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.

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