Clinical and immunopathological spectrum of immunoglobulin M pemphigoid: a multicenter case series.

ABSTRACT:

Background and objectives

Pemphigoid diseases are primarily mediated by IgG or IgA autoantibodies against the cutaneous basement membrane zone (BMZ). Although recent observations suggest the existence of exclusively IgM-mediated pemphigoid disease, a larger study is lacking.

Patients and methods

This prospective multicenter study included ten patients with exclusive IgM deposition along the BMZ by direct immunofluorescence (IF). Circulating IgM was detected by indirect IF and immunoblotting with recombinant BP180.

Results

The cohort, four females and six males with a median age of 77 years, presented predominantly with prurigo-like lesions without blisters and mucosal involvement. Sera from nine patients demonstrated linear IgM labeling the blister roof of human salt-

SUBMITTER: Yilmaz K 

PROVIDER: S-EPMC12697332 | biostudies-literature | 2025 Dec

REPOSITORIES: biostudies-literature