Long‐term dupilumab therapy in Netherton syndrome with severe atopic manifestations: Case report and review of the literature

We herein present a unique patient of Netherton syndrome (NS) with ichthyosis linearis circumflexa (ILC) lesions associated with severe atopic manifestations since infancy, showing different responses of atopic and ILC lesions to a 2‐year dupilumab therapy. The atopic eczematous lesions and pruritus...

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Bibliographic Details
Published in:Australasian journal of dermatology 2023-05, Vol.64 (2), p.272-277
Main Authors: Özkaya, Esen, Günay, Muhammed Burak, Babuna Kobaner, Goncagül, Keskinkaya, Zeynep, Gökalp, Mehmet Onur
Format: Article
Language:English
Subjects:
Antibodies, Monoclonal, Humanized - therapeutic use
atopic dermatitis
Atopy
Case reports
Dermatitis, Atopic - complications
Dermatitis, Atopic - drug therapy
Dermatitis, Atopic - pathology
Hair
Humans
hypohidrosis
hypotrichosis
Ichthyosis
Lesions
Literature reviews
Netherton syndrome
Netherton Syndrome - complications
Netherton Syndrome - drug therapy
Netherton Syndrome - pathology
Patients
Pruritus
Quality of Life
Scalp
Sweating
therapy‐systemic
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Summary:We herein present a unique patient of Netherton syndrome (NS) with ichthyosis linearis circumflexa (ILC) lesions associated with severe atopic manifestations since infancy, showing different responses of atopic and ILC lesions to a 2‐year dupilumab therapy. The atopic eczematous lesions and pruritus healed remarkably, dramatically improving the patient's quality of life, whilst the scalp hair showed a clinical and light microscopic improvement. The additional recovery in axillary/pubic/extremity hair growth, sweating and nail growth in the presented case was not previously reported in NS patients treated with dupilumab. However, dupilumab had no therapeutic effect on ILC lesions which were not pruritic and showed a treatment‐independent wax and waned course.
ISSN:0004-8380
1440-0960
DOI:10.1111/ajd.13986