IgG4-related disease (IgG4-RD) is characterized by masses at multiple sites, a dense lymphoplasmacytic infiltrate containing numerous IgG4+ plasma cells, storiform fibrosis, and often elevated serum IgG4 concentrations. We present a third case of alopecia (in this instance, cicatricial) caused by IgG4-RD. Based on our findings combined with those seen in two other cases, the histopathologic features of IgG4-RD alopecia include: sparing of the epidermis, cicatricial (scarring) alopecia with a markedly decreased number of hairs, miniaturization of residual hairs, and total loss of the sebaceous glands. Groups of follicles with their associated sebaceous glands (follicular units) are replaced by an extremely dense infiltrate of lymphocytes and especially plasma cells. Histiocytic aggregates, both foamy and non-foamy, may also be present. Variable degrees of fibroplasia may be present but are not an important feature in this type of alopecia.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.