Noninfectious cutaneous granulomas in primary immunodeficiency disorders: report from a national registry.

[omenn syndrome]

: The association of noninfectious cutaneous granulomas with primary immunodeficiency disorders (PIDs ) is a rare but well-recognized phenomenon . With the recent advent of new classification and broadening of the list of PIDs , there is now ever-growing number of PIDs having being reported with noninfectious cutaneous granulomas or granulomatous tissue reactions . The authors observed 4 patients with cutaneous granulomas associated with PIDs that constitute 2 % of total PIDs registered with them . In this report , the authors describe these 4 patients with cutaneous granulomas /granulomatous skin reactions associated one each with common variable immunodeficiency , Omenn syndrome , combined immunodeficiency , and Blau syndrome (BS ), and briefly review the literature on various clinicopathological patterns of cutaneous granulomas with possible underlying pathogenetic mechanisms responsible for such tissue reactions in patients with PID .