Paraneoplastic pemphigus (PNP) is an often lethal paraneoplastic mucocutaneous blistering disease that is most commonly cause by lymphoproliferative disorders [1]. Paraneoplastic autoimmune multiorgan syndrome (PAMS) is another term used to point to PNP. This term resounds the inclusion of the nonbullous cutaneous eruptions and pulmonary involvement that may develop within the setting of PNP (4).
Panel test:
· Immunogenetics:
o PNP was related with the DRB1*03 allele and HLA-Cw*14 alleles.
· Immunopathology:
o DIF shows IgG and C3 deposition, both in the intercellular substance and along the basement membrane.
· Autoantibodies:
o Autoantigens are desmosomal proteins, desmoplakin I and II, desmogleins 1 and 3, and possibly other antigens (230kDa BPAg1, envoplakin, plectin, and periplakin)
References
- Anhalt GJ, Kim SC, Stanley JR, et al. Paraneoplastic pemphigus. An autoimmune mucocutaneous disease associated with neoplasia. N Engl J Med 1990; 323:1729.
- Czernik A, Camilleri M, Pittelkow MR, Grando SA. Paraneoplastic autoimmune multiorgan syndrome: 20 years after. Int J Dermatol 2011; 50:905.
- Tsuchisaka A, Numata S, Teye K, et al. Epiplakin Is a Paraneoplastic Pemphigus Autoantigen and Related to Bronchiolitis Obliterans in Japanese Patients. J Invest Dermatol 2016; 136:399.
