A case of netherton syndrome: successful treatment with omalizumab and pulse prednisolone and its effects on cytokines and immunoglobulin levels

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.

• Atopic diathesis
• CXCL8
• ichthyosis linearis circumflexa
• IL-4
• IL-5
• IL-1β
• IL17A
• netherton syndrome
• omalizumab

Acknowledgements

The authors thank patient.

Declaration of interest

The authors declare that they have no conflict of interest.