Indian Journal of Dermatology
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Year : 2009  |  Volume : 54  |  Issue : 4  |  Page : 372-374

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

Department of Dermatology, Madras Medical College, Chennai, India

Correspondence Address:
Anupama S Roshan
Plot No 21, ABC Avenue, Kaladipet Market Lane, Chennai - 600 019, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-5154.57617

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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.

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