Efficacy of acitretin in the treatment of reactive neutrophilic dermatoses in adult-onset immunodeficiency due to interferon-gamma autoantibody

J Dermatol. 2020 Jun;47(6):563-568. doi: 10.1111/1346-8138.15312. Epub 2020 Mar 23.

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.

Keywords: Sweet syndrome; acitretin; adult-onset immunodeficiency; interferon-gamma autoantibody; reactive neutrophilic dermatoses.

MeSH terms

  • Acitretin / administration & dosage*
  • Acitretin / adverse effects
  • Age of Onset
  • Autoantibodies / blood
  • Autoantibodies / immunology
  • Chemical and Drug Induced Liver Injury / epidemiology*
  • Chemical and Drug Induced Liver Injury / etiology
  • Dose-Response Relationship, Drug
  • Female
  • Humans
  • Immunologic Deficiency Syndromes / blood
  • Immunologic Deficiency Syndromes / complications*
  • Immunologic Deficiency Syndromes / drug therapy
  • Immunologic Deficiency Syndromes / immunology
  • Interferon-gamma / immunology*
  • Male
  • Middle Aged
  • Retrospective Studies
  • Sweet Syndrome / blood
  • Sweet Syndrome / drug therapy*
  • Sweet Syndrome / immunology
  • Treatment Outcome

Substances

  • Autoantibodies
  • IFNG protein, human
  • Interferon-gamma
  • Acitretin