Background The term benign hypermobility syndrome is applied to those children with musculoskeletal pain associated with generalized hypermobility of the joints without any associated congenital syndrome or abnormality of connective tissue, such as Marfan's or Ehlers-Danlos syndrome. The aim of this study was to determine the prevalence of joint hypermobility among school students and to define...

Stiff skin syndrome is a rare scleroderma-like disorder of unknown etiology characterized by stone-hard indurations of skin, mild hypertrichosis and limited joint mobility. No effective treatment has yet been found. Exercises and rehabilitative therapy are important in maintaining the patient's quality of life. The authors present a case of a two-year-old boy with progressive skin hardening sin...

hyper-igm syndromes are characterized by profound reduction of serum igg, iga, and ige levels with normal or increased concentrations of serum igm. cd40 ligand deficiency is x-linked form of the disease, which results in a lack of immunoglobulin class switching from igm to igg in b cells. in addition to the recurrent infections, a number of patients suffer from neutropenia. there are some evide...

the hyperimmunoglobulin e  syndrome is a rare complex primary immunodeficiency characterized by high serum ige levels, eczema, and recurrent infections. we present a case of 2-years-old girl with eczema and repeated bacterial skin and lung infections since the period of infancy. the patient also had eosinophilia, high serum levels of ige, and cows-milk hypersensitivity. we describe the case, in...

conclusions all clinical findings were directly related to the features described for eds. there are occasions when dentists would be the first health professionals to see these individuals at a young age; knowing the classic signs of eds could help in identifying and managing them properly. introduction this study aimed to elaborate a case with several complications and recommended some sugges...

background: hyper ige syndrome (hies) is a rare primary immune deficiency, described as job`s syndrome characterized by increased serum levels of ige, eczema, recurrent cutaneous and pulmonary infections. in this paper, we presented a case of hyper ige syndrome.case presentation: a 16-year-old iranian boy presented with a one year history of skin lesions in knees and elbows was diagnosed of pso...

hyper - immunoglobulin e syndrome is a rare primary immunodeficiency disease characterized by recurrent abscess formation, respiratory tract infections and very high titers of serum ige associated with peculiar face and skeletal features. we report a seven-year old  girl  presenting with persistent productive cough and  history of chronic eczematoid facial lesions since infancy  and  two episod...