Prevalence and clinical manifestations of cutaneous findings in patients with adult-onset immunodeficiency due to anti-interferon gamma autoantibodies: an eight-year retrospective study

Abstract

Background: Cutaneous findings in adult-onset immunodeficiency due to anti-interferon gamma autoantibodies (anti-IFN-γ autoAbs) are common. Currently, data on this topic are scarce.

Methods: We retrospectively reviewed medical records of 202 skin episodes from 77 patients diagnosed with adult-onset immunodeficiency due to anti-IFN-γ autoAbs. The exclusion of drug eruptions left 180 episodes from 74 patients for further analysis.

Results: Reactive dermatosis was diagnosed in 66.1%, followed by disseminated skin infection (18.3%) and local skin infection (15.6%). Neutrophilic dermatosis (ND) tended to appear on the upper part of bodies, while leg lesions were common in the non-ND. Disseminated infection occurred more frequently with ND. Mycobacterium abscessus was the most common pathogen of concomitant infection. Remission was achieved in 21.6% and was significantly associated with females.

Conclusion: Reactive dermatosis was the most common skin manifestation. ND was found in the upper part of bodies and associated with disseminated infection. Drug-free remission was scarcely achieved.