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Case Reports
. 2017 Nov 6;3(6):550-552.
doi: 10.1016/j.jdcr.2017.07.019. eCollection 2017 Nov.

Infliximab therapy for Netherton syndrome: A case report

Ângela Roda  1 , Maria Mendonça-Sanches  1 , Ana Rita Travassos  1 , Luís Soares-de-Almeida  1   2   3 , Dieter Metze  4
Affiliations
Case Reports

Infliximab therapy for Netherton syndrome: A case report

Ângela Roda et al. JAAD Case Rep. .
No abstract available

Keywords: KLK5, kallikrein 5; NS, Netherton syndrome; Netherton syndrome; PAR2, protease-activated receptor 2; SPINK5, serine protease inhibitor Kazal type 5; TNF, tumor necrosis factor; TSLP, thymic stromal lymphopoietin; Th, helper T cell; ichthyosis; infliximab; tumor necrosis factor-α.

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Figures

Fig 1
Fig 1
Netherton syndrome. Light microscopic examination of scalp hair: “bamboo hair.”
Fig 2
Fig 2
Netherton syndrome. Immunofluorescence staining with polyclonal antibody against LEKTI: very weak and focal expression of LEKTI in the epidermis.
Fig 3
Fig 3
Netherton syndrome. Clinical response to infliximab. A, At week 0; B, at week 22 (fewer inflammatory lesions and desquamation).
See this image and copyright information in PMC

References

    1. Wilkinson R.D., Curtis G.H., Hawks W.A. Netherton's disease: Trichorrhexis invaginata (bamboo hair), congenital ichthyosiform erythroderma and the atopic diathesis. A histopathologic study. Arch Dermatol. 1964;89:46–54. - PubMed
    1. Comèl M. Ichthyosis linearis circumflexa. Dermatologia. 1949;98(3):133–136. - PubMed
    1. Netherton E.W. A unique case of trichorrhexis nodosa; bamboo hairs. AMA Arch Derm. 1958;78(4):483–487. - PubMed
    1. Sarri C.A., Roussaki-Schulze A., Vasilopoulos Y. Netherton syndrome: a genotype-phenotype review. Mol Diagn Ther. 2017;21(2):137–152. - PubMed
    1. Richard G., Ringpfeil F. Ichthyosis, erythroleratodermas and related disorderes. In: Bolognia J.L., Jorizzo J.L., Schaffer J.V., editors. Dermatology. 3rd Ed. Mosby Elsevier Publishing; 2012. pp. 837–870.

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