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BackgroundClinical scores to determine early (6-month) antiretroviral therapy (ART) mortality risk have not been developed for sub-Saharan Africa (SSA), home to 70% of people living with HIV. In the absence of validated scores, WHO eligibility criteria (EC) for ART care intensification are CD4 MethodsWe used Botswana XPRES trial data for adult ART enrollees to develop CD4-independent and CD4-dependent multivariable prognostic models for 6-month mortality. Scores were derived by rescaling coefficients. Scores were developed using the first 50% of XPRES ART enrollees, and their accuracy validated internally and externally using South African TB Fast Track (TBFT) trial data. Predictive accuracy was compared between scores and WHO EC.ResultsAmong 5553 XPRES enrollees, 2838 were included in the derivation dataset; 68% were female and 83 (3%) died by 6 months. Among 1077 TBFT ART enrollees, 55% were female and 6% died by 6 months. Factors predictive of 6-month mortality in the derivation dataset atp 37.5 °C (2 points). The same variables plus CD4 ConclusionsSensitivity of the CD4-independent score was nearly twice that of WHO stage in predicting 6-month mortality and could be used in settings lacking CD4 testing to inform ART care intensification. The CD4-dependent score improved specificity versus WHO EC. Both scores should be considered for scale-up in SSA.

BACKGROUND: Bullous pemphigoid is a blistering skin disorder with increased mortality. We tested whether a strategy of starting treatment with doxycycline gives acceptable short-term blister control while conferring long-term safety advantages over starting treatment with oral corticosteroids. METHODS: We did a pragmatic, multicentre, parallel-group randomised controlled trial of adults with bullous pemphigoid (three or more blisters at two or more sites and linear basement membrane IgG or C3). Participants were randomly assigned to doxycycline (200 mg per day) or prednisolone (0·5 mg/kg per day) using random permuted blocks of randomly varying size, and stratified by baseline severity (3-9, 10-30, and >30 blisters for mild, moderate, and severe disease, respectively). Localised adjuvant potent topical corticosteroids (

Summary Toxic epidermal necrolysis (TEN) is a life-threatening, immune-mediated reaction, characterized by severe cutaneous and mucosal blisters and erosions. It often presents with flu-like symptoms, followed by a maculopapular, urticarial, purpuric or erythema multiforme-like eruption, which then evolves into blisters and sheet-like erosions. Presentation with pustules, however, is not well described in the English literature, and may lead to delayed diagnosis. We present two unusual cases of TEN that initially presented with pustular lesions.

Germline mutations of the fumarate hydratase (FH, fumarase) gene are found in the recessive FH deficiency syndrome and in dominantly inherited susceptibility to multiple cutaneous and uterine leiomyomatosis (MCUL). We have previously reported a number of germline FH mutations from MCUL patients. In this study, we report additional FH mutations in MCUL and FH deficiency patients. Mutations can readily be found in about 75% of MCUL cases and most cases of FH deficiency. Some of the more common FH mutations are probably derived from founding individuals. Protein-truncating FH mutations are functionally null alleles. Disease-associated missense FH changes map to highly conserved residues, mostly in or around the enzyme's active site or activation site; we predict that these mutations severely compromise enzyme function. The mutation spectra in FH deficiency and MCUL are similar, although in the latter mutations tend to occur earlier in the gene and, perhaps, are more likely to result in a truncated or absent protein. We have found that not all mutation-carrier parents of FH deficiency children have a strong predisposition to leiomyomata. We have confirmed that renal carcinoma is sometimes part of MCUL, as part of the variant hereditary leiomyomatosis and renal cancer (HLRCC) syndrome, and have shown that these cancers may have either type II papillary or collecting duct morphology. We have found no association between the type or site of FH mutation and any aspect of the MCUL phenotype. Biochemical assay for reduced FH functional activity in the germline of MCUL patients can indicate carriers of FH mutations with high sensitivity and specificity, and can detect reduced FH activity in some patients without detectable FH mutations. We conclude that MCUL is probably a genetically homogeneous tumour predisposition syndrome, primarily resulting from absent or severely reduced fumarase activity, with currently unknown functional consequences for the smooth muscle or kidney cell.

The purpose of this study was to validate the use of HPlusQA, spot-scanning proton therapy (SSPT) dose calculation software developed at The University of Texas MD Anderson Cancer Center, as second-check dose calculation software for patient-specific quality assurance (PSQA). The authors also showed how HPlusQA can be used within the current PSQA framework.The authors compared the dose calculations of HPlusQA and the Eclipse treatment planning system with 106 planar dose measurements made as part of PSQA. To determine the relative performance and the degree of correlation between HPlusQA and Eclipse, the authors compared calculated with measured point doses. Then, to determine how well HPlusQA can predict when the comparisons between Eclipse calculations and the measured dose will exceed tolerance levels, the authors compared gamma index scores for HPlusQA versus Eclipse with those of measured doses versus Eclipse. The authors introduce the αβγ transformation as a way to more easily compare gamma scores.The authors compared measured and calculated dose planes using the relative depth, z∕R × 100%, where z is the depth of the measurement and R is the proton beam range. For relative depths than less than 80%, both Eclipse and HPlusQA calculations were within 2 cGy of dose measurements on average. When the relative depth was greater than 80%, the agreement between the calculations and measurements fell to 4 cGy. For relative depths less than 10%, the Eclipse and HPlusQA dose discrepancies showed a negative correlation, -0.21. Otherwise, the correlation between the dose discrepancies was positive and as large as 0.6. For the dose planes in this study, HPlusQA correctly predicted when Eclipse had and had not calculated the dose to within tolerance 92% and 79% of the time, respectively. In 4 of 106 cases, HPlusQA failed to predict when the comparison between measurement and Eclipse's calculation had exceeded the tolerance levels of 3% for dose and 3 mm for distance-to-agreement.The authors found HPlusQA to be reasonably effective (79% ± 10%) in determining when the comparison between measured dose planes and the dose planes calculated by the Eclipse treatment planning system had exceeded the acceptable tolerance levels. When used as described in this study, HPlusQA can reduce the need for patient specific quality assurance measurements by 64%. The authors believe that the use of HPlusQA as a dose calculation second check can increase the efficiency and effectiveness of the QA process.

Summary Background It is unclear whether clinical features of pemphigoid gestationis (PG), such as timing of onset and severity, may affect pregnancy outcomes or whether the adverse outcomes in pregnancies complicated by PG are related to or worsened by systemic corticosteroid treatment. Objectives To evaluate the associations of adverse pregnancy outcomes with clinical features, autoantibody titre of PG, and systemic corticosteroid treatment. Methods We conducted a retrospective cohort study recruiting 61 pregnancies complicated by PG from the St John’s Institute of Dermatology database which enrolled cases from dermatologists across the U.K., and two tertiary hospitals in the U.K. and Taiwan. Outcome measures included gestational age at delivery, preterm birth, birthweight, low birthweight (LBW, i.e. birthweight

Giant congenital pigmented naevi and neurofibromatosis (NF-1) may rarely occur together. We report an unusual case where extensive congenital melanocytic naevi were associated with neurofibroma-like lesions that were clinically and histologically confused with neurofibromatosis. The development of malignant melanomas within the pigmented and pendulous lesions representing multiple congenital melanocytic naevi highlights the importance of an accurate diagnosis and a close follow-up of such patients.

Concerns were raised in our department when four of our patients receiving PUVA treatment developed internal malignancy. We reviewed the medical and phototherapy case notes of patients who received either systemic or bath PUVA therapy in our department between 1986 and 1999. Among the 197 patients for whom we were able to trace the hospital records we identified five patients with internal malignancies. Over the same period (1986-1999) we calculated, using the Kaplan-Meier nonparametric estimator, that 4.6 cases of internal malignancy would have been anticipated in our study population. Therefore PUVA therapy did not appear to be a risk factor for internal malignancy.

Background Viral warts represent a large workload for dermatology departments. Cryotherapy with liquid nitrogen is the most widely used method of treatment by dermatologists and is increasingly used by general practitioners in the U.K. Existing data relating to the response to cryotherapy are virtually all derived from the use of a cotton wool bud as the applicator. There is an increasing trend to use the cryo-spray to freeze warts and it has been assumed that this is equally effective. In view of the workload involved it is important to test this assumption. Objectives This prospective study was undertaken to compare these two methods of liquid nitrogen cryotherapy with regards to cure rate after 3 months of treatment. Methods Patients referred to two hospital dermatology departments with hand or foot warts were allocated to have liquid nitrogen applied with either a cryo-spray or with a cotton wool bud. Using either technique, liquid nitrogen was applied until ice-ball formation had spread from the centre to include a margin of 2 mm around each wart. Treatment was done fortnightly for up to 3 months. Plantar warts were pared and treated with a double freeze–thaw cycle. The endpoint of the study was complete clearance of all warts. Results Three hundred and sixty-three patients were enrolled, mean age 21 years (range 3–75), 188 male and 175 female. The mean duration of the warts was 98 weeks (median 78, range 2–936). The number of warts on the hands and feet varied from one to 80 (mean 5). The treatment groups were comparable with regards to baseline demographics. Two hundred and seven patients were evaluable. Cure rates at 3 months were 47% in the cotton wool bud group and 44% in the cryo-spray group (P = 0·8). Warts that had been present for 6 months or less (n = 31) had a greater chance of clearance (84%) compared with warts that had been present for more than 6 months (39%, n = 176) (P

We present the results of a questionnaire-based survey amongst the general practitioners and consultant dermatologists regarding maximum acceptable waiting times for a range of skin conditions. Statistically significant differences were found in the relative prioritization between the general practitioners and the consultants for a selection of the diagnoses. This has implications for planning and implementation of future services in general and developing waiting list prioritization scoring systems in particular.

Pityriasis rosea is a relatively common skin disorder. In its typical form it is easily recognizable; however, atypical forms can pose diagnostic problems. We report a 44-year-old woman with an acute onset of a localized eruption on her left breast. The morphology of the rash and the time course were typical of pityriasis rosea. Localized pityriasis rosea is an unusual variant, which has been described previously.

In our departments, curettage and cautery (CC) and liquid nitrogen cryotherapy are the preferred methods of treatment for Bowen's disease (BD).We aimed to compare these two treatments with regard to efficacy, time to heal, morbidity and recurrence rate.Cryotherapy was performed using a liquid nitrogen spray giving two freeze-thaw cycles, each freeze cycle being maintained for 5-10 s after the formation of an ice ball to the intended margin. Curettage was performed with a conventional disposable curette under local anaesthesia, and electrocautery was then used for haemostasis.Eighty lesions in 67 patients (55 female) were analysed. The mean age of the patients was 74 years (range 46-89). The most frequent site was the lower leg, below the knee (n = 59, 74%). The average time taken for complete healing after the procedure was 60 days. The mean size of the lesions was 336 mm(2) (range 30-1890). The patients were followed up for a mean of 22 months (range 6-24, median 2 years). In the cryotherapy group (n = 36 lesions), the median time to complete healing was 46 days (range 14-210; mean 69). Twelve lesions took more than 90 days to heal. Infection requiring antibiotics developed in four patients. Thirteen of the treated lesions had recurred by 24 months. In the CC group (n = 44 lesions), the median time to healing was 35 days (range 14-330; mean 53). Six of the lesions took more than 90 days to heal. Infection developed in two patients. Recurrence occurred in four lesions over the follow-up period. Considering BD on the lower legs separately, lesions took on average 90 days to heal in the cryotherapy group (n = 23), whereas in the CC group (n = 36) they took 39 days to heal (P0.001). During the procedure and the subsequent 24 h, patients were 10.4 times more likely to report pain of any degree for lesions treated by cryotherapy than by CC (P0.001).This study suggests a superiority of CC over cryotherapy in the treatment of BD, especially for lesions on the lower leg. Curettage of lesions of BD is associated with a significantly shorter healing time, less pain, fewer complications and a lower recurrence rate when compared with cryotherapy.

Actinic granuloma of O'Brien and giant molluscum contagiosum in immunocompetent adults are rare skin disorders. There have been no previous reports of these two conditions occurring together. We report this occurrence in a 57-year-old Caucasian woman.

Summary A 67-year-old man presented with discrete mucinous papules on the scalp and fingers, and bilateral carpal tunnel syndrome. Both features remitted spontaneously within several months. The acral distribution, and histopathological features, were in keeping with the recently described acral persistent papular mucinosis. Involvement of the scalp, however, has not previously been described, and transient mucin deposition is unusual. The history of bilateral carpal tunnel compression raises the possibility of extracutaneous involvement, not previously reported in this group of patients.

Summary A case of haemorrhagic pompholyx occurring in a 29-year-old man with linear IgA disease is described. There were several features in our patient that are usually seen in chronic bullous disease of childhood. Treatment with dapsone cleared the eruption but induced a progressive yet reversible neutropenia.

There is a lack of routinely available information about dermatology outpatient consultations which form the majority of the clinical work of this specialty. This survey was undertaken to prepare for clinical audit and assist in planning future developments of the service. Data were recorded from 2,940 outpatient consultations held during one week by 20 consultant dermatologists and their staff in the West Midlands Health Region in November 1988. The results show that collection and analysis of Regionwide data for a small specialty is feasible, and have indicated future areas of study for clinical audit.

Summary Recurrent episodes of urticaria and erythema multiforme which occur in the luteal phase of each menstrual cycle are well recognized, albeit rare. This clinical entity was described by Hart (1977)1 and attributed to a hypersensitivity to endogenous progesterone. We report a patient with a cyclical urticarial eruption which commenced during pregnancy and recurred pre-menstrually over a period of 11 years. Her symptoms were controlled by suppressing ovulation and finally by bilateral oophorectomy and hysterectomy. Post-operative challenge with an oral oestrogen resulted in a prompt recurrence of the eruption, whereas the administration of a progesterone had no effect. We suggest that this premenstrual eruption was oestrogen-rattier than progesterone-dependent.