Guidelines for the management of pemphigus vulgaris

These guidelines for management of pemphigus vulgaris have been prepared for dermatologists on behalf of the British Association of Dermatologists. They present evidence-based guidance for treatment, with identification of the strength of evidence available at the time of preparation of the guidelines, and a brief overview of epidemiological aspects, diagnosis and investigation.     

Psoriasis bullosa acquisita

We report a 51-year-old man with a 20-year history of chronic plaque psoriasis who developed an autoimmune subepidermal blistering eruption that had clinical features of bullous pemphigoid, erythema multiforme and epidermolysis bullosa acquisita. Investigations revealed a 1 : 400 titre circulating and in vivo bound IgG autoantibody that mapped to the dermal side of 1 m NaCl-split skin and localized to the lower lamina lucida/upper lamina densa on immunogold electron microscopy. Immunoblotting, using dermal extracts, showed serum binding to antigens of approximately 200- and approximately 260 kDa. Indirect immunofluorescence microscopy, using the patient's serum on archival skin sections taken from selected individuals with different forms of inherited epidermolysis bullosa as substrate, showed normal basement membrane labelling on all samples apart from recessive dystrophic epidermolysis bullosa skin (with inherent mutations in the type VII collagen gene): in these cases there was a complete absence of immunostaining. Clinically, the patient responded rapidly to combination treatment with intravenous immunoglobulin and oral corticosteroids, dapsone and mycophenolate mofetil. Autoimmune subepidermal blistering has been reported in other patients with psoriasis, although no specific target antigen has ever been determined. Our study provides preliminary evidence that, for this patient at least, the autoantibody may be targeted against a skin component closely associated with type VII collagen (the epidermolysis bullosa acquisita antigen). Therefore, we propose the term 'psoriasis bullosa acquisita' for this and possibly other patients with similar skin eruptions.     

Diagnosis of pemphigus by ELISA: a critical evaluation of two ELISAs for the detection of antibodies to the major pemphigus antigens, desmoglein 1 and 3

Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are characterized by autoantibodies to the desmosomal glycoproteins desmoglein 3 (Dsg 3) and Dsg 1 (Dsg 1), respectively. In this study, two enzyme-linked immunosorbent assays (ELISA) which detect IgG autoantibodies to Dsg 1 and Dsg 3 have been evaluated. A total of 317 normal and disease controls, 82 patients with PV and 25 with PF were studied. The Dsg 3 ELISA was positive in all 34 patients with untreated PV and the Dsg 1 ELISA was positive in all 10 with untreated PF. When patients undergoing treatment were included, the sensitivities fell to 95% and 92%, respectively, but still compared favourably to the sensitivity of indirect immunofluorescence which was 79% in PV and 84% in PF. All PF sera were negative in the Dsg 3 ELISA and the specificity of both assays was 98% or greater. Large numbers of samples could be analysed simultaneously over a relatively short time period. The Dsg 1 and Dsg 3 ELISAs also provided objective, quantitative, reproducible data which allowed differentiation of PV from PF and in view of these advantages, they are likely to become a routine technique in diagnostic laboratories.     

Temporal case clustering in pityriasis rosea: a regression analysis on 1379 patients in Minnesota, kuwait, and diyarbakir, Turkey

OBJECTIVE: To detect temporal case clustering in patients with pityriasis rosea in different geographic locations. DESIGN: Regression analysis of dates on which 1379 patients were diagnosed as having pityriasis rosea in 3 different geographic locations. The control data consisted of dates of diagnosis of patients with psoriasis in the same settings. SETTING: Dermatology clinics in Kuwait, Minnesota, and Diyarbakir, Turkey.Patients Patients with pityriasis rosea and psoriasis seeking care in the clinics. RESULTS: Three significant positive clusters (P = .005, P =.001, and P =.01, respectively) and 1 significant negative cluster (P<.001) were detected in these series of patients. No cluster was detected in 2 corresponding series of patients with psoriasis in Kuwait and Turkey. CONCLUSION: Temporal case clustering exists in pityriasis rosea.     

Outcome evaluation of active support training in Taiwan

Active Support was implemented for the first time in Taiwan in March, 2009. This study aims to evaluate whether the supervisors and front line managers of residential services receiving Active Support Training (AST) caused a positive impact on their users with intellectual disabilities (ID) while comparing this with their counterparts with ID whose residential staff were not being involved in the training. The nonequivalent groups design was used for the evaluation; the participants included 49 residents of 12 community living homes as the experimental group and 19 residents of another 5 community living homes as the comparative group. The pretest evaluation was conducted before the AST and the post-test and follow-up evaluations were conducted following 4 months and 14 months after the pre-test respectively. The assessment package contained questionnaires relating to domestic engagement, community inclusion, choice, social network, mood scales, challenging behaviors, adaptive behavior and demographic questions among the residents with ID. Within the group, analyses showed that the residents whose staff received AST showed increased levels of choice and adaptive behavior and decreased levels of depression in the post-test and follow up in addition the residents’ engagement in domestic activities improved in the follow up. The intervention did not affect the frequency of family contact, community inclusion and challenging behavior among the residents. The residents in the comparative group showed no significant change except the levels of depression decreased comparing follow-up test and post-test. Based on a cross groups comparison of the effect of the intervention among the residents, only a decreased level of depression was found in the post-test results of the both groups. This study suggests Active Support is practicable but only partially effective in Taiwan; thus, conducting an AST Package of Taiwan version is expectable.     

MSC frequency correlates with blood vessel density in equine adipose tissue

Mesenchymal stem cells (MSCs) are multipotent cells that have the capacity to develop into different mature mesenchymal cell types. They were originally isolated from bone marrow, but MSC-like cells have also been isolated from other tissues. The common feature of all of these tissues is that they all house blood vessels. It is, thus, possible that MSCs are associated with perivascular locations. The objective of this work was to test the hypothesis that MSCs are associated with blood vessels by verifying if MSC frequency positively correlates with blood vessel density. To this end, samples from highly and poorly vascularized adipose tissue sites of two equine donors were collected and processed for histology and cell isolation. MSC frequency in these samples was estimated by means of CFU-F assays, which were performed under MSC conditions. Culture-adherent cells from equine adipose tissue and bone marrow were culture expanded, tested for differentiation into mesenchymal cell types in vitro, and implanted in vivo in porous ceramic vehicles to assess their osteogenic capacity, using human MSCs and brain pericytes as controls. The differentiation assays showed a difference between adipose tissue-derived cells as compared to equine bone marrow MSCs. While differences in CFU-F frequencies between both donors were evident, the CFU-F numbers correlated directly with blood vessel densities (r(2) = 0.86). We consider these preliminary data as further evidence linking MSCs to blood vessels.     

Poststroke restless legs syndrome and lesion location: anatomical considerations

Several case studies have reported on restless legs syndrome (RLS) associated with stroke. In this study, we investigated the prevalence and the lesion topography of poststroke RLS. There were 137 patients with ischemic stroke included in this study. The diagnosis of RLS was made 1 month after the index stroke using the criteria established by the International RLS Study Group. All patients enrolled underwent magnetic resonance imaging within 7 days of the onset of the stroke. The prevalence of stroke-related RLS was calculated, and the topography of the associated ischemic lesions was analyzed. Among 137 patients, 17 patients (12.4%) were diagnosed with RLS after a stroke. Stroke-related RLS was found in 10 out of 33 patients with a basal ganglia/corona radiata infarct (30.3%), 1 out of 8 patients with an internal capsular infarct (12.5%), and 1 out of 7 patients with a thalamic infarct (14.3%). In addition, one out of 54 with a cortical lesion with/without subcortical involvement (1.9%), and 4 out of 18 patients with a pontine lesion (22.2%) had RLS. The analysis of the lesions in the cortical and subcortical group showed only 1 patient in the cortical group had stroke-related RLS, whereas 16 in the subcortical group had stroke-related RLS. The results of this study suggest that lesions of the subcortical brain areas such as the pyramidal tract and the basal ganglia-brainstem axis, which are involved in motor functions and sleep-wake cycles, may lead to RLS symptoms in patients after an ischemic stroke.