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

Roshan AS, Janaki C, Parveen B, Gomathy N - Indian J Dermatol (2009)

Bottom Line: 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.The patient was being treated with monteleukast and dapsone.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Madras Medical College, Chennai, India. dranupamaroshan@yahoo.co.in

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.

No MeSH data available.


Related in: MedlinePlus

Chest X-ray revealing bilateral cervical ribs
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC2807717&req=5

Figure 0005: Chest X-ray revealing bilateral cervical ribs

Mentions: Staphylococeous aureus sensitive to amikacin and ceftrioxone was grown from the pus. Scraping of scalp revealed dermatophyte hyphae. Absolute eosinophil count was 2710 and IgE was 1,10,000. Chest X-ray revealed bilateral cervical ribs, but was otherwise clear. Skull X-ray showed osteopenic lesions and retained primary dentition [Figures 5 and 6] and hip joint X-ray showed dislocation on the right side. CT scan of the skull showed craniosynostosis with scalp abscess in the right parietal region.


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

Roshan AS, Janaki C, Parveen B, Gomathy N - Indian J Dermatol (2009)

Chest X-ray revealing bilateral cervical ribs
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2807717&req=5

Figure 0005: Chest X-ray revealing bilateral cervical ribs
Mentions: Staphylococeous aureus sensitive to amikacin and ceftrioxone was grown from the pus. Scraping of scalp revealed dermatophyte hyphae. Absolute eosinophil count was 2710 and IgE was 1,10,000. Chest X-ray revealed bilateral cervical ribs, but was otherwise clear. Skull X-ray showed osteopenic lesions and retained primary dentition [Figures 5 and 6] and hip joint X-ray showed dislocation on the right side. CT scan of the skull showed craniosynostosis with scalp abscess in the right parietal region.

Bottom Line: 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.The patient was being treated with monteleukast and dapsone.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Madras Medical College, Chennai, India. dranupamaroshan@yahoo.co.in

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.

No MeSH data available.


Related in: MedlinePlus