Paraneoplastic pemphigus

    Article Contributors: 
    Sean Klepper M.D.
    Artur Zembowicz M.D....

    Clinical Features:

    • Associated with a multitude of neoplasms, such as hematopoietic neoplasms, carcinomas, sarcomas, thymoma and Castleman disease
    • Most commonly seen in middle-aged to elderly adults, with a male predominance
    • Affects both the skin and mucous membranes
      • Skin lesions are most common on the proximal extremities, palms and soles.  They present as polymorphous papulosquamous lesions that progress to form blisters.
      • Mucous membrane lesions present as painful erosions.
    • Patients have circulating antibodies to a variety of keratinocyte antigens.
    • Associated with a high mortality rate

    Histologic Features:

    • Variable histologic findings, which may include:
      • Suprabasal acantholysis,k often resembling pemphigus vulgaris, sometimes with vesicle formation
      • Vacuolar interface change
      • Dyskeratotic keratinocytes
      • Exocytosis of lymphocytes
      • Spongiosis
      • Upper dermal perivascular and lichenoid mononuclear infiltrate
      • Pigment incontinence
    • Immunofluorescence shows full-thickness epidermal intercellular IgG and C3 in the lower layers of the epidermis and along the basement membrance zone.
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    Cases associated with this book:

  • Paraneoplastic pemphigus
    Author: Artur Zembowicz M.D. Ph.D.

    Conference: DermatopathologyConsultations.com Teaching Collection
  • Paraneoplastic pemphigus
    Author: Artur Zembowicz M.D. Ph.D.

    Conference: DermatopathologyConsultations.com Teaching Collection