Granuloma faciale and erythema elevatum diutinum in relation to immunoglobulin G4-related disease: An appraisal of 32 cases

Objectives: To elucidate whether granuloma faciale (GF) and erythema elevatum diutinum (EED), two inflammatory skin dermatoses, meet the consensus histopathologic diagnostic criteria for immunoglobulin G4-related disease (IgG4-RD). Methods: With institutional review board approval, we assessed the clinical, microscopic, and immunophenotypic features of skin specimens of patients with GF and EED. We compared these findings with previously published consensus diagnostic criteria for IgG4-RD. Results: Thirty-two patients (GF, n=25; EED, n=7) met study inclusion criteria. Histopathologic findings of smallvessel vasculitis, dermal fibrosis, and plasma cell infiltrates were uniformly present, and eosinophilic inflammation was frequent. No specimen met diagnostic criteria for IgG4-RD. Conclusions: Our results indicate that despite some histopathologic similarities between GF/EED and IgG4-RD, the cases did not meet the consensus immunohistochemical diagnostic criteria for IgG4-RD.