Long-term dupilumab therapy in Netherton syndrome with severe atopic manifestations: Case report and review of the literature.
Australas J Dermatol
; 64(2): 272-277, 2023 May.
Article
en En
| MEDLINE
| ID: mdl-36745433
ABSTRACT
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.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Dermatitis Atópica
/
Síndrome de Netherton
Aspecto:
Patient_preference
Límite:
Humans
Idioma:
En
Revista:
Australas J Dermatol
Año:
2023
Tipo del documento:
Article
País de afiliación:
Turquía