Prevalence of Atopic Disease in Pediatric Patients with Hypermobile Ehlers-Danlos Syndrome.

Background

The Ehlers-Danlos syndromes (EDSs) are a group of connective tissue disorders characterized by skin laxity and hypermobile joints. EDS is associated with many comorbidities that often necessitate multidisciplinary care. Adults with EDS report more atopic conditions such as allergic rhinitis and asthma; however, gaps exist around the prevalence of atopic conditions in pediatric patients with EDS. The objective of this study was to determine the prevalence of atopic conditions (allergies, asthma, and atopic dermatitis) in pediatric patients with hypermobile EDS (hEDS) and compare it to the general pediatric population in the United States.

Methods

A convenience sample of 149 patients aged 8-22 years, with a diagnosis of hEDS and atopic disease was evaluated. Atopic conditions and medication use were reviewed.

Results

Patients were mostly females (85%) and Caucasian (89%) with an average age of 15.5 years (standard deviation: 3.1). Many patients had allergic rhinitis (46%); medication allergies (44%), with antibiotics as the most common; and asthma (39%), mild intermittent as the most common severity. All prevalences were higher compared to the general United States pediatric population except for atopic dermatitis.

Conclusions

There was an increased prevalence of atopic conditions in pediatric patients with hEDS compared to the United States pediatric population. Due to the increased prevalence of atopic conditions, health care providers should be aware of and monitor for signs of atopic and allergic conditions in pediatric patients with hEDS. Allergy and pulmonology specialists should be considered in the management of pediatric patients with hEDS if symptoms are present.