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Background and Objectives: Asthma is one of the most common diseases among children in the United States. Increasing provider adherence to national asthma guidelines and connecting patients to Health Homes can increase optimal asthma care. The objectives of this article are to report the results of an asthma learning collaborative and explore the role of Health Homes in contributing to its success., Methods: Quantitative and qualitative data were collected regarding the experiences of 14 pediatric primary care practices and 6 Health Homes participating in a 9-month learning collaborative., Results: Practices exceeded process aims of 80% compliance with optimal asthma care and the use of an Asthma Action Plan among patients aged 2 to 21 years. Health Home care coordinators also reported improvements in self-management strategies for asthma conditions, including the presence of an Asthma Action Plan, medications, spacers, and proper spacing techniques. Providers and Health Home care coordinators identified role clarity, mitigation of environmental triggers, and management of asthma conditions as benefits of the experience., Conclusions: The results of this asthma learning collaborative increased provider adherence to national guidelines and significantly improved optimal asthma care for patients. This multipronged, holistic approach to asthma care proved successful for controlling and maintaining asthma conditions among patients.

Objective: Q-sort methodology is an underutilized tool for differentiating among multiple priority measures. The authors describe steps to identify, delimit, and sort potential health measures and use selected priority measures to establish an overall agenda for continuous quality improvement (CQI) activities within learning collaboratives., Methods: Through an iterative process, the authors vetted a list of potential child and adolescent health measures. Multiple stakeholders, including payers, direct care providers, and organizational representatives sorted and prioritized measures, using Q-methodology., Results: Q-methodology provided the Alabama Child Health Improvement Alliance (ACHIA) an objective and rigorous approach to system improvement. Selected priority measures were used to design learning collaboratives. An open dialogue among stakeholders about state health priorities spurred greater organizational buy-in for ACHIA and increased its credibility as a statewide provider of learning collaboratives., Conclusions: The integrated processes of Q-sort methodology, learning collaboratives, and CQI offer a practical yet innovative way to identify and prioritize state measures for child and adolescent health and establish a learning agenda for targeted quality improvement activities.

Pemphigus vulgaris (PV) is an autoimmune blistering disease affecting the skin and mucous membranes. Rituximab, a CD20 chimeric monoclonal antibody, has efficacy in PV management. We report a case of severe oral PV that showed a progressive response to repeated courses of rituximab, culminating in a rapid response within 4 weeks following severe relapse 4 years after initial therapy. It demonstrates the progressively shorter time to achieve partial or complete remission following rituximab infusions, combined with minimal adjuvant therapy over a 7-year follow-up period., (© 2016 British Association of Dermatologists.)

Background: There is no prognostic index for primary cutaneous T-cell lymphomas such as mycosis fungoides (MF) and Sezary syndrome (SS)., Method: Two prognostic indices were developed for early (IA-IIA) and late stage (IIB-IVB) disease based on multivariate data from 1502 patients. End-points included overall survival (OS) and progression free survival (PFS). External validation included 1221 patients., Findings: Significant adverse prognostic factors at diagnosis consisted of male gender, age >60, plaques, folliculotropic disease and stage N1/Nx for early stage, and male gender, age >60, stages B1/B2, N2/3 and visceral involvement for late stage disease. Using these variables we constructed two separate models each defined using 3 distinct groups for early and late stage patients: 0-1 (low risk), 2 (intermediate risk), and 3-5 factors (high risk). 10 year OS in the early stage model was 90.3% (low), 76.2% (intermediate) and 48.9% (high) and for the late stage model 53.2% (low), 19.8% (intermediate) and 15.0% (high). For the validation set significant differences in OS and PFS in early stage patients (both p<0.001) were also noted. In late stage patients, only OS differed between the groups (p=0.002)., Interpretation: This proposed cutaneous lymphoma prognostic index provides a model for prediction of OS in early and late stage MF/SS enabling rational therapeutic choices and patient stratification in clinical trials., (Crown Copyright © 2013. Published by Elsevier Ltd. All rights reserved.)

Background: Cutaneous disease is thought to account for 10-15% of patient consultations with general practitioners, but relatively little is known about the demography of dermatological conditions in primary care., Aim: To assess the proportion and diagnostic profile of dermatological conditions seen in primary care in the southeast of Scotland, and to draw comparisons with secondary dermatological care., Methods: General practitioners in 13 general practices were asked to note all skin-related consultations during a 2-week period. The case notes of these patients were reviewed, and diagnosis and treatment was recorded. Patients who had consulted for the same skin disorder on >or= 3 occasions during the previous year were invited for assessment by a consultant dermatologist. Where possible, the case notes from 10% of all consultations during the 2-week study period were examined to assess accuracy of recording., Results: The percentage of consultations relating to cutaneous disorders varied between practices, ranging from 3% to 18.8%, with a mean of 8.4%. Eczema accounted for 22.5%, infections 20.3% and benign tumours for 11.4% of consultations with a dermatological basis. In contrast, in secondary care, benign tumours accounted for 23.8%, malignant tumours for 16.4% and eczema for 16.3% of dermatological consultations., Conclusions: Dermatological disorders make up a significant proportion of general practitioners' workload. The diagnostic profile of primary-care dermatology differs markedly from that of hospital practice. General practitioners may benefit from training specifically tailored to the common primary-care dermatological conditions.

Eosinophilic folliculitis (EF) is a descriptive histopathological term applied to a heterogeneous group of disorders. In EF, the characteristic histopathological features are eosinophilic spongiosis and pustulosis involving the infundibular region of the hair follicle. EF may be seen in association with bacterial and fungal infection, drug reactions and haematological disorders. However, in those conditions, the histopathological changes are rarely restricted to the infundibula but in most cases include a moderate to dense perifollicular or even diffuse dermal infiltrate of lymphocytes, or eosinophilic or neutrophilic granulocytes. We present two cases of EF after mini-allogeneic bone-marrow transplantation (BMT) in order to highlight this rare and perhaps under-recognized clinical association.

Granulomatous slack skin disease (GSS) is a very rare form of T-cell lymphoma, with only 52 cases reported in the literature. In the recent World Health Organization-European Organization for Research and Treatment of Cancer consensus classification GSS is considered to be a variant of mycosis fungoides. We describe a patient with GSS and histologic evidence of necrobiosis, which has not been previously reported.

Background: In order to plan appropriate delivery of dermatology services we need periodically to assess the type of work we undertake and to examine changing trends in the numbers and type of referrals and the workload these referrals generate., Objectives: To quantify outpatient workload in hospital-based and private practice; to assess reasons for referral to secondary care and to examine the changes over 25 years in the diagnostic spectrum of conditions referred., Methods: During November 2005, all outpatient dermatological consultations in the south-east of Scotland were recorded. Demographic data, source of and reason for referral, diagnoses, investigations performed, treatment administered and disposal were recorded, and comparisons made with four previous studies., Results: During the 1-month study, attendances were recorded for 2118 new and 2796 review patients (new/review 1 : 1.3, female/male 1.3 : 1, age range 0-106 years). Eighty-nine per cent of new referrals came from primary care and 11% from secondary care. Fifty-seven per cent of referrals were for diagnosis and 38% for management advice. Benign tumours accounted for 33.4%, malignant tumours 11.6%, eczema 16% and psoriasis 7.4% of new cases. For return patients, 20% had skin cancer, 16.5% eczema, 13.4% psoriasis and 9% acne. The referral rate has risen over 25 years from 12.6 per 1000 population in 1980 to 21 per 1000 in 2005, with secondary care referrals increasing from 61 in November 1980 to 230 in November 2005., Conclusions: Attendances for benign and malignant skin tumours have increased sixfold since 1980. Patients with eczema and psoriasis account for one third of clinic visits. New referrals have risen by 67%, with those from other hospital specialties almost quadrupling since 1980 to 11% of the total in 2005. These results confirm the demand from both primary and secondary care for a specialist dermatology service.

Background: Polymorphic light eruption and erythropoietic protoporphyria (EPP) have been demonstrated to have a moderate and large impact on the quality of life (QoL) of patients, respectively. However, there is little information available about the impact of other photodermatoses on QoL., Objectives: To assess and compare the impact of all forms of photodermatoses on patients' QoL using the standard 1-week Dermatology Life Quality Index (DLQI) questionnaire and a modified questionnaire to assess the impact over the previous year., Methods: All patients with photodermatoses seen between 2001 and 2005 at five U.K. photobiology centres were contacted by post on the same day during a forecasted sunny week across the U.K. and asked to complete DLQI questionnaires., Results: A total of 1877 patients were contacted. Seven hundred and ninety-seven (42%) patients replied, with a range from 30% to 48% for the five individual centres. Nearly two-thirds of patients with actinic prurigo (AP) and more than one-third of patients with photoaggravated dermatoses (PAD), chronic actinic dermatitis, EPP and solar urticaria had a DLQI of > 10, confirming a very large effect of the disorders on QoL. Of the cutaneous porphyrias, both variegate porphyria (median DLQI 3) and porphyria cutanea tarda (median DLQI 1.5) had a much lower impact on QoL than EPP., Conclusion: This is the first large-scale study to attempt to measure the impact of a range of photodermatoses on QoL. Photodermatoses have a major impact on QoL. This impact is highest in AP and PAD.

Purpose: To evaluate the Preventive Health Achievable Benchmarks Curriculum, a multifaceted improvement intervention that included an objective, practice-based performance evaluation of internal medicine and pediatric residents' delivery of preventive services., Method: The authors conducted a nonrandomized experiment of intervention versus control group residents with baseline and follow-up of performance audited for 2001-2004. All 130 internal medicine and 78 pediatric residents at two continuity clinics at the University of Alabama School of Medicine, Birmingham, participated. Performance of preventive care was assessed by structured chart review. The multifaceted feedback curriculum included individualized performance feedback, academic detailing by faculty, and collective didactic sessions. The main outcome was difference in receipt of preventive care for patients seen by intervention and control residents, comparing baseline and follow-up., Results: Charts were reviewed for 3,958 patients. Receipt of preventive care increased for patients of intervention residents, but not for patients of control residents. For the intervention group, significant increases occurred for five of six indicators in internal medicine: smoking screening, quit smoking advice, colon cancer screening, pneumonia vaccine, and lipid screening; and four of six in pediatrics: parental quit smoking advice, car seats, car restraints, and eye alignment (p < .05 for all). For control residents, no consistent improvements were seen. There was greater improvement for intervention than for control residents for four of six indicators in internal medicine, and two of six in pediatrics., Conclusions: Using a multifaceted feedback curriculum, the authors taught residents about the care they provide and improved documented patient care.

A 70-year-old man presented with haematuria and restrictive urinary symptoms 7 years after a transurethral prostatectomy (TURP). Onset of symptoms coincided with development of a large skin ulcer on his back. A diagnosis of pyoderma gangrenosum was made based on clinical signs and histopathology. On repeated cystoscopy the urethral sphincter was obliterated by necrotic tissue, suspicious for malignancy. Histopathology revealed only necrotic tissue consistent with infarction of the prostate and sphincter. This clinical presentation with infarction of the prostate and sphincter, concurrent with development of skin ulcers, was consistent with pyoderma gangrenosum affecting the prostatic urothelium and sphincter. Previous prostatic surgery may have predisposed our patient to development of pyoderma gangrenosum in the area of the prostate.

We describe a male patient with Rothmund-Thomson syndrome (RTS) given postoperative radiotherapy for squamous carcinoma of the tongue. This was well tolerated. This is only the second reported case of oral cancer and radiotherapy in RTS.

Oral terbinafine is licensed for use in onychomycosis after positive confirmation of infection. We describe five cases of subacute cutaneous lupus erythematosus associated with terbinafine therapy. All cases had positive antibodies to extractable nuclear antigens, predominantly anti-Ro, and several had a history of pre-existing autoimmune disease. Terbinafine should only be prescribed after confirmation of infection by microscopy or culture.

Classification and subdivision of primary cutaneous diffuse large B-cell lymphoma (PCDLBCL) are a matter of ongoing debate. In this study we assessed the morphologic, immunophenotypic, and clinical features of 30 cases of PCDLBCL identified during a review of all primary cutaneous B-cell lymphomas in the Scotland and Newcastle Lymphoma Group database. We also determined the number of cases harboring t(14;18) using a polymerase chain reaction and primers to the major breakpoint cluster region. The effect on prognosis of a variety of clinical and pathologic factors was assessed for the group of 30 PCDLBCL and the 5-year disease-specific survival (DSS) of this cohort compared with that of 195 cases of stage I diffuse large B-cell lymphoma arising primarily in lymph nodes, also identified from within the Scotland and Newcastle Lymphoma Group database. Location on the leg was the only independent prognostic factor for determining outcome in PCDLBCL (67% 5-year DSS compared with 100% for the upper body; P = 0.0047). The presence of multiple lesions, involvement of more than one body site, and expression or not of CD10, bcl-2, bcl-6, and CD10 and bcl-6, had no effect on survival. Compared with cases arising above the waist, those on the leg were more often female, were of an older age, and had a significantly higher incidence of bcl-2 expression (P = 0.002) as well as the aforementioned poorer prognosis. They also showed more frequent co-expression of CD10 and bcl-6, supporting a follicle center cell origin for some, but this difference was not statistically significant. Although there was no significant difference in the 5-year DSS between the group of PCDLBCL and the cases of stage I nodal diffuse large B-cell lymphoma (88% 5-year DSS vs. 78%; P = 0.06), the latter were generally treated with more aggressive therapy. Moreover, a significant difference in 5-year DSS was seen when the nodal DLBCLs were compared with PCDLBCLs arising above the waist (78% vs. 100% respectively; P = 0.0135). These results support the current EORTC approach of subdividing PCLBCL on the basis of site to produce prognostically relevant groupings.

Primary cutaneous B-cell lymphomas displaying a prominent follicular growth pattern are rare and remain poorly defined, particularly in terms of the frequency of detection of t(14;18) and whether or not, as a group, they represent an entity distinct from follicular lymphoma arising in lymph nodes. The morphologic, immunophenotypic, and clinical features of 16 cases of primary cutaneous follicular lymphoma, identified during a review of all PCBCL in the Scotland and Newcastle Lymphoma Group database, were studied and the number of cases harboring t(14;18) assessed by polymerase chain reaction using primers to the major breakpoint cluster region. Comparisons were made with stage I follicular lymphoma arising in lymph nodes and follicular lymphoma secondarily involving the skin. All cases of primary cutaneous follicular lymphoma had undergone thorough staging, including physical examination and CT scans of chest and abdomen, with 15 of 16 cases also having bone marrow aspiration and/or trephine performed. The morphology and immunophenotype of the lesions were similar to that expected in lymph nodes. All cases displayed a follicular architecture complete with follicular dendritic cell networks and comprised an admixture of CD10 and/or bcl-6-positive neoplastic centrocytes and centroblasts with 13 of 16 cases also expressing bcl-2 protein. None harbored t(14;18), a significantly different finding compared with cases of stage I nodal follicular lymphoma (p <0.001) and secondary cutaneous follicular lymphoma (p <0.039). Relapses occurred in five of 15 patients with a median time to first relapse of 20 months (range 1-73 months; mean 27.2 months). These were multiple in two patients and involved extracutaneous sites in two patients. The propensity for relapse was similar to that in a comparative cohort of stage I nodal follicular lymphoma, but the group of primary cutaneous follicular lymphoma were significantly more likely to attain complete remission; all cases of primary cutaneous follicular lymphoma were in complete remission when last seen compared with 49 of 87 patients with stage I nodal follicular lymphoma (p <0.005). No lymphoma-related deaths were encountered in 15 cases with a mean follow-up >60 months (range 5-119 months). These results support the concept of a subtype of follicular lymphoma lacking t(14;18) involving the major breakpoint cluster region, and with a propensity to arise in the skin. Despite a high relapse rate patients with primary cutaneous follicular lymphoma are more likely to achieve complete remission and may ultimately have a more favorable long-term prognosis than those with equivalent nodal disease.

In this study we have determined the ability of IGF-1 to protect cardiac fibroblasts against osmotic-induced apoptosis and investigated the potential mechanism(s) underlying this protection. Treatment with IGF-1 (1-100 ng/ml) promoted a dose dependent increase in cell survival against osmotic cell death. Both Akt and ERK1/2 were rapidly phosphorylated by IGF-1 and blocked by wortmannin and PD98059, inhibitors of their upstream activators respectively. However, IGF-1-induced protection was mediated via a wortmannin-dependent but PD98059-independent pathway as determined by cell survival assay suggesting a role of PI3-K/Akt. Furthermore, IGF-1 appeared to reduce the activation of a number of early components in the apoptotic pathway in a wortmannin dependent manner including the osmotic stress-induced perturbation in mitochondrial membrane potential, cleavage and activation of caspase-3 and DNA fragmentation. Thus, the results suggest that IGF-1 regulates osmotic stress-induced apoptosis via the activation of the PI3-K/Akt pathway at a point upstream of the mitochondria and caspase-3., (Copyright 2000 Academic Press.)

Most people will experience infection with human papilloma virus (HPV) at some point in their life. Diagnosis, based on clinical examination, is usually straight forward. Treatment can, however, be challenging. Indications for treatment include pain, interference with function, cosmetic embarrassment, and risk of malignancy. Clearance rates are highest in young, healthy individuals with short duration of infection. Treatment may be with destructive agents (keratolytics, cryotherapy, curettage and cautery, laser, photodynamic therapy), with antimitotic agents (podophyllin, bleomycin, retinoids), with immune stimulants (topical sensitizers, cimetidine), or with topical virucidal agents [formaldehyde (formalin), glutaral (gluteraldehyde)]. As yet, there is no single totally effective treatment for viral warts. Some patients may choose to leave their warts untreated until spontaneous resolution. In those who seek intervention, simple, well tolerated therapies should be chosen initially in preference to more complicated, potentially harmful agents. It is likely that future research will be directed to developing an antiviral agent specific for HPV which would be safe, effective and not prohibitively expensive.

Linear scleroderma 'en coup de sabre' is characterized by atrophy and furrowing of the skin of the frontoparietal region. In most cases it occurs as a single, paramedian line that may be associated with hypoplasia of underlying structures and hemiatrophy of the face. Rarely, lesions form two lines, either both on the same side or bilaterally. We describe an 18-year-old male with an unusual tri-linear pattern of scleroderma 'en coup de sabre' following Blaschko's lines.

Keratosis follicularis spinulosa decalvans (KFSD) is a rare, X linked disorder with skin and eye involvement (MIM 308800). We have studied a large British family with KFSD using polymorphic markers from Xp21-p23 and obtained a lod score of 2.056 at theta=0 with markers proximal and distal to the KFSD candidate region Xp22.13-p22.2 identified by Oosterwijk et al. Our data confirm the linkage to Xp22.13-p22.2 observed in the previously reported Dutch family, but fail to narrow the candidate interval for the KFSD locus.

The role of human papillomavirus (HPV) in the aetiology of in situ and invasive carcinoma of the genital tract is well established. In the rare disorder epidermodysplasia verruciformis (EV), in which patients develop extensive warts of unusual types and multiple cutaneous squamous cancers on light-exposed skin, current evidence suggests a probable role for a specific group of EV HPVs in the carcinogenic process. Determination of the possible role of HPV in the aetiology of non-melanoma skin cancers (NMSCs), which occur frequently in immunosuppressed organ allograft recipients, has been limited, until recently, by the lack of availability of a sensitive detection system for a wide range of cutaneous HPV types. We have used a combination of 2 sets of PCR primers to examine 68 benign and malignant tumours collected over a 12-year period from 25 renal allograft recipients. Cloning and sequencing of the PCR products were carried out to distinguish HPV DNA from cellular sequences. A combination of these techniques revealed HPV DNA in all viral warts, 65% of keratoses, 91% of intra-epidermal cancers and 91% of invasive squamous cancers. Both cutaneous and EV HPV types were detected, including 18 novel types. In 4 patients with multiple cancers, the most prevalent types were in the EV group: HPV 20, 23, 38 and 2 novel types, DL40 and DL267 (related to HPV 10 and 38, respectively). These 5 HPV types were present in a total of 73% of all malignant lesions tested. The technique described represents a reliable method of HPV DNA detection in NMSC. The EV group of HPVs predominate in the cancers, but the multiplicity of HPV types detected with double infection in some lesions suggests virus/virus in addition to virus/host interaction in the carcinogenic process.

The persistence of human papillomavirus at cutaneous sites may be due to impaired trafficking of immune effector cells to the epidermis. We investigated whether HPV infection modulates cytokine mRNA expression in skin, thereby influencing local immunity. The mRNA expression of tumour necrosis factor-alpha, interleukin-1 alpha (IL-1 alpha), IL-1 beta, IL-1 receptor antagonist (IL-1ra), IL-4, IL-8, IL-10, IL-12, granulocyte macrophage colony-stimulating factor, transforming growth factor-beta, interferon-gamma and amphiregulin were assayed in cutaneous warts and normal skin by semiquantitative reverse transcriptase-polymerase chain reaction. The expression of the cytokines was heterogeneous in the specimens but, of the 12 mRNA species investigated, only IL-10 mRNA was significantly downregulated in warts compared with normal skin (P = 0.002). IL-1 alpha mRNA expression was significantly upregulated in common warts (P = 0.019) and plantar warts (P = 0.003) compared with normal skin. The expression of IL-1 alpha and IL-1ra mRNAs were significantly correlated in plantar warts (P < 0.05). Warts expressing IL-1 alpha also expressed amphiregulin, and there was a significant correlation between the expression of these two genes (P < 0.05). It is possible that IL-1 alpha expression in cutaneous warts may modulate the growth of papillomavirus-infected keratinocytes, mediated by amphiregulin, thus ensuring viral persistence.

We report two cases of the trigeminal trophic syndrome, complicating peripheral and central interruption of the trigeminal sensory pathways. These cases illustrate the diagnostic and management problems that this syndrome presents.

Keratosis follicularis spinulosa decalvans is a rare, X-linked genodermatosis characterized by follicular hyperkeratosis, scarring alopecia of the scalp, eyebrows and eyelashes, corneal dystrophy and photophobia. We describe two cases from a large family, the first with keratosis follicularis spinulosa decalvans to be reported in the U.K.

Milia en plaque is an unusual eruption typically occurring in the retroauricular area. Two cases of this disorder occurring in a novel position and treated with oral minocycline are now reported.

In vitro lymphoproliferative responses of peripheral blood mononuclear cells (PBMC) from patients with cutaneous warts, caused by infection with human papillomavirus type 1 (HPV-1) or type 2 (HPV-2), were assayed during the course of treatment. Purified HPV-1 and HPV-2 were used as antigens, as well as herpes simplex virus (HSV) and concanavalin A (Con A). All patients had normal percentages of subsets within the PBMC population and normal lymphoproliferative responses to Con A, and those with a clinical history of HSV infections had positive lymphoproliferative responses to HSV. Responses to both HPV antigens were poor. Only 10 of 100 assays of PBMC from 26 patients showed a stimulation index greater than 2. Addition of interleukin 2 made little difference in most cases. No correlation of clinical status of warts, i.e. improving, unchanged or resolved, with lymphoproliferation was found. When the PBMC were depleted of plastic-adherent cells and enriched for T cells, some samples which had not shown a lymphoproliferative response to HPV-1 or HPV-2 became positive; this response was abolished when the adherent cells were re-added. Thus it is possible that the adherent cell population from a proportion of patients contains cells which suppress lymphoproliferation, or that an immunoregulatory network is present so that lymphoproliferation does not take place in vitro without prior activation and cloning of T cells.

A study of out-patient dermatological services (NHS and private) in the south-east of Scotland was carried out by medical staff in the Department of Dermatology in Edinburgh during the month of November 1988. The aim was to assess changes in referral patterns and workload compared with the findings of an identical investigation undertaken in November 1981. Of particular interest were the possible effects of recent publicity campaigns aimed at increasing public awareness about skin cancer. The medical complement of the dermatology department had changed minimally since 1981 and the population increase in the south-east of Scotland over the same period was 1.5%. During November 1988 1592 new patients and 2037 review patients were seen. This represented an increase of 29.2% and 28.3%, respectively, since 1981. The most striking changes in diagnostic groups were a 173% rise in new cases presenting with benign tumours (excluding viral warts) and a 106% increase in new patients with malignant tumours. Viral warts and eczema were, as in 1981, the second and third most common diagnostic categories amongst new patients. There was a 98% increase in the number of surgical procedures performed on new patients compared with 1981. We conclude that the substantial increase in numbers of both benign and malignant tumours and the consequent doubling in surgical treatments was due to increased public awareness and concern about skin cancer.