Arzu Didem Yalcin1. 1. a Department of Internal Medicine , Clinical Immunology and Allergy Unit, Antalya Training and Research Hospital , Antalya , Turkey.
Abstract
CONTEXT: Netherton syndrome (NS) is associated with the mutation in the SPINK5 gene, which codes LEKTI (lymphoepithelial Kazaltype related inhibitor), a serine protease inhibitor. As a result of aging coupled with immune deficiency, clinical symptoms may vary. METHODS: The patient was presented to our clinic with sparse and brittle hair along with pruritic, erythematous and scaling cutaneous lesions. The patient underwent a clinical examination and laboratory analyzes. Based on the clinical and laboratory findings, the patient was diagnosed with NS. Moreover, CRP, Complement-3 (C3), C4 IL-4, IL-5, IL-1β and IL-17A levels of serum were investigated as an apoptotic marker and a negative marker for inflammation. RESULTS: Having undergone omalizumab treatment and a short-term (4 months) later, he had a decreased IgE, Ig G, prolactin, CRP, IL-4, IL-5, IL-1β and IL-17A levels. The IgA, IgM and C3, C4 levels were insignificant between before and after Omalizumab treatment. CONCLUSION: To the best of our knowledge, this is the first time that an association between omalizumab and NS was documented. In conclusion allergic skin symptoms (pruritus, erythema and desquamation) and mucosal symptoms decreased in the patient.
CONTEXT: Netherton syndrome (NS) is associated with the mutation in the SPINK5 gene, which codes LEKTI (lymphoepithelial Kazaltype related inhibitor), a serine protease inhibitor. As a result of aging coupled with immune deficiency, clinical symptoms may vary. METHODS: The patient was presented to our clinic with sparse and brittle hair along with pruritic, erythematous and scaling cutaneous lesions. The patient underwent a clinical examination and laboratory analyzes. Based on the clinical and laboratory findings, the patient was diagnosed with NS. Moreover, CRP, Complement-3 (C3), C4 IL-4, IL-5, IL-1β and IL-17A levels of serum were investigated as an apoptotic marker and a negative marker for inflammation. RESULTS: Having undergone omalizumab treatment and a short-term (4 months) later, he had a decreased IgE, Ig G, prolactin, CRP, IL-4, IL-5, IL-1β and IL-17A levels. The IgA, IgM and C3, C4 levels were insignificant between before and after Omalizumab treatment. CONCLUSION: To the best of our knowledge, this is the first time that an association between omalizumab and NS was documented. In conclusion allergic skin symptoms (pruritus, erythema and desquamation) and mucosal symptoms decreased in the patient.
Authors: Tejas P Joshi; Hannah Y Wang; Prazwal Athukuri; Sarah Bohac; Morgan A Farr; Darien Hinson; Justin A Kahla; Nasim Khalfe; Dylan B McBee; Rachel Stroh; Nicole Walters; Vicky Ren Journal: Am J Clin Dermatol Date: 2022-05-23 Impact factor: 6.233
Authors: Kira Stuvel; Jorn J Heeringa; Virgil A S H Dalm; Ruud W J Meijers; Els van Hoffen; Susan A M Gerritsen; Menno C van Zelm; Suzanne G M A Pasmans Journal: Allergy Date: 2020-02-21 Impact factor: 13.146