Dilek Doğruel, Turkey

Çukurova University Pediatric Immunology and Allergy

Presenter Of 1 Presentation

Poster Display Autoinflammation

HYPERIMMUNOGLOBULINEMIA D SYNDROME WITH RECURRENT PERIANAL ABSCESS SUCCESSFULLY TREATED WITH CANAKINUMAB.

Lecture Time
10:09 - 10:10
Room
Poster Area
Date
19.09.2019, Thursday
Session Time
10:00 - 17:00
Board Number
4
Presentation Topic
Autoinflammation

Abstract

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.

Methods

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.

Results

After the initiation of canakinumab, the patient was clinically improved with complete healing of perianal fistulas/abscesses.

Conclusions

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.

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