Pemphigus vegetans Management

Pemphigus vegetans

Pemphigus vegetans is a rare variant of pemphigus vulgaris, characterized by vegetating (wart-like), hypertrophic plaques that develop after fragile blisters rupture. It is a chronic autoimmune blistering disease in which autoantibodies attack proteins responsible for adhesion between epidermal cells, resulting in blister formation followed by excessive tissue overgrowth during healing.

The disease primarily affects skin folds (intertriginous areas) such as the axillae and groin and may also involve the oral mucosa. Although less common than pemphigus vulgaris, pemphigus vegetans requires early diagnosis and immunosuppressive therapy to prevent complications and improve quality of life.

Pathophysiology (Brief)

The main mechanism is autoimmune destruction of desmosomes, primarily involving desmoglein 3 and sometimes desmoglein 1.

This leads to:

• Loss of adhesion between epidermal cells (acantholysis)

• Formation of fragile blisters

• Rupture of blisters

• Excessive epidermal hyperplasia during healing, producing vegetating plaques

Signs & Symptoms

Typical lesions include:

• Flaccid vesicles and bullae

• Moist, vegetating plaques

• Thick crusted erosions

• Painful fissures

• Mild itching or burning

Common sites:

• Axillae

• Groin

• Perineum

• Neck folds

• Inframammary folds

• Oral mucosa

Other findings:

• Positive Nikolsky sign

• Painful oral erosions

• Foul-smelling vegetative lesions due to secondary infection

• Difficulty eating when oral lesions are extensive

Diagnostic Criteria

Diagnosis is based on:

• Vegetating plaques occurring in flexural areas

• History of flaccid blisters

• Mucosal involvement

• 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 prolonged corticosteroid therapy

Treatment

Treatment aims to suppress autoimmune activity, heal skin and mucosal lesions, prevent secondary infection, and reduce disease recurrence.

Non-pharmacological Treatment

• Maintain meticulous skin hygiene.

• Keep skin folds clean and dry.

• Use non-adherent dressings over erosions.

• Maintain adequate nutrition and hydration.

• Promptly treat secondary bacterial or fungal infections.

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

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

• Secondary fungal infection

• Painful skin fissures

• Fluid and protein loss in extensive disease

• Adverse effects of prolonged corticosteroid therapy

• Disease relapse

• Reduced quality of life

Prevention

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

• Early diagnosis and treatment

• Maintaining good skin hygiene

• Keeping flexural areas dry

• Regular follow-up

• Prompt treatment of secondary infections

Patient counselling (Important)

• Pemphigus vegetans is a chronic autoimmune disease and is not contagious.

• Long-term treatment is usually required to maintain disease control.

• Keep affected skin folds clean and dry.

• Do not stop corticosteroids or immunosuppressive medicines without medical advice.

• Report fever, increasing redness, foul-smelling discharge, or rapidly worsening skin lesions immediately.