Persistent methicillin-resistant Staphylococcus aureus bacteremia in an adult patient with Netherton's syndrome: A case report

Published:March 08, 2022DOI:


      Netherton's syndrome, a rare congenital disorder, is clinically characterized by chronic dermatologic disorders such as ichthyosiform erythroderma and ichthyosis linearis circumflexa. Curable treatment is yet to be established, and corticosteroid ointment is required to maintain good dermatological condition. Because of the permanent skin barrier impairment, patients with Netherton's syndrome are considered to be vulnerable to cutaneous infections. However, its clinical characteristics are yet to be elucidated due to the limited number of reported cases. Herein, we describe the clinical course of a patient who developed persistent methicillin-resistant Staphylococcus aureus (MRSA) bacteremia. A 19-year-old Japanese woman who had been diagnosed with Netherton's syndrome in her infancy and had been applying topical corticosteroid agents all over her body since her then, was referred to our hospital because of persistent MRSA bacteremia and secondary adrenal insufficiency. The patient was diagnosed with a central line-associated bloodstream infection and was appropriately treated with antibiotics and corticosteroid therapies. We assume that the damaged skin barrier due to the congenital dermatological disorder causes a disruption in the normal bacterial flora of the skin, leading to the invasion of harmful bacteria, such as S. aureus. In addition, internal (humoral immunodeficiency by decreased antibody against bacterial polysaccharide antigens) and external (prolonged and systemic use of corticosteroid ointment) factors bring about an immunodeficiency state in such patients. We highlight that in the absence of radical treatment, clinicians need to recognize that patients with Netherton's syndrome are vulnerable to bacterial infections owing to the mixture of immunosuppressive factors.



      MRSA (methicillin-resistant Staphylococcus aureus), PICC (peripherally inserted central venous catheter), CA-MRSA (community-acquired MRSA)
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        • Emre S.
        • Metin A.
        • Demirseren D.D.
        • Yorulmaz A.
        • Onursever A.
        • Kaftan B.
        Two siblings with Netherton syndrome.
        Turk J Med Sci. 2010; 40: 819-823
        • Bitoun E.
        • Chavanas S.
        • Irvine A.D.
        • et al.
        Netherton syndrome: disease expression and spectrum of SPINK5 mutations in 21 families.
        J Invest Dermatol. 2002; 118: 352-361
        • Boskabadi H.
        • Maamouri G.
        • Mafinejad S.
        Netherton syndrome, a case report and review of literature.
        Iran J Pediatr. 2013; 23: 611-612
        • Hovnanian A.
        Netherton syndrome: skin inflammation and allergy by loss of protease inhibition.
        Cell Tissue Res. 2013; 351: 289-300
        • Renner E.D.
        • Hartl D.
        • Rylaarsdam S.
        • et al.
        Comèl-Netherton syndrome defined as primary immunodeficiency.
        J Allergy Clin Immunol. 2009; 124: 536-543
        • Fontao L.
        • Laffitte E.
        • Briot A.
        • et al.
        Infliximab infusions for netherton syndrome: sustained clinical improvement correlates with a reduction of thymic stromal lymphopoietin levels in the skin.
        J Invest Dermatol. 2011; 131: 1947-1950
        • Andreasen T.H.
        • Karstensen H.G.
        • Duno M.
        • Lei U.
        • Zachariae C.
        • Thyssen J.P.
        Successful treatment with dupilumab of an adult with Netherton syndrome.
        Clin Exp Dermatol. 2020; 45: 915-917
        • Iwamoto K.
        • Moriwaki M.
        • Miyake R.
        • Hide M.
        Staphylococcus aureus in atopic dermatitis: strain-specific cell wall proteins and skin immunity.
        Allergol Int. 2019; 68: 309-315
        • Edslev S.M.
        • Agner T.
        • Andersen P.S.
        Skin microbiome in atopic dermatitis.
        Acta Derm Venereol. 2020; 100adv00164
        • Paller A.S.
        • Kong H.H.
        • Seed P.
        • et al.
        The microbiome in patients with atopic dermatitis.
        J Allergy Clin Immunol. 2019; 143: 26-35
        • Gaudinski M.R.
        • Milner J.D.
        Atopic dermatitis and allergic urticaria: cutaneous manifestations of immunodeficiency.
        Immunol Allergy Clin. 2017; 37: 1-10
        • Rademaker M.
        • Agnew K.
        • Anagnostou N.
        • et al.
        Psoriasis and infection. A clinical practice narrative.
        Australas J Dermatol. 2019; 60: 91-98
        • Stryk S.
        • Siegfried E.C.
        • Knutsen A.P.
        Selective antibody deficiency to bacterial polysaccharide antigens in patients with Netherton syndrome.
        Pediatr Dermatol. 1999; 16: 19-22
        • Smith D.L.
        • Smith J.G.
        • Wong S.W.
        • deShazo R.D.
        Netherton's syndrome: a syndrome of elevated IgE and characteristic skin and hair findings.
        J Allergy Clin Immunol. 1995; 95: 116-123
        • Otto M.
        Community-associated MRSA: what makes them special?.
        Int J Med Microbiol. 2013; 303 (0): 324-330
        • Watanabe S.
        • Ohnishi T.
        • Yuasa A.
        • et al.
        The first nationwide surveillance of antibacterial susceptibility patterns of pathogens isolated from skin and soft-tissue infections in dermatology departments in Japan.
        J Infect Chemother. 2017; 23: 503-511
        • Japan Society of Chemotherapy
        • Japan Society for Infectious Diseases
        Guidelines for the treatment of MRSA infections.
        (Published online)
        • Fridkin S.K.
        • Hageman J.C.
        • Morrison M.
        • Sanza L.T.
        • Como-Sabetti K.
        • Jernigan J.A.
        • et al.
        Methicillin-resistant Staphylococcus aureus disease in three communities.
        N Engl J Med. N Engl J Med. 2005; 352: 1436-1444
        • Hagiya H.
        • Hisatsune J.
        • Kojima T.
        • et al.
        Comprehensive analysis of systemically disseminated ST8/non-USA300 type community-acquired methicillin-resistant Staphylococcus aureus infection.
        Intern Med. 2014; 53: 907-912