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

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.

Factors causing oral and skin pathological featuresin the hyperimmunoglobulin E syndrome patient including the environmental component: a review of the literature and own experience

“…Therefore, HIES sufferers may complain of double rows of teeth with both primary and permanent intermixed dentition [ 4 ]. Roshan et al presented a case of a 10-year-old girl whose skull X-ray revealed retained teeth with the history of natal teeth (the upper two incisors were present at birth) as well as osteopenic lesions [ 16 ]. O’Connell et al .…”

“…Therefore, HIES sufferers may complain of double rows of teeth with both primary and permanent intermixed dentition [4]. Roshan et al presented a case of a 10-yearold girl whose skull X-ray revealed retained teeth with the history of natal teeth (the upper two incisors were present at birth) as well as osteopenic lesions [16]. O'Connell et al showed that 75% of patients above the age of 7 years reported problems with the retention of deciduous teeth including retained teeth, "double teeth" or elective extractions or exfoliation of milk teeth in adolescence and beyond [13].…”

“…HIES is a multi-system disorder, with non-immunological and immunological abnormalities, and affects the dentition, skeleton, connective tissue and immune system [6,[8][9][10][11][12][13][14][15]. However, the syndrome is characterized by a triad of the most typical symptoms, such as pneumonia with pneumatocele for-mation, recurring staphylococcal skin abscesses as well as a high serum concentration of IgE [8,10,16].…”

A case of hyper immunoglobulin-e syndrome associated with scrotal tongue and intraoral hyperpigmentation

“…[ 3 ] Characteristic facies include greater interalar width, longer outer canthal distance and prominent brows/supraorbital ridge with deep-seated eyes. [ 6 ] Intraorally, retention of primary dentition, high arched palate, central tongue depressions and recurrent oral candidiasis occur. [ 3 8 ] Skeletal abnormalities include hyperextensibility of joints, scoliosis and osteoporosis.…”

“…[ 8 ] Interferons, immunoglobulin supplementation, cyclosporine A, methotrexate, levamisole, cimetidine, ascorbic acid and transfer factor have occasionally been used. [ 6 7 ]…”