Paraneoplastic Pemphigus

Paraneoplastic Pemphigus

 

Paraneoplastic pemphigus is a severely debilitating blistering disease affecting skin and mucous membranes in patients with malignancy, particularly hematologic malignancies.

Epidemiology

  • Incidence – very rare
  • Age – 60 years or older, can occur younger
  • Gender – may have female predominance

Risk Factors

  • Malignancies
    • Most common – lymphoma and leukemia
    • Others include sarcomas, thymomas
    • Castleman syndrome

Pathology

  • Suprabasilar acantholysis and basal cell vacuolation
  • May have interface inflammatory changes as in lichen planus
  • Features of pemphigus vulgaris and erythema multiforme

Clinical Presentation

  • Lesions in paraneoplastic pemphigus are variable and may show flaccid bullae, lichenoid or erythema-multiforme-like lesions
  • The disease may involve esophageal, respiratory or oral epithelium in addition to skin; characteristically involves vermilion border of lips
  • Erythematous maculopapular lesions with dusky centers mimic erythema multiforme

Diagnosis

  • As in other types of pemphigus, IgG is deposited on the cell surfaces of epidermal and epithelial cells in and around affected areas
  • IgG antibodies to basement membrane zone may also be observed.
  • IgA antibodies rarely have been associated with paraneoplastic pemphigus

Differential Diagnosis

  • Pemphigus vulgaris
  • Erythema multiforme
    • Stevens Johnson syndrome (severe expression)
  • Lichen planus, lichenoid drug reaction
  • Toxic epidermal necrolysis

Treatment

  • Treat malignancy
  • Supportive care

See Also