4 CONCLUSION
The variant in MVK (C.298G>A) is a mutation that can lead primary immunodeficiency in patients. Autoinflammatory syndromes always pose diagnostic and therapeutic challenges for therapists. A clinical description of the variety of periodic fever syndromes is useful in the evaluation and management of these patients. Our report highlights the broad clinical phenotype of MVK, and emphasizes the need to consider early genetic screening for young children presenting with attacks of fever associated with skin lesions. Effective management of HIDS involves a multidisciplinary approach in which rheumatologists, immunologists, and geneticists collaborate to provide comprehensive care. Moreover, genetic counseling should be provided to affected individuals and their families to discuss the inheritance pattern and the potential risk of recurrence in future pregnancies.