Pemphigus: A concise review of clinical features, diagnosis and management

Vidhya1*, Sofiya2

1Staff Nurse, Kauvery Hospital, Alwarpet, Chennai, Tamil Nadu

2Clinical Instructor, Kauvery Hospital, Alwarpet, Chennai, Tamil Nadu

*Correspondence

Introduction

Pemphigus is a group of uncommon autoimmune blistering disorders affecting the skin and, in some forms, mucous membranes. Autoantibodies directed against desmosomal proteins disrupt keratinocyte adhesion, resulting in intraepidermal blister formation and painful erosions.

Types of Pemphigus

The major forms include:

Pemphigus vulgaris: Primarily associated with antibodies against desmoglein-3 and commonly presents with fragile blisters and painful erosions.

Pemphigus foliaceus: Primarily involves desmoglein-1 and produces superficial erosions and crusting.

Paraneoplastic pemphigus: A severe form associated with an underlying neoplastic disorder and extensive mucocutaneous involvement.

Pathophysiology

Autoantibodies against desmosomal proteins interfere with keratinocyte adhesion, causing acantholysis and subsequent intraepidermal blister formation. The fragile blisters rupture easily, resulting in painful erosions.

Autoantibody production → Desmosomal disruption → Acantholysis → Intraepidermal blister formation → Erosions.

Clinical Manifestations

Common clinical features include:

  • Fragile, easily ruptured blisters
  • Painful erosions
  • Crusting and oozing
  • Bleeding from lesions
  • Mucosal involvement in appropriate types
  • Positive Nikolsky sign
  • Delayed healing of extensive lesions

Complications

Major complications include:

  • Secondary bacterial infection
  • Fluid and electrolyte imbalance
  • Protein loss and hypoalbuminemia
  • Pain and impaired mobility
  • Nutritional difficulties
  • Reduced quality of life

Diagnosis: Diagnosis is based on clinical assessment supported by immunopathological investigations.

Direct immunofluorescence: Demonstrates characteristic intercellular immunoreactant deposition in perilesional skin.

Indirect immunofluorescence: Detects circulating autoantibodies in serum.

ELISA: Detects antibodies against desmoglein-1 and desmoglein-3 and may assist in disease assessment.

Immunoelectron microscopy: May further demonstrate the location of antibody and complement deposits.

Management: The primary goals of treatment are to suppress autoimmune activity, prevent new blister formation, promote healing and reduce complications.

Systemic corticosteroids: Used to control active disease.

Immunosuppressive/steroid-sparing agents: Agents such as azathioprine, cyclophosphamide and mycophenolate mofetil may be used according to disease severity and clinical response.

Rituximab: An anti-CD20 monoclonal antibody used as an important treatment option in appropriate patients.

Supportive care: Includes wound care, pain management, nutritional support, hydration and infection prevention.

Nursing Management

Nursing care focuses on maintaining skin integrity and preventing complications.

  • Assess skin lesions and monitor for new blister formation.
  • Maintain gentle skin and wound care.
  • Prevent friction and trauma.
  • Assess and manage pain.
  • Monitor for infection.
  • Monitor hydration, nutritional status and electrolyte balance.
  • Administer medications as prescribed and monitor adverse effects.
  • Educate the patient regarding medication adherence, skin protection and follow-up.
  • Provide psychological support.

Discussion

Pemphigus can cause significant morbidity because of extensive skin and mucosal involvement. Accurate diagnosis using clinical and immunopathological investigations is essential for initiating appropriate therapy. Corticosteroids remain an important component of treatment, while immunosuppressive agents and rituximab may be used to achieve disease control and reduce corticosteroid exposure. Nursing care plays a vital role in wound management, infection prevention, pain control and patient education.

Conclusion

Pemphigus is a potentially serious autoimmune blistering disorder requiring early recognition and multidisciplinary management. Appropriate diagnosis, immunosuppressive treatment and comprehensive supportive nursing care can help control disease activity, promote healing and prevent complications.

Nightingale Journals

Kauvery Hospital
Find Doctor
Book Appointment