Dilek Doğruel, TurkeyÇukurova University Pediatric Immunology and Allergy
Presenter Of 1 Presentation
HYPERIMMUNOGLOBULINEMIA D SYNDROME WITH RECURRENT PERIANAL ABSCESS SUCCESSFULLY TREATED WITH CANAKINUMAB.
Background and Aims
Hyperimmunoglobulinaemia D syndrome is an autoinflammatory disease usually representing recurrent episodes of fever, arthralgia/arthritis, cervical lymphadenopathy, vomiting, diarrhoea, abdominal pain and skin rashes lasting 3-7 days every 4-8 weeks since their infancy.
Herein, we report an 18-month-old patient with recurrent attacks of fever and pharyngitis lasting 2-3 days every 10-15 days since the first two weeks of life. Inflammatory attacks were accompanied by diarrhoea, oral aphthous ulcers, cervical lymphadenopathy, maculopapular rash, severe leukocytosis and perianal fistulae/abscess.
After the initiation of canakinumab, the patient was clinically improved with complete healing of perianal fistulas/abscesses.
In conclusion, hyperimmunoglobulinaemia D syndrome should be considered in differential diagnosis of inflammatory bowel disease and recurrent perianal abscess/fistula in a patient with inflammatory attacks.