occur without treatment.

1. Case Presentation:
A case of chronic mouth ulcers…
Mazin Eragat
GPST1

2. Case
Presenting complaint
History of presenting complaint

3. Case
Past medical history
Drug history
Allergies

4. Case
Social history
Family history
Travel history

5. Case
Examination
Investigations

6. Case
Differential diagnoses
Management plan

8. Pemphigus
The name is derived from the
Greek root "pemphix",
meaning "pustule”
• Rare, autoimmune mediated inflammatory blistering disorders
• M = F, equally affected genders
• Affects 4th – 6th decades of life
• Life threatening without treatment
• Variants include Pemphigus -
• Vulgaris (80%)
• Vegetans
• Foliaceus
• Erythematosus
• Paraneoplastic pemphigus

9. Diagnosis
Histological morphology; Acantholysis – the lysis of
intercellular adhesions that connect squamous epithelial
cells
• Skin biopsy specimen(s)
• Suprabasilar acantholysis  Vulgaris
• The single layer of intact basal cells that form
the blister base likened to ‘tombstones’
• Subcorneal acantholysis  Foliaceus
• Suprabasilar acantholysis & dyskeratotic
keratinocytes with basal cell vacuolization

10. Pathophysiology
• IgG autoantibodies result in dissolution of
intracellular attachments (Desmoglesins)
• Attacks epidermis  mucosal epithelium
• Bullous diseases can be dramatic
• Bullae can occur in different levels within skin,
causing mild to dramatic blisters
• Variable superficial dermal infiltration by
lymphocytes, histiocytes, and eosinophils
accompany all forms of pemphigus
• Direct immunofluorescence – shows net-like
pattern of intercellular IgG deposits

11. History (Vulgaris)
• Pemphigus is the commonest type
• Can involve scalp, face, axilla, groin, trunk, points of pressure
• May present as oral ulcers, classically persisting for months before
skin involvement appears
• Primary lesions are superficial vesicles / bullae that rupture easily
• This leaves shallow erosions which are crusted

12. Treatment
• Regimens depend on patient’s age, degree of involvement, rate of progression,
and subtype of pemphigus.
• Systemic corticosteroids (eg. 1mg/kg Prednisolone oral OD) required for P.
Vulgaris, whereas topical corticosteroids (0.05% fluocinonide ointment topical
BD) can occasionally control P. foliaceus.
• One course of IV immunoglobulin (400 mg/kg 5/7 days) is safe for steroid
resistant disease.
• Other agents include; Dapsone, Hydroxychloroquine, Mycophenolate mofetil,
Azathioprine, Cyclophosphamide & Rituximab.
• Although steroid sparing agents are clinically effective, few controlled studies
demonstrate benefit.
• Tumour removal in paraneoplastic pemphigus is often curative, but in
malignancies can be resistant and require pulse methylpred, immuno-apheresis,
immunoablative cyclophosphamide & rituximab.

13. Prognosis
• Mainstay of treatment is immunosuppressive agents, which reduce
titre of pathogenic antibodies.
• Before use of corticosteroids, 60-90% of patients with vulgaris died
• Since introduction of steroids, dropped to 5-10% mortality rate
• Paraneoplastic relates to the type of cancer
• Benign tumours – usually lesions clear after tumour resection
• Malignant tumours (most commonly non-Hodgkin lymphoma) – have a
poorer prognosis and treatment does not entirely prevent mortality

14. Questions?
• Many thanks