Morphoea profunda and its relationship to eosinophilic fasciitis.

In this small case series, all eight patients were women in their fifth and sixth decades. This is similar to the female predominance in morphoea and less in keeping with eosinophilic fasciitis (EF). All cases had diffuse induration of their limbs with both proximal and distal patterns of distribution, and five of the patients exhibited peau d'orange skin. All patients had diffuse induration of the lower limbs and half had restricted ankle movements. Six patients had concomitant superficial morphoea. This group of patients demonstrated a unique subtype of the morphoea spectrum with some features overlapping with EF. However, there appear to be points of distinction, and we propose that some previously reported cases labelled as EF would be better described as having morphoea profunda (MP). Methotrexate may be a useful treatment for MP, hence it is important to distinguish this from EF, as management may differ.

Assessment of two screening tools to identify psoriatic arthritis in patients with psoriasis.

BACKGROUND: Many patients with psoriasis have undiagnosed psoriatic arthritis. Low specificity is found with many PsA screening tools. A new instrument, the CONTEST questionnaire, was developed utilizing the most discriminative items from existing instruments. OBJECTIVE: The aim of this study was to compare the CONTEST and PEST screening tools. METHODS: People attending secondary care clinics with psoriasis, but not PsA, completed the questionnaires, were assessed for function and quality of life, and had a physical examination. Patients thought to have PsA were compared to those without. The performance of CONTEST and PEST was compared using area under the receiver operating curve (AUC), and sensitivity and specificity at the previously published cut-offs. RESULTS: A total of 451 dermatology patients were approached, 35% were reviewed and 27 (17%, 95% CI 12.3-21.7) had unidentified psoriatic arthritis. The sensitivity and specificity (95% CI) of PEST were 0.60 (0.42-0.78)/0.76 (0.69-0.83) and for CONTEST 0.53 (0.34-0.72)/0.71 (0.63-0.79). The confidence limits for the AUC overlapped (AUC for PEST 0.72 (0.61-0.84), for CONTEST 0.66 (0.54-0.77). CONCLUSIONS: PEST and CONTEST questionnaires performed equally well, with no superiority of the new CONTEST tool.

Allergy to oxidized limonene and linalool is frequent in the U.K.

BACKGROUND: The oxidized forms of the fragrance terpenes limonene and linalool are known to cause allergic contact dermatitis. Significant rates of contact allergy to these fragrances have been reported in European studies and in a recent worldwide study. Patch testing to oxidized terpenes is not routinely carried out either in the U.K. or in other centres internationally. OBJECTIVES: To investigate the prevalence of contact allergy to oxidized limonene and linalool in the U.K. METHODS: Between 1 August 2011 and 31 December 2012, 4731 consecutive patients in 13 U.K. dermatology departments were tested for hydroperoxides of limonene 0·3% pet., hydroperoxides of linalool 1·0% pet., stabilized limonene 10·0% pet. and stabilized linalool 10·0% pet. Doubtful (?+) and equivocal (±) reactions were grouped together as irritant reactions. RESULTS: Two hundred and thirty-seven patients (5·0%) had a positive patch test reaction to hydroperoxides of limonene 0·3% pet. and 281 (5·9%) to hydroperoxides of linalool 1·0% pet. Irritant reactions to one or both oxidized terpenes were found in 242 patients (7·3%). Eleven patients (0·2%) had a positive patch test reaction to the stabilized terpenes alone. CONCLUSIONS: This large, multicentre U.K. audit shows a significant rate of allergy to the hydroperoxides of limonene and linalool plus a high rate of irritant reactions. Testing to the oxidized forms alone captures the majority (97·0%; 411 of 422) of positive reactions; testing to nonoxidized terpenes appears to be less useful. We recommend that the hydroperoxides of limonene and linalool be added to an extended baseline patch test series.

Scanning laser Doppler imaging may predict disease progression of localized scleroderma in children and young adults.

BACKGROUND: Localized scleroderma is a rare but potentially disfiguring and disabling condition. Systemic treatment should be started early in those with active disease in key functional and cosmetic sites, but disease activity is difficult to determine clinically. Superficial blood flow has been shown to correlate with disease activity in localized scleroderma. OBJECTIVES: To examine whether superficial blood flow measured by laser Doppler imaging (LDI) has the potential to predict disease progression and therefore select patients for early systemic treatment. METHODS: A group of 20 individuals had clinical assessment and scanning LDI blood-flow measurements of 32 affected body sites. After a mean follow-up of 8.7 months their clinical outcome was compared with the results of the initial LDI assessment. RESULTS: Eleven out of 15 patients with an assessment of active LDI had progressed clinically, and 16 out of the 17 scans with inactive LDI assessment had not progressed, giving a positive predictive value of 73% and a negative predictive value of 94%. CONCLUSIONS: We believe that LDI can be a useful tool in predicting disease progression in localized scleroderma, and it may help clinicians to decide which patients to treat early.

Comparison of three screening tools to detect psoriatic arthritis in patients with psoriasis (CONTEST study).

BACKGROUND: Multiple questionnaires to screen for psoriatic arthritis (PsA) have been developed but the optimal screening questionnaire is unknown. OBJECTIVES: To compare three PsA screening questionnaires in a head-to-head study using CASPAR (the Classification Criteria for Psoriatic Arthritis) as the gold standard. METHODS: This study recruited from 10 U.K. secondary care dermatology clinics. Patients with a diagnosis of psoriasis, not previously diagnosed with PsA, were given all three questionnaires. All patients who were positive on any questionnaire were invited for a rheumatological assessment. Receiver operating characteristic (ROC) curves were used to compare the sensitivity, specificity and area under the curve of the three questionnaires according to CASPAR criteria. RESULTS: In total, 938 patients with psoriasis were invited to participate and 657 (70%) patients returned the questionnaires. One or more questionnaires were positive in 314 patients (48%) and 195 (62%) of these patients attended for assessment. Of these, 47 patients (24%) were diagnosed with PsA according to the CASPAR criteria. The proportion of patients with PsA increased with the number of positive questionnaires (one questionnaire, 19·1%; two, 34·0%; three, 46·8%). Sensitivities and specificities for the three questionnaires, and areas under the ROC curve were, respectively: Psoriatic Arthritis Screening Evaluation (PASE), 74·5%, 38·5%, 0·594; Psoriasis Epidemiology Screening Tool (PEST), 76·6%, 37·2%, 0·610; Toronto Psoriatic Arthritis Screen (ToPAS), 76·6%, 29·7%, 0·554. The majority of patients with a false positive response had degenerative or osteoarthritis. CONCLUSION: Although the PEST and ToPAS questionnaires performed slightly better than the PASE questionnaire at identifying PsA, there is little difference between these instruments. These screening tools identify many cases of musculoskeletal disease other than PsA.

A prospective trial of the effects of isotretinoin on quality of life and depressive symptoms.

BACKGROUND: Isotretinoin is an efficacious treatment for acne, but has been controversially linked with depression. OBJECTIVES: This study aimed to examine the effects of isotretinoin on quality of life(QoL) and depression using a prospective design. METHODS: The WHOQOL-BREF QoL measure and Centre for Epidemiological Studies Depression Scale were administered to consecutive outpatients with acne who were prescribed either isotretinoin (n = 65) or antibiotic treatment (n = 31).Patients and physicians rated acne severity independently. Groups were compared at baseline with a matched community sample (n = 94) and measurements repeated at 3 months for treatment groups. RESULTS: There were no differences between the three groups at baseline in terms of age, gender, depression or overall QoL. Acne was more severe in the treatment groups (P < 0·001). Depression was negatively correlated with QoL (P < 0·001)and hence was included as a covariate in repeated-measures analyses of QoL.Acne improved over time in both treatment groups (F = 48·2, P < 0·001). There was no detectable deterioration in depression score in either group (F = 1·1, not significant). QoL in the physical and social domains improved (P < 0·001) while psychological and environmental QoL was unchanged over time. The improvement in social QoL was greater in the isotretinoin group (P < 0·05). Those patients with higher baseline depression scores showed greater improvements in physical, psychological and social QoL (P < 0·001). CONCLUSIONS: Treatment of acne improves QoL, particularly in those with more depressive symptoms at the outset. Mood deterioration was not detected, but the possibility of subtle or rare mood effects of isotretinoin cannot be ruled out.

Crystallization and preliminary crystallographic data for formyltetrahydrofolate synthetase from Clostridium thermoaceticum.

Formyltetrahydrofolate synthetase from Clostridium thermoaceticum has been crystallized using the hanging-drop method from ammonium sulphate and PEG solutions. Crystals are trigonal, the space group is R32, a = 163 A, c = 259 A. A 4.2 A resolution data set has been collected. Analysis of the data using the self-rotation function shows that tetramers have approximate 222 symmetry and are positioned on a crystallographic 2-fold axis.

Fourier transform infrared reflectance microspectroscopy study of Bacillus subtilis engineered without dipicolinic acid: the contribution of calcium dipicolinate to the mid-infrared absorbance of Bacillus subtilis endospores.

Mid-infrared spectra of spores of two strains of Bacillus subtilis, PS832 (wild-type) and FB122 (sleB spoVF), that are isogenic except for the two mutations in FB122 were obtained by Fourier transform infrared (FT-IR) reflectance microspectroscopy. The mutations in FB122 cause the spores of this strain to be devoid of dipicolinic acid (pyridine-2,6-dicarboxylic acid; DPA), a biomarker characteristic of bacterial spores. Analysis of these two strains by difference spectroscopy revealed a spectrum similar to that of calcium dipicolinate (CaDPA), a chelate salt of DPA. This difference spectrum was compared to mid-infrared spectra of both DPA and CaDPA, and was attributed to CaDPA only. This is the first report known to the authors of a genetically engineered organism being used to identify the spectral contribution of a particular cellular component.

UVB-induced collagen changes in the skin of the hairless albino mouse.

Biochemical techniques have been used to measure ultraviolet-B (UVB)-induced changes in dermal collagen composition. Hairless albino mice were irradiated dorsally with a daily dose of 62 mJ/cm2 UVB for 12, 24, 30, and 36 weeks. Nonirradiated controls were housed under identical conditions. Additional groups were irradiated for similar periods and kept for a further 6-24 weeks without irradiation. Skin samples were taken from dorsal and ventral (nonirradiated) surfaces and types I and III collagen were quantified densitometrically after cyanogen bromide digestion and polyacrylamide gel electrophoresis. Type III collagen was expressed as a percentage of the total types I and III collagen and the ratio of dorsal/ventral type III (D/V III) was determined for each mouse. The ratio increased significantly in irradiated animals whereas it decreased in the corresponding period in control animals. In irradiated mice withdrawn from UV exposure the ratio D/V III tended to revert to control levels. These data are in agreement with those of our previous human studies, which showed an increase in type III collagen in sun-exposed skin when compared with covered sites.

Late-onset erythropoietic porphyria caused by a chromosome 18q deletion in erythroid cells.

The erythropoietic porphyrias, erythropoietic protoporphyria and congenital erythropoietic porphyria, result from germline mutations in the ferrochelatase gene and uroporphyrinogen III synthase gene, respectively. Both conditions normally present in childhood but rare cases with onset past the age of 40 y have been reported. Here we show that late-onset erythropoietic protoporphyria can be caused by deletion of the ferrochelatase gene in hematopoietic cells with clonal expansion as part of the myelodysplastic process. This is the first direct demonstration of porphyria produced by an acquired molecular defect restricted to one tissue. Some other cases of late-onset erythropoietic porphyria may be explained by a similar mechanism.

Physiological Diversity of Rhizoplane Diazotrophs of the Saltmeadow Cordgrass, Spartina patens: Implications for Host Specific Ecotypes.

Diazotrophic bacteria are important contributors to salt marsh productivity, but the biotic and abiotic factors that influence their distributions and function and the extent of their diversity cannot be understood in the absence of physiological information. Here we examine the physiological diversity and distribution patterns of diazotrophic bacteria associated with the rhizoplane of the saltmeadow cordgrass, Spartina patens, in comparison with diazotrophs from other intertidal grasses (tall and short form Spartina alterniflora and Juncus roemerianus) from the same salt marsh. S. patens plants were collected from two distinct habitats, and a total of 115 strains (111 Gram negative and 4 Gram positive strains) were isolated into pure culture by stab inoculating roots and rhizomes into combined nitrogen-free semisolid media. Most strains were microaerophilic and approximately one-half were motile. API test strips were used to eliminate redundancy within the culture collection, resulting in 21 physiologically different API groups (17 Gram negative and 4 Gram positive groups). A representative strain from each API group was selected for dot blot hybridization with a nifH specific probe and 16 strains (13 Gram negative and 3 Gram positive) were scored as positive. The nifH positive API group representative strains were characterized further using BIOLOG test plates. Substrate utilization potentials defined two S. patens strain clusters, and only one S. patens strain was physiologically similar to any other strain from a different host plant origin. No distinctions could be made based on the different S. patens habitats, suggesting that the host plant may have a greater impact than abiotic environmental conditions on the distributions of the rhizoplane diazotrophs recovered.

Latex allergy in an orthognathic patient and implications for clinical management.

A 19-year-old girl with mild asthma had had 16 months of orthodontic treatment as part of the joint orthodontic/orthognathic approach to her 9.5 mm overjet. At the time of banding her second molars she developed latex protein allergy as a reaction to the operator's non-sterile powdered latex gloves. She also gave a history of allergy to other substances as well as of eczema. The patient was confirmed as allergic to latex protein by radioallergosorbent test (RAST) for IgE, requiring precautions be taken during further orthodontic procedures as well as during the subsequent orthognathic surgery for the underlying Class II skeletal pattern.

How appropriate is the WHOQOL-BREF for assessing the quality of life of adolescents?

BACKGROUND: Using mixed-methods research, we investigated whether the adult version of the WHOQOL-BREF was acceptable and feasible to use with adolescents (13-19), and what features might need to be changed to develop it. Differences from young adults quality of life (QoL) (20-30 years) could add justification. METHODS: Preliminary psychometric properties of the adult WHOQOL-BREF were examined in 208 adolescents and 204 young adults. Unhealthy adolescents diagnosed with acne or elevated depressive symptoms (CES-D) were compared with healthy adolescents. Cognitive interviewing used 'think aloud' techniques with six healthy adolescents during WHOQOL-BREF completion. Concepts and wording were thematically analysed for relevance, comprehension and comprehensiveness. RESULTS: Little data were missing from the WHOQOL-BREF suggesting some feasibility and acceptability to adolescents. Compared with adults, adolescents perceived greater access to information, a better home environment, worse pain, and medication dependency. Internal consistency reliability for adolescents was good (α = .89), especially psychological and environmental QoL. Content validity especially for social and environment domains was supported. Domains validly discriminated between high- and low-depressive symptoms but not acne groups. Additional new facets on autonomy, altruism and physical fitness and changed item contents are proposed. CONCLUSION: The evidence indicates that developing a new adolescent version is justifiable. Cross-cultural research should build on these preliminary findings.