Pemphigus vulgaris Management

Pemphigus vulgaris is a rare, chronic autoimmune blistering disease characterized by the formation of flaccid blisters and painful erosions involving the skin and mucous membranes. The disease results from autoantibodies directed against proteins responsible for maintaining adhesion between epidermal cells, leading to separation of skin cells (acantholysis) and blister formation.

Pemphigus vulgaris most commonly affects middle-aged and older adults but may occur at any age. Without treatment, the disease can be life-threatening due to extensive skin loss, secondary infections, fluid and electrolyte imbalance, and malnutrition. Early diagnosis and prompt immunosuppressive therapy have significantly improved patient outcomes.

Pathophysiology (Brief)

The main mechanism is autoimmune destruction of desmosomes, particularly desmoglein 3 and, in many patients, desmoglein 1.

This results in:

• Loss of adhesion between epidermal cells (acantholysis)

• Formation of fragile intraepidermal blisters

• Painful skin erosions after blister rupture

• Mucosal ulceration

Signs & Symptoms

Typical lesions include:

• Flaccid vesicles and bullae

• Painful erosions after blisters rupture

• Fragile blisters that rupture easily

• Crusted erosions

Common sites:

• Oral mucosa (often the first site affected)

• Lips

• Face

• Scalp

• Chest

• Back

• Axillae

• Groin

• Genital mucosa

Other findings:

• Painful oral ulcers

• Difficulty eating or swallowing

• Positive Nikolsky sign

• Secondary bacterial infection

• Weight loss in severe disease

Diagnostic Criteria

Diagnosis is based on:

• Flaccid blisters involving the skin and mucous membranes

• Painful oral erosions

• Positive Nikolsky sign

• Histopathological findings

• Positive direct immunofluorescence

Investigations

Recommended investigations

• Skin biopsy

• Full blood picture (FBP)

• Liver function tests (LFTs)

• Renal function tests (RFTs)

Special investigations

• Direct immunofluorescence (gold standard)

• Indirect immunofluorescence where available

• ELISA for desmoglein antibodies (where available)

Monitoring

During prolonged immunosuppressive therapy monitor:

• Full blood count

• Liver function

• Renal function

• Blood glucose in patients receiving corticosteroids

Treatment

Treatment aims to suppress autoimmune activity, heal skin and mucosal lesions, prevent relapse, and minimize treatment complications.

Non-pharmacological Treatment

• Maintain meticulous skin hygiene.

• Protect ruptured blisters with non-adherent dressings.

• Maintain adequate nutrition and hydration.

• Use antiseptic mouthwash if oral lesions are present.

• Promptly treat secondary bacterial infection.

• Regular follow-up to monitor disease activity and medication toxicity.

Pharmacological Treatment

• Betamethasone cream/ointment (topical) — apply every 12 hours until improvement.

OR

• Clobetasol propionate cream/ointment (topical) 0.05% — apply every 12 hours until improvement.

AND

• Prednisolone (PO) 0.5–1 mg/kg once daily for 2 months, then gradually taper to the lowest effective dose.

AND (for extensive involvement)

• Methotrexate (PO) 7.5–20 mg once weekly for not less than 4 months.

OR (steroid-sparing alternatives, specialist level):

• S: Methotrexate (PO) 10–15 mg once weekly for at least 4 months with folic acid.

• OR S: mycophenolate mofetil (PO) 1 g/day for at least 4 months.

• OR S: rituximab (IV) 375 mg/m² weekly for 4 weeks.

Complications

• Secondary bacterial infection

• Sepsis

• Fluid and electrolyte imbalance

• Malnutrition

• Weight loss

• Adverse effects of prolonged corticosteroid therapy

• Steroid-induced diabetes

• Osteoporosis

• Death if untreated

Prevention

There is no known method of preventing pemphigus vulgaris. However, complications can be reduced by:

• Early diagnosis

• Prompt initiation of treatment

• Good wound care

• Regular monitoring during immunosuppressive therapy

• Early treatment of secondary infections

Patient Counselling (Important)

• Pemphigus vulgaris is an autoimmune disease and is not contagious.

• Long-term treatment is usually required.

• Do not stop corticosteroids abruptly without medical advice.

• Keep affected skin clean and protected.

• Eat soft, nutritious foods if mouth ulcers are painful.

• Seek medical attention immediately if fever, pus, or rapidly worsening skin lesions develop.