title: 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.
authors: Department of Microbiology, Queen Mary Hospital, The University of Hong Kong, Hong Kong, SAR, China.
authors: Jasper Fuk-Woo
authors: JF
authors: Chan
authors: Nigel Jeremy
authors: NJ
authors: Trendell-Smith
authors: Johnny Chun-Yin
authors: JC
authors: Chan
authors: Ivan Fan-Ngai
authors: IF
authors: Hung
authors: Bone Siu-Fai
authors: BS
authors: Tang
authors: Vincent Chi-Chung
authors: VC
authors: Cheng
authors: Chi-Keung
authors: CK
authors: Yeung
authors: Kwok-Yung
authors: KY
authors: Yuen
mesh_terms: D000163:Acquired Immunodeficiency Syndrome / Q000150:complications*; D001323:Autoantibodies / Q000276:immunology*; D005260:Female; D006801:Humans; D007371:Interferon-gamma / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D009165:Mycobacterium Infections, Nontuberculous / Q000150:complications; D016926:Mycobacterium chelonae; D009181:Mycoses / Q000150:complications; D010407:Penicillium; D010446:Peptide Fragments / Q000276:immunology*; D016463:Sweet Syndrome / Q000209:etiology*
chemical_list: D001323:Autoantibodies; D010446:Peptide Fragments; C069261:interferon gamma (1-39); D007371:Interferon-gamma