Immunobullous Diseases Testing Algorithm - Neoplastic Disease

This algorithm outlines a workup, including biopsy and serum antibody testing, for patients with mucocutaneus lesions and possible or known neoplastic disease. If testing is negative for paraneoplastic pemphigus (PNP), also known as paraneoplastic autoimmune multiorgan syndrome (PAMS), testing for other immunobullous diseases should be performed. In patients with PNP/PAMS and other immunobullous diseases, biopsy and serum testing together provide the most useful diagnostic information. However, it is important to note that in individuals with PNP/PAMS, biopsy results may be positive when serology is negative, and vice versa.

The testing used for suspected PNP/PAMS differs from that used to assess for paraneoplastic neurologic syndromes. For additional information on testing for autoimmune neurologic diseases such as paraneoplastic neurologic syndromes, refer to Autoimmune Neurologic Diseases - Antineural Antibody Testing.

Refer to ARUP Laboratories’ test offerings below.

ARUP Laboratory Tests

Recommended Tests

Includes epithelial cell surface/intercellular substance (ICS) and basement membrane zone (BMZ) IgG antibodies by IIF using rodent substrates (rat bladder and mouse bladder, heart, and liver) and envoplakin, IgG antibody by ELISA
Includes cell surface/intercellular substance (ICS) and BMZ IgG and IgA antibodies by IIF using three substrates (monkey esophagus, intact human skin, and human BMZ-split skin, also known as salt-split skin); desmoglein 1 and 3 antibodies, IgG by ELISA; BP180 and BP230 antibodies, IgG by ELISA; and type VII collagen antibodies, IgG by ELISA