MILWAUKEE – Itching is more problematic than is pain for patients with epidermolysis bullosa, according to an online survey of 146 patients.
Symptoms reported in conjunction with itching included pain, stinging, burning, and a sensation of ants crawling on the skin, as well as a feeling that the itch was too deep to scratch, Christina Danial and her associates reported at the annual meeting of the Society for Pediatric Dermatology.
Although data from a prior study of 11 children with epidermolysis bullosa (EB) hinted that pruritus is more bothersome than pain is (Acta. Derm. Venereol. 2008;88:143-50), the results are nonetheless startling given that EB is a mutilating disease of the skin and mucosa, Ms. Danial said. Patients with this rare condition cope with blistering of the skin and/or epithelial lining of the organs, GI complications, anemia, and shortened survival due to infection or cancer.
In this study, 216 EB patients and/or caregivers registered in the Epidermolysis Bullosa Clinical Research Consortium received a 42-item online questionnaire, and 146 completed the survey. Responses were based on a 5-point Likert scale in which 1 was "never" and 5 was "always."
Itchiness was rated the most bothersome item (mean 3.3), followed by acute pain (mean 2.9), chronic pain (mean 2.7), problems eating (mean 2.7), stomach problems (mean 2.6), dental problems (mean 2.6), movement (2.5), and surgical procedures (mean 2.4), reported Ms. Danial, a medical student at Stanford (Calif.) University.
Patients with the more severe recessive dystrophic EB subtype had significantly more pruritus than did those with EB simplex (mean 3.9 vs. 3.1; P = .01).
The frequency of itching increased as the day progressed, with bedtime having the highest frequency (mean 3.8). Not surprisingly, itching was found to interfere with sleep (mean 3.1), she noted.
Sweating (mean 3.9) and stress (mean 4.0) increased itching, as did dryness (mean 4.0), heat (mean 3.8), and humidity (mean 3.5).
The 146 respondents comprised 90 patients, 36 caregivers, and 20 patient/caregivers. The average age of the respondents was 22 years; 73 were male and 73 were female.
EB patients reported that pruritus makes them feel frustrated, agitated, and as if they cannot control the itch, although activity was noted to relieve the itching, Ms. Danial reported.
Ms. Danial suggested that further investigation is necessary to determine which treatments are most effective against itching in EB, but recommended that patients avoid dryness, heat, and humidity, and consider nighttime treatment. Treatments to control pain also may help alleviate pruritus, since patients may experience pain while itching, she added.
In all, 66% of patients had dystrophic EB, 21% had EB simplex, 10% had junctional EB, and the EB subtype of 3% was unknown. Severe disease was reported in 38% of those with recessive dystrophic EB, 14% with dominant dystrophic EB, 17% with unknown dystrophic subtype, 3% with EB simplex, 36% with junctional EB, and none with unknown EB.
Ms. Danial and her coauthors reported having no financial disclosures.