Clinical Information

Paraneoplastic pemphigus (PNP) (also paraneoplastic autoimmune multiorgan syndrome [PAMS] to denote the systemic nature of the syndrome) is an autoimmune mucocutaneous blistering disease affecting adults or, rarely, children that generally heralds the presence of an underlying malignancy.

PNP/PAMS can be defined and identified by a combination of the following features:

1. Painful stomatitis and a polymorphous cutaneous eruption with lesions that may be blistering, lichenoid, erythema multiforme-like, or morbilliform

2. Variable histopathologic findings, including acantholysis, lichenoid, or interface change

3. Variable direct immunofluorescence findings from a perilesional biopsy, often demonstrating deposition of IgG and complement in the epidermal intercellular spaces, granular/linear complement deposition along the epidermal basement membrane zone, or a lichenoid tissue reaction

4. Indirect immunofluorescence evidence of cell surface deposition on primate esophagus or rat bladder epithelium

5. Enzyme-linked immunosorbent assay evidence of serum autoantibodies against desmogleins 1 or 3 and, possibly, against bullous pemphigoid 180 and 230 antigens

The incidence of the disease is unknown, but it is less common than pemphigus vulgaris or foliaceus. Clinical features of the disease can mimic those seen in a drug reaction, erythema multiforme, Stevens-Johnson syndrome, pemphigus, lichen planus, or toxic epidermal necrolysis.

In the majority of cases, PNP/PAMS is associated with non-Hodgkin lymphoma, chronic lymphocytic leukemia, thymoma, or Castleman disease. A serious complication includes bronchiolitis obliterans, which may lead to respiratory failure.