Acta Dermato-Venereologica 98-8CompleteContent

742 CLINICAL REPORT Skin Manifestations in Patients with Adult-onset Immunodeficiency due to Anti-interferon-gamma Autoantibody: A Relationship with Systemic Infections Kamonwan JUTIVORAKOOL 1 , Prattana SITTIWATTANAWONG 2,3 , Kornphaka KANTIKOSUM 2 , Cameron P. HURST 4 , Chanat KUMTORNRUT 2,3 , Pravit ASAWANONDA 2 , Jettanong KLAEWSONGKRAM 5 and Pawinee RERKNIMITR 2 * Division of Infectious Diseases and 3 Dermatology Unit, Department of Medicine, King Chulalongkorn Memorial Hospital, Thai Red Cross Society, 2 Division of Dermatology, Department of Medicine, 4 Center for Excellence in Biostatistics and 5 Division of Allergy and Clinical Immunology, Department of Medicine, Faculty of Medicine, Skin and Allergy Research Unit, Chulalongkorn University, Bangkok, Thailand 1 Adult-onset immunodeficiency due to anti-interferon-γ autoantibody is an emerging acquired immunodeficien- cy with frequent skin manifestations. A retrospective chart review was conducted and identified 41 patients with the syndrome. Skin involvement was detected in 33 (80%) patients, 15 (45%) with infective skin dis- eases and 27 (82%) with reactive skin disorders. Reac- tive lesions were mostly neutrophilic dermatoses, e.g. Sweet syndrome. Of note, the presence of neutrophilic dermatoses was highly associated with infections of other sites. An adjusted odds ratio for the existence of infections in patients with neutrophilic dermatoses was 14.79 (95% CI: 5.13, 42.70; p < 0.001). Moreover, neutrophilic dermatoses were significantly correlated with opportunistic infections observed in those with defects in cell-mediated immunity including non-tu- berculous mycobacterium and disseminated fungal infection. The odds ratio for opportunistic infections in the presence of neutrophilic dermatoses was 12.35 (95% CI: 5.00, 30.55; p < 0.001). Thus, the presence of neutrophilic dermatoses in patients with the syndrome can signal opportunistic infections that warrant physi- cian attention. Key words: skin signs; neutrophilic dermatoses; Sweet syndro- me; IFN-gamma; autoantibody; acquired immunodeficiency. Accepted Apr 26, 2018; Epub ahead of print Apr 27, 2018 Acta Derm Venereol 2018; 98: 742–747. Corr: Pawinee Rerknimitr, MD, MsC, Division of Dermatology, Department of Medicine, Faculty of Medicine, Chulalongkorn University, 1873 Rama IV Road, Pathumwan, Bangkok 10330, Thailand. E-mail: pawinee.r@chula. ac.th A dult-onset immunodeficiency due to anti-interferon (IFN)-γ autoantibody is a distinct and emerging clinical entity, usually found in Asia, e.g. Thailand and Taiwan (1–5). The syndrome was first described in 2012 by Browne et al. (1). It is characterized by non-tuber- culous mycobacterium (NTM) infections of the lymph nodes which often progress to systemic dissemination. Additional co-infections with other opportunistic orga- nisms including non-typhoidal salmonella, Cryptococ- cus neoformans, Talaromyces marneffei, Histoplasma capsulatum and varicella zoster virus (VZV) of various organ systems are usually present (1). The condition is doi: 10.2340/00015555-2959 Acta Derm Venereol 2018; 98: 742–747 SIGNIFICANCE An adult-onset immunodeficiency due to anti-interferon-γ autoantibody is an emerging acquired immunodeficiency, also known as AIDS-like illness in non-HIV-infected indivi- duals. Skin manifestations, mostly neutrophilic dermatoses (ND) e.g. generalized pustular eruption is a frequent featu- re. Of note, the presence of ND was highly associated with the existence of infections of other sites. The presence of ND in patients with the syndrome can signal opportunistic infections that warrant physicians’ attentions. known as an AIDS-like illness, though these patients are non-HIV-infected and not on immunosuppressive therapy. Intriguingly, neutralizing anti-IFN-γ autoanti- bodies are found in the plasma/serum of these patients and are responsible for the increased susceptibility of infections by intracellular organisms (1, 6). The origin of the neutralizing antibodies remains elusive (1). The disease onset is around 30–50 years old (1). Essentially, skin manifestation is a frequent feature of the syndrome. Cutaneous involvement includes infec- tions of the skin itself and reactive conditions, notably, neutrophilic dermatoses (ND) such as Sweet syndrome, generalized pustular eruption, and panniculitis (1, 3, 7). However, thorough investigations focusing on skin manifestations are still lacking. In the present study, we conducted a comprehensive retrospective chart review to evaluate the prevalence, types, characteristic and treatment outcomes of skin manifestations in patients with adult-onset immunodeficiency due to anti-IFN-γ autoantibody and sought to study the temporal relation- ship of the reactive skin lesions with systemic infections. MATERIALS AND METHODS Study design and ethical consideration A retrospective chart review was conducted in King Chulalongkorn Memorial Hospital, Bangkok, Thailand. A patient registry with adult-onset immunodeficiency due to anti-IFN-γ autoantibody was established in our hospital since 2000. From this 16-year period (2000–2015), we were able to identify 41 patients in our registry. Detailed information of every patient visit was retrieved and subsequently analyzed. The study protocol was approved by the local Institutional Review Board (no 245/59). This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Journal Compilation © 2018 Acta Dermato-Venereologica.

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