Prevalence of atopic disorders and immunodeficiency in patients with ectodermal dysplasia syndromes.

BACKGROUND: Ectodermal dysplasia (ED) syndromes are a diverse group of disorders that affect multiple ectodermally derived tissues. Small studies and case reports suggest an increase in atopy and primary immunodeficiencies (PIDs) among patients with ED syndromes. OBJECTIVE: To determine the prevalence of clinical symptoms suggestive of atopy or immunodeficiency among a large cohort of children with ED syndromes. METHODS: A 9-page questionnaire was mailed to families who were members of the National Foundation for Ectodermal Dysplasias. The surveys were completed by parents of children younger than 18 years with a diagnosis of an ED syndrome or carrier state. Portions of the questionnaire were adapted from previously validated questionnaires developed by the International Study of Asthma and Allergies in Childhood (ISAAC). RESULTS: We received 347 completed questionnaires (41%). When compared with the 13- to 14-year-old children surveyed by ISAAC, we found both all-aged and age-matched children with ED syndromes, respectively, had significantly higher rates of asthma (32.2% and 37.2% vs 16.4%), rhinitis symptoms (76.1% and 78.3% vs 38.9%), and eczema (58.9% and 48.9% vs 8.2%). The prevalence of physician-diagnosed food allergies (20.7%) and PIDs (6.1%) in these ED patients also exceeded known rates in the general pediatric population. CONCLUSION: This large-scale, retrospective study demonstrates a greater reported prevalence of symptoms suggestive of atopic disorders and PIDs among children with ED syndromes than the general pediatric population. A combination of genetic and environmental factors in ED syndromes may contribute to breaches of skin and mucosal barriers, permitting enhanced transmission and sensitization to irritants, allergens, and pathogens.

Allergic contact dermatitis.

The pathophysiology of ACD follows an intricate design and results in the characteristic, delayed inflammatory response. Although the astute physician may correctly diagnose ACD from its initial, classic history and presentation, alternative diagnoses should be considered and excluded. Patch testing performed with a relevant panel of contact allergens is the ultimate confirmatory test of ACD. Correctly identifying the inciting allergen permits appropriate personal avoidance. Corticosteroids remain the principal treatment options.

Are fish oil supplements safe in finned fish-allergic patients?

Fish oil supplements are popular alternative medicines. Many manufacturers label their products with the warning "avoid this product if you are allergic to fish." The objective of this study was to determine if finned fish (FF)-allergic patients could safely tolerate fish oil supplements. Six FF-sensitive subjects as determined by history and skin testing were selected. They were skin tested with two different fish oil supplements and given an oral challenge of each supplement 1 hour apart. Vital signs were measured at baseline and at 20-minute intervals after each challenge. Spirometry was measured at baseline and 1 hour after each challenge. Six of six patients with positive skin tests to at least one FF had negative skin tests to both fish oil supplements. All six subjects then had negative oral challenges to both supplements. In this pilot study, FF-sensitive patients tolerated fish oil supplements.