558 - Atopic Disease in Pediatric Patients with Ehlers-Danlos Syndrome

Poster Number: 558
Publication Number: 558.271

Background: Ehlers-Danlos syndrome (EDS) is a connective tissue disorder often characterized by increased skin laxity and hypermobile joints. EDS affects many different body systems and patients experience a wide variety of comorbidities. Patients frequently require subspecialty care to treat the comorbidities they experience. Many adult patients with EDS present with atopic symptoms such as allergies, asthma, and eczema, however, there is little data about the frequency of atopic conditions in pediatric patients with EDS.

Objective: The objective of this study was to determine the prevalence of food allergies, allergic rhinitis, asthma, and eczema in pediatric patients with EDS.

Design/Methods: Through a retrospective chart review, 149 patients, aged 8-22 years and seen in an EDS multidisciplinary care clinic were evaluated in their entirety. Demographic information was obtained, and atopic disease diagnosis reviewed (food allergies, allergic rhinitis, asthma, and eczema). Medications to treat atopic disease were reviewed for each patient.

Results: The average patient age was 15.5 years (SD 3.1), and most identified as female (85%) and Caucasian (89%). Of the 149 patients evaluated, 21% had food allergies. Of the patients that had food allergies, 19% were prescribed injectable epinephrine. Many patients had a diagnosis of allergic rhinitis (46%), eczema (14%), and asthma (39 %). Of those with asthma, most (56%) had mild intermittent asthma, followed by mild persistent (33%), moderate persistent (8%), and severe persistent (4%). A small percentage (7%) had allergic rhinitis, asthma, and eczema. Over half of the patients (53%) were prescribed an antihistamine with almost half (46%) on multiple antihistamines.

Conclusion(s): Compared to reported national averages, pediatric patients with EDS have a higher prevalence of food allergies (6% vs. 21%), allergic rhinitis (19% vs. 46%), eczema (11% vs. 14%), and asthma (7% vs. 39%). Due to the increased prevalence of these atopic conditions, healthcare providers should be diligent and monitor for signs of atopy in pediatric patients with EDS. Specialists in allergy and pulmonology should be included in the management of pediatric patients with EDS as atopic conditions are diagnosed more frequently in this population.