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      RARE ASSOCIATION OF HYPER IgE SYNDROME WITH CERVICAL RIB AND NATAL TEETH

      case-report

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          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.

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          Delayed eruption of permanent teeth in hyperimmunoglobulinemia E recurrent infection syndrome.

          To determine the incidence of abnormal tooth eruption in patients with hyperimmunoglobulinemia E (hyper-IgE) syndrome. This study evaluated 34 individuals with hyper-IgE syndrome (age range, 2-40 years). A comprehensive dental history and a head and neck evaluation were performed on all patients. Dental age was assessed in patients younger than 17 years by 2 methods: (1) clinical assessment of tooth eruption and (2) a radiographic method. Relationships between the chronologic age, dental developmental age, and age at tooth eruption were determined. Other oral or dental anomalies were recorded. Of patients older than 7 years, 75% reported problems with permanent tooth eruption, as evidenced by retained primary teeth or the need for elective extractions of primary teeth to allow eruption of permanent teeth. None of the patients experienced problems with eruption of primary teeth. Eruption of the first and second permanent molars also occurred on time. Dental maturity scores were established for 14 patients 17 years of age or younger. In each case, the difference between chronologic age and the estimated dental developmental age was less than 12 months; however, we found a significant discrepancy between the chronologic age and the mean age of tooth eruption in 80% of these patients when using a particular set of standardized values. Persistence of Hertwig's epithelial root sheath was observed on histologic examination. Chronic multifocal oral candidiasis was a consistent feature in patients with hyper-IgE recurrent infection syndrome. Other oral anomalies were also noted. We confirmed that a disorder of tooth eruption is part of the hyper-IgE syndrome. This problem occurs because of delayed primary tooth exfoliation rather than a developmental delay in the formation of the permanent dentition. The persistence of Hertwig's epithelial root sheath is unusual and may be associated with the lack of resorption of the primary teeth. Dentists should be aware of this feature of hyper-IgE syndrome because timely intervention will allow normal eruption to occur.
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            Natal teeth: a review.

            The incidence of natal teeth is approximately 1:2,000 to 1:3,000 live births. The most commonly affected teeth are the lower primary central incisors. Natal teeth usually occur in pairs. The eruption of more than two natal teeth is rare. The majority of natal teeth represent the early eruption of normal primary deciduous dentition. Less than 10% of natal teeth are supernumerary. Natal teeth might resemble normal primary dentition in size and shape; however, the teeth are often smaller, conical and yellowish, and have hypoplastic enamel and dentin with poor or absent root formation. Complications include discomfort during suckling, sublingual ulceration, laceration of the mother's breasts and aspiration of the teeth. A dental roentgenogram is indicated to differentiate the premature eruption of a primary tooth from a supernumerary tooth. Tooth extraction is indicated if the tooth is supernumerary or excessively mobile. If the tooth does not interfere with breastfeeding and is otherwise asymptomatic, no treatment is necessary.
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              Hyperimmunoglobulin E syndrome.

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                Author and article information

                Journal
                Indian J Dermatol
                IJD
                Indian Journal of Dermatology
                Medknow Publications (India )
                0019-5154
                1998-3611
                Oct-Dec 2009
                : 54
                : 4
                : 372-374
                Affiliations
                From the Department of Dermatology, Madras Medical College, Chennai, India.
                Author notes
                Address for correspondence: Dr. Anupama S Roshan, Plot No 21, ABC Avenue, Kaladipet Market Lane, Chennai - 600 019, Tamil Nadu, India. E-mail: dranupamaroshan@ 123456yahoo.co.in
                Article
                IJD-54-372
                10.4103/0019-5154.57617
                2807717
                20101342
                ad292160-3d35-4f56-bf1f-c62af97f4ebd
                © Indian Journal of Dermatology

                This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : March 2008
                : May 2009
                Categories
                Case Report

                Dermatology
                natal teeth,hyper ige syndrome,cervical rib
                Dermatology
                natal teeth, hyper ige syndrome, cervical rib

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