Abstract
Reactive neutrophilic dermatoses in adult‐onset immunodeficiency due to interferon‐γ autoantibody (AOID) are usually associated with concomitant active opportunistic infections. Data focusing on the treatment of these dermatoses with non‐immunosuppressive drugs are still lacking. The aim of this study was to assess the efficacy and safety of acitretin treatment of reactive neutrophilic dermatoses in AOID. We conducted a retrospective review of all patients with AOID who had reactive neutrophilic dermatoses and had been treated with acitretin from January 2008 to December 2018. In total, 23 patients had been diagnosed with AOID, with 27 episodes of reactive neutrophilic dermatoses (20 episodes of Sweet syndrome and seven episodes of generalized pustular eruption) and treated with acitretin. The median effective dose of acitretin was 10 mg/day. The mean initial response was 5.6 ± 2.3 days. The rash had almost or completely cleared within 2 weeks in 70.4% of patients. One case had developed a reversible acitretin‐induced liver injury with hepatocellular pattern. The median total duration of treatment was 3 months. In conclusion, this study demonstrates the potential role of acitretin as one of the treatments of choice for reactive neutrophilic dermatoses in AOID, attributable to its favorable response and good tolerability.
Highlights
Adult-onset immunodeficiency resulting from the acquired autoantibodies to interferon-c (IFN-c; AOID) is a well-recognized disease
Skin manifestations are categorized into skin infection and reactive dermatoses, neutrophilic dermatoses, including Sweet syndrome, generalized pustular eruption and neutrophilic lobular panniculitis.[7,8]
The benefit of acitretin in reactive neutrophilic dermatoses associated with non-tuberculous mycobacteria (NTM) infection among four highly suspected cases with AOID has been previously reported from our institute.[9]
Summary
Adult-onset immunodeficiency resulting from the acquired autoantibodies to interferon-c (IFN-c; AOID) is a well-recognized disease. The IFN-c is an important cytokine in the cellmediated immune (CMI) response. Most reported cases have been from East Asia and South-East Asia, including Thailand, Taiwan, China and Japan.[1]. Patients with AOID are susceptible to disseminated opportunistic infections (OI) from intracellular organisms, such as non-tuberculous mycobacteria (NTM), Talaromyces marneffei and non-typhoidal Salmonella spp.[1–3]. The most common organ involvement reported in patients with AOID is lymph node, followed by skin and soft tissue.[4–6]. Skin manifestations are categorized into skin infection and reactive dermatoses, neutrophilic dermatoses, including Sweet syndrome, generalized pustular eruption and neutrophilic lobular panniculitis.[7,8]. These neutrophilic reactive dermatoses occur concurrently with active OI, predominantly disseminated NTM infection.[8] Patients with AOID are susceptible to disseminated opportunistic infections (OI) from intracellular organisms, such as non-tuberculous mycobacteria (NTM), Talaromyces marneffei and non-typhoidal Salmonella spp.[1–3] The most common organ involvement reported in patients with AOID is lymph node, followed by skin and soft tissue.[4–6] Skin manifestations are categorized into skin infection and reactive dermatoses, neutrophilic dermatoses, including Sweet syndrome, generalized pustular eruption and neutrophilic lobular panniculitis.[7,8] Most of the time, these neutrophilic reactive dermatoses occur concurrently with active OI, predominantly disseminated NTM infection.[8]
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