Rare association of hyper IgE syndrome with cervical rib and natal teeth.

Indian Journal of Dermatology
Anupama S RoshanN Gomathy

Abstract

Hyper IgE syndrome (HIES) is a rare immunodeficiency syndrome characterized by a triad of cutaneous abscesses, mostly caused by Staphylococus aureus; pneumonia; and raised IgE levels. Nonimmunological associations include course facial features, multiple bone fractures, joint hyperextensibility, and retained primary dentition. Patients require long-term antibiotic therapy. We report here a classical case of HIES with rare associations of natal teeth, bilateral cervical ribs, and conductive deafness. The patient was being treated with monteleukast and dapsone.

References

Mar 4, 1999·The New England Journal of Medicine·B GrimbacherJ M Puck
Feb 16, 2000·Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics·A C O'ConnellS M Holland

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Citations

Sep 12, 2013·ISRN Pediatrics·Shubhangi MhaskeSwati Saawarn
Jun 30, 2015·Indian Journal of Dermatology·Subha V PatilPradeep M Mahajan

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Methods Mentioned

BETA
X-ray

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