Reactive and Infective Dermatoses Associated with Adult-Onset Immunodeficiency due to Anti-Interferon-Gamma Autoantibody: Sweet's Syndrome and Beyond

Abstract

Immunodeficiency due to anti-interferon-gamma autoantibody (anti-IFN-γ autoAb) is an emerging adult-onset immunodeficiency syndrome predominantly found in Southeast Asians. It is associated with severe or disseminated infections caused by non-tuberculous mycobacteria (NTM) and other opportunistic pathogens. We describe 3 patients with anti-IFN-γ autoAb who developed reactive and infective dermatoses, and thoroughly review the existing literature on dermatoses associated with the immunodeficiency syndrome. Case 1 developed Sweet's syndrome associated with Mycobacterium chelonae lymphadenitis and penicilliosis. Case 2 suffered from multiple episodes of lobular panniculitis during recurrent infections by NTM, Penicillium marneffei and Burkholderia pseudomallei. Both cases responded to immunomodulating agents including corticosteroid and non-steroidal anti-inflammatory drugs. Case 3 had direct skin invasion by M. chelonae and responded to prolonged anti-mycobacterial therapy. A novel working algorithm is proposed for the diagnosis and treatment of these patients who may be encountered by the dermatologist and histopathologist in clinical practice.

Clinical manifestations, course, and outcome of patients with neutralizing anti-interferon-γ autoantibodies and disseminated nontuberculous mycobacterial infections

“…Neutrophilic dermatoses (NDs) are another clinical feature that can occur in patients with nAIGAs and dNTM infection. [3,8,23] Similar skin lesions were also observed in our patients (ST6). As described by Chan et al, [23] most of the NDs appeared while patients were having an active NTM infection.…”

“…[3,8,23] Similar skin lesions were also observed in our patients (ST6). As described by Chan et al, [23] most of the NDs appeared while patients were having an active NTM infection. IL-17 has been linked to certain autoimmune diseases and skin disorders, [24] and an increased percentage of IL-17 + cells was observed in the ND lesion site.…”

Fatal anti-aquaporin-4 seropositive neuromyelitis optica spectrum disorder in tuberculosis

“…An underlying immunological defect was considered in view of the clinical deterioration despite in-vitro drug susceptibility. The combined HIV-antigen/antibody test and anti-interferon-gamma autoantibody were negative [16, 17]. …”

Sweet's syndrome associated with Talaromyces marneffei and Mycobacterium abscessus infection due to anti-interferon-gamma autoantibodies

“…[12] Moreover, this was the first reported case of a patient developed SS associated with T. marneffei and M. abscessus infection due to anti-IFN-γ autoantibodies.…”

“…[1] reported a 57-year-old Chinese male with anti-IFN-γ autoantibodies positivity developed SS with T. marneffei infection. The hallmark edema in SS was not seen in that case but was present in our case.…”