Sean Klepper M.D.
Artur Zembowicz M.D....
- 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
- 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
- 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.