Translating Patient-Oriented Eczema Measure (POEM) scores into clinical practice by suggesting severity strata derived using anchor-based methods.

BACKGROUND: The Patient-Oriented Eczema Measure (POEM) is a validated, patient-derived assessment measure for monitoring atopic eczema severity, although further information on how different POEM scores translate into disease severity categories is needed for clinical trials, epidemiological research and audit. OBJECTIVES: We sought to determine the relationship between Patient-Oriented Eczema Measure (POEM) scores (range 0-28) and two Global Questions (GQ1 and 2) concerning patients'/parents' views of the overall severity of their/their child's atopic eczema, in order to stratify POEM scores into five severity bands. METHODS: POEM scores and GQs were completed by 300 patients from general practice and 700 patients from dermatology outpatient clinics, including 300 adults aged ≥ 16 years and 700 children. RESULTS: The mean POEM score was 13·6 (range 0-28), and standard deviation (SD) was 7·2. Mean GQ1/GQ2 scores were 2·1/2·1, respectively (range 0-4 and SD 1·1 for both). The mean, mode and median of the GQ scores for each POEM score were used to devise possible POEM bandings. The proposed banding for POEM scores are: 0-2 (clear/almost clear); 3-7 (mild); 8-16 (moderate); 17-24 (severe); 25-28 (very severe), kappa coefficient 0·46. CONCLUSIONS: Severity banding of the POEM will allow more clinically meaningful use in everyday clinical practice and as a core outcome measure in future atopic eczema research.

Reliability testing of the Six Area, Six Sign Atopic Dermatitis severity score.

BACKGROUND: The Six Area, Six Sign Atopic Dermatitis (SASSAD) severity score is an objective atopic eczema severity index designed principally to assess response to treatment in therapeutic trials. Validity has been demonstrated in single and multicentre clinical trials, although data on the reliability of the index have not previously been published. OBJECTIVES: To assess inter- and intraobserver variability of the SASSAD index. METHODS: Six observers with experience in the assessment of atopic eczema were each asked to score disease severity in six patients with moderate to severe atopic eczema using the SASSAD index. Repeat observations were carried out on randomly selected patients by each observer to estimate intraobserver variation. RESULTS: The interobserver variation in total SASSAD scores for each patient ranged from 7 to 30 (median 15.5) out of a maximum possible score of 108. The intraclass correlation coefficient ri for the total scores among all six observers was quite high at 0.70, although interobserver agreement for individual components of the index was poor to moderate. The maximum recorded intraobserver variation in total SASSAD score in any of the examined patients was 8 units. CONCLUSIONS: As with many other tested atopic eczema scoring indices, the SASSAD index is subject to significant interobserver variation, reflecting the difficulties in reliably assessing eczema severity objectively.

Topical corticosteroid phobia in patients with atopic eczema.

Topical corticosteroids are widely prescribed by dermatologists caring for patients with atopic eczema. Patients' fears about using topical corticosteroids may have important implications for compliance with treatment. We carried out a questionnaire-based study of 200 dermatology outpatients with atopic eczema (age range 4 months-67.8 years) to assess the prevalence and source of topical corticosteroid phobia. We also questioned patients on their knowledge of the potencies of different topical corticosteroids. Overall, 72.5% of people worried about using topical corticosteroids on their own or their child's skin. Twenty-four per cent of people admitted to having been non-compliant with topical corticosteroid treatment because of these worries. The most frequent cause for concern was the perceived risk of skin thinning (34.5%). In addition, 9.5% of patients worried about systemic absorption leading to effects on growth and development. The most commonly used topical corticosteroid was hydrocortisone, yet 31% of patients who used this preparation classified it as either strong, very strong or did not know the potency. Only 62.5% of the 48 patients who had used both Dermovate (Glaxo) and hydrocortisone in the past were able to correctly grade Dermovate as being more potent than hydrocortisone. The most common source of patient information regarding topical corticosteroid safety was the general practitioner. Although skin thinning and systemic effects can develop very occasionally in people using topical corticosteroids, the concern expressed by people using them seems out of proportion in relation to the evidence of harm. This study highlights the need for provision of better information and education to patients and possibly general practitioners regarding the safety, potency and appropriate use of topical corticosteroids.

The Nottingham Eczema Severity Score: preliminary refinement of the Rajka and Langeland grading.

A method for assessing disease severity of atopic dermatitis (AD) in children has been developed for population-based research. Based on an index first described by Rajka and Langeland in 1989, disease severity is determined by evaluating the three elements of clinical course, disease intensity and extent of examined AD. This paper describes development of the index for use in epidemiological studies based on a community-based study of 290 pre-school children (aged 1-5 years). Construct validity of the index was evaluated with respect to clinical severity assessment according to a dermatologist, parental severity assessment, use of topical corticosteroids and impairment of quality of life. The severity distribution of AD in this community-based sample of children was: mild 82% (n = 237), moderate 12% (n = 36) and severe 6% (n = 17) according to this new index. In this sample 24% of children had suffered from AD of more than 9 months duration in the preceding 12 months, 4.5% had experienced significant sleep loss (6 or more nights of average sleep loss per week over 12 months) and 11% had experienced widespread extent of involvement (more than 10 body sites involved). Construct validity of the index was demonstrated for clinical and patient-derived severity assessment. This included a comparison between the new index and a global severity assessment by a dermatologist in which exact agreement was achieved in 88% of the cases. A small subgroup of children suffering from persistent localized forms of AD (discoid pattern, hand/ foot dermatitis, perioral dermatitis), who reported considerable morbidity, was identified using quality of life measures of severity; they would otherwise have been misclassified by the dermatologist or new index. Preliminary use of the Nottingham Eczema Severity Score would support further development as a research tool for a simple assessment of disease severity that could be used in epidemiological studies. Further validation is required with respect to use in older children, administration by researchers/health professionals and development as a wholly questionnaire-based assessment.

Characterization of photosensitivity in the Smith-Lemli-Opitz syndrome: a new congenital photosensitivity syndrome.

Photosensitivity has recently been reported as a feature of the Smith-Lemli-Opitz syndrome (SLO). The aim of this study was to establish the photobiological features of this disorder and to examine the hypothesis that the photosensitivity is caused by the high levels of 7-dehydrocholesterol found in SLO. All known cases of SLO in the U.K. were reviewed and clinical details of photosensitivity were recorded in detail. The action spectrum of the photosensitive eruption was defined by monochromator light testing. Thirteen of the 23 subjects (57%) had severe photosensitivity, and in 10 there was no photosensitivity. No correlation was identified between levels of 7-dehydrocholesterol and severity of photosensitivity, suggesting that the photosensitivity in SLO is not caused by a direct phototoxic effect mediated by 7-dehydrocholesterol. A novel pattern of photosensitivity was observed, with onset of a sunburn-like erythema on sun-exposed skin within minutes of sun exposure, which persisted in most cases for up to 24-48 h before fading. Monochromator light testing in three subjects showed an ultraviolet (UV) A-mediated photosensitivity eruption with greatest photosensitivity at 350 nm. Photosensitivity is a common and prominent feature of SLO and appears to be UVA-mediated. Elucidation of its biochemical basis may provide insight into normal cutaneous protective mechanisms against UVA-induced photodamage, and also sun sensitivity in general.

Measurement of body surface area involvement in atopic eczema: an impossible task?

Estimation of the area of skin involvement is an important part of atopic eczema severity scoring systems. We examine the variation seen when six dermatologically trained observers assessed disease extent in six patients with atopic eczema using the rule of nines. The median scores ranged from 4.8% to 37.2%. The level of agreement for the classification of scores into quintile categories was 55%, with a chance-corrected agreement (kappa statistic) of 0.09, representing very poor interobserver agreement. To measure the effects of health care intervention accurately, reliable methods of assessment should be used. Scoring methods in which reliability cannot be demonstrated may be misleading and should be interpreted with caution.

Photosensitivity associated with the Smith-Lemli-Opitz syndrome.

A case of severe photosensitivity in a girl with the Smith-Lemli-Opitz syndrome is reported. Children with this recessively inherited metabolic disorder of cholesterol metabolism present with a variety of congenital abnormalities of the nervous system and internal organs in association with varying degrees of mental retardation. Photosensitivity is a feature which has previously only briefly been mentioned in the literature in association with this syndrome. However, more recently, it has become apparent that photosensitivity is not uncommon among children with the Smith-Lemli-Opitz syndrome, although the nature of the photosensitivity in these patients has remained undefined. Our patient has suffered from sunlight intolerance since early infancy, with redness and pruritus of sun-exposed skin developing within minutes of sun exposure. Monochromator ultraviolet (UV) radiation and visible light testing revealed an immediate and persistent reaction to low-dose UVA at 350 nm, and an abnormal erythemal response to visible light at 400 nm.