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Vitiligo is a common disease that causes a great degree of psychological distress. In its classical forms it is easily recognised and diagnosed. This review provides an evidence based outline of the management of vitiligo, particularly with the non-specialist in mind. Treatments for vitiligo are generally unsatisfactory. The initial approach to a patient who is thought to have vitiligo is to make a definite diagnosis, offer psychological support, and suggest supportive treatments such as the use of camouflage cosmetics and sunscreens, or in some cases after discussion the option of no treatment. Active therapies open to the non-specialist, after an explanation of potential side effects, include the topical use of potent or highly potent steroids or calcineurin inhibitors for a defined period of time (usually 2 months), following which an assessment is made to establish whether or not there has been a response. Patients whose condition is difficult to diagnose, unresponsive to straightforward treatments, or is causing psychological distress, are usually referred to a dermatologist. Specialist dermatology units have at their disposal phototherapy, either narrow band ultraviolet B or in some cases photochemotherapy, which is the most effective treatment presently available and can be considered for symmetrical types of vitiligo. Depigmenting treatments and possibly surgical approaches may be appropriate for vitiligo in selected cases. There is no evidence that presently available systemic treatments are helpful and safe in vitiligo. There is a need for further research into the causes of vitiligo, and into discovering better treatments. [ABSTRACT FROM AUTHOR]

Critically appraised topics (CATs) are essential tools for busy clinicians who wish to ensure that their daily clinical practice is underpinned by evidence-based medicine. CATs are short summaries of the most up-to-date, high-quality available evidence that is found using thorough structured methods. They can be used to answer specific, patient-orientated questions that arise recurrently in real-life practice. This article provides readers with a detailed guide to performing their own CATs. It is split into four main sections reflecting the four main steps involved in performing a CAT: formulation of a focused question, a search for the most relevant and highest-quality evidence, critical appraisal of the evidence and application of the results back to the patient scenario. As well as helping to improve patient care on an individual basis by answering specific clinical questions that arise, CATs can help spread and share knowledge with colleagues on an international level through publication in the evidence-based dermatology section of the British Journal of Dermatology., (© 2017 British Association of Dermatologists.)

Background: Published methodology used to determine psoralen plus ultraviolet A (PUVA) erythemal action spectrum does not reflect current clinical practice for psoralen sensitization. We re-evaluated the PUVA action spectrum using aqueous 8-methoxypsoralen (8-MOP) 2·6 mg L(-1) as used routinely in current clinical practice., Objectives: To determine the UVA erythema action spectrum of topical 8-MOP-sensitized normal skin., Methods: Twenty healthy volunteers with skin phototypes I-V were recruited. Forearms were psoralen-sensitized at 37 °C for 10 min. Six UVA irradiations at 10-nm intervals between 325 and 375 nm were randomly allocated to forearm sites and were applied using a 10-nm bandwidth irradiation monochromator. The visual minimal phototoxic dose (MPD) was recorded on each site at 96 h., Results: Volunteer Boston phototypes were: I, n = 2; II, n = 6; III, n = 6; IV, n = 5 and V, n = 1. The mean MPD (J cm(-2) ) for all subjects at each wavelength was as follows: 325 nm, 0·64 (SD 0·37); 335 nm, 0·80 (SD 0·58); 345 nm, 0·96 (SD 0·55); 355 nm, 1·50 (SD 0·85); 365 nm, 2·19 (SD 0·90); and 375 nm, 2·89 (SD 1·06). Therefore, the relative sensitization at each wavelength (erythemal action spectrum) was: 1, 0·83, 0·67, 0·43, 0·29 and 0·22. There were significant differences between the PUVA erythemal effectiveness at different wavelengths but none between skin types., Conclusions: This study has established the erythemal action spectrum for bath/soak PUVA therapy as is currently performed. In all volunteers, the peak sensitivity was at 325 nm. All volunteers showed a similar trend across the wavelengths studied irrespective of skin type. The determination of the action spectrum for PUVA-induced erythema is important as it permits reliable estimates of erythemal efficacy of any UVA source where the emission spectrum of the lamp is known or can be measured., (© 2015 British Association of Dermatologists.)

A 2006 survey of dermatologists showed that home phototherapy should be used with caution, and that general, nonevidence-based opinions were widespread about this form of therapy. This led to a randomized controlled trial to assess the safety and efficacy of home phototherapy vs. outpatient phototherapy by the same authors in 2009, which concluded that a lower burden of treatment and increased patient satisfaction were associated with home phototherapy. In the UK National Health Service, with a single exception, phototherapy is currently carried out exclusively in hospital. This contrasts with the Netherlands, where home phototherapy is now widely available. NHS dermatology services in the UK have yet to adopt home phototherapy as a treatment option, despite the strong evidence base and robust service models established elsewhere. In this review, we discuss evidence-based papers on home phototherapy, its advantages and disadvantages, economic effectiveness, and a suggested model for service sustainability., (© 2015 British Association of Dermatologists.)

Background: Erythropoietic protoporphyria is a severe photodermatosis that is associated with acute phototoxicity. Patients with this condition have excruciating pain and a markedly reduced quality of life. We evaluated the safety and efficacy of an α-melanocyte-stimulating hormone analogue, afamelanotide, to decrease pain and improve quality of life., Methods: We conducted two multicenter, randomized, double-blind, placebo-controlled trials of subcutaneous implants containing 16 mg of afamelanotide. Patients in the European Union (74 patients) and the United States (94 patients) were randomly assigned, in a 1:1 ratio, to receive a subcutaneous implant containing either afamelanotide or placebo every 60 days (a total of five implants in the European Union study and three in the U.S study). The type and duration of sun exposure, number and severity of phototoxic reactions, and adverse events were recorded over the respective 180-day and 270-day study periods. Quality of life was assessed with the use of validated questionnaires. A subgroup of U.S. patients underwent photoprovocation testing. The primary efficacy end point was the number of hours of direct exposure to sunlight without pain., Results: In the U.S. study, the duration of pain-free time after 6 months was longer in the afamelanotide group (median, 69.4 hours, vs. 40.8 hours in the placebo group; P=0.04). In the European Union study, the duration of pain-free time after 9 months was also longer in the afamelanotide group than in the placebo group (median, 6.0 hours vs. 0.8 hours; P=0.005), and the number of phototoxic reactions was lower in the the afamelanotide group (77 vs. 146, P=0.04). In both trials, quality of life improved with afamelanotide therapy. Adverse events were mostly mild; serious adverse events were not thought to be related to the study drug., Conclusions: Afamelanotide had an acceptable side-effect and adverse-event profile and was associated with an increased duration of sun exposure without pain and improved quality of life in patients with erythropoietic protoporphyria. (Funded by Clinuvel Pharmaceuticals and others; ClinicalTrials.gov numbers, NCT01605136 and NCT00979745.).

Targeted ultraviolet B (UVB) phototherapy is defined as UVB radiation applied only to clinically diseased skin, with sparing of adjacent normal skin, unlike conventional phototherapy, which involves irradiation of both diseased and normal skin. Targeted UVB radiation is a relatively new concept, which is now widely available because of advances in technology. Devices developed for targeted UVB phototherapy of the skin include the monochromatic excimer laser and lamp, both of which are now used by dermatologists in developed and developing countries. The aim of this review is to collate data from research studies on targeted phototherapy and to provide a concise description of currently available devices, their clinical indications and therapeutic efficacy. Additionally, potential adverse effects are summarized, and the limitations of these novel devices are highlighted., (© 2014 British Association of Dermatologists.)

The porphyrias are a group of mainly inherited disorders of heme biosynthesis where accumulation of porphyrins and/or porphyrin precursors gives rise to 2 types of clinical presentation: cutaneous photosensitivity and/or acute neurovisceral attacks. The cutaneous porphyrias present with either bullous skin fragility or nonbullous acute photosensitivity. This review discusses the epidemiology, pathogenesis, clinical presentation, laboratory diagnosis, complications, and current approach to porphyria management. Although focusing mainly on their dermatological aspects, the article also covers the management of acute porphyria, which by virtue of its association with variegate porphyria and hereditary coproporphyria, may become the responsibility of the clinical dermatologist., (Copyright © 2014 Elsevier Inc. All rights reserved.)

X-linked dominant protoporphyria (XLDPP) was first reported in the genetics literature in 2008. It has a phenotype very similar to erythropoietic protoporphyria (EPP), but is distinguished from EPP by higher concentrations of erythrocyte protoporphyrin (of which a high proportion is zinc-chelated), its apparently higher incidence of liver disease, and an X-linked dominant pattern of inheritance. Dermatologists should understand how XLDPP differs from EPP, in order to advise newly diagnosed patients correctly about the genetic implications and the long-term management strategy. We present a case series of XLDPP to introduce this condition to the dermatology literature., (© 2013 British Association of Dermatologists.)

Molluscum contagiosum virus is an important human skin pathogen: it can cause disfigurement and suffering in children, in adults it is less common and often sexually transmitted. Extensive and persistent skin infection with the virus can indicate underlying immunodeficiency. Traditional ablative therapies have not been compared directly with newer immune-modulating and specific antiviral therapies. Advances in research raise the prospect of new approaches to treatment informed by the biology of the virus; in human skin, the infection is localised in the epidermal layers, where it induces a typical, complex hyperproliferative lesion with an abundance of virus particles but a conspicuous absence of immune effectors. Functional studies of the viral genome have revealed effects on cellular pathways involved in the cell cycle, innate immunity, inflammation, and cell death. Extensive lesions caused by molluscum contagiosum can occur in patients with DOCK8 deficiency-a genetic disorder affecting migration of dendritic and specialised T cells in skin. Sudden disappearance of lesions is the consequence of a vigorous immune response in healthy people. Further study of the unique features of infection with molluscum contagiosum virus could give fundamental insight into the nature of skin immunity., (Copyright © 2013 Elsevier Ltd. All rights reserved.)

Background: The traditional method of assessing minimal phototoxic dose (MPD) prior to photochemotherapy with psoralen-ultraviolet A (PUVA) is inconvenient and cannot directly determine PUVA start doses. A handheld minimal erythema dose UVB tester can be modified by fitting a TL-10 UVA compact fluorescence lamp (CFL)., Objectives: To determine whether MPD testing is possible with a CFL and to calculate a fixed factor to convert observed MPD to PUVA-equivalent MPD., Methods: Patients had two sets of MPD tests performed on symmetrical, contralateral sites on the lower back. MPD test results from a panel of PUVA lamps were compared with MPD from the modified handheld tester. Additionally, a questionnaire survey was completed by 43 U.K. phototherapy units to assess routine practice concerning MPD testing prior to PUVA therapy., Results: Thirty-seven patients with psoriasis were recruited. Boston phototypes in the 31 with conclusive MPD reactions were: I, four; II, 11; III, 12; and IV, four. The handheld MPD results were linearly related to the PUVA panel MPD results as follows: PUVA MPD = 0·48 × handheld MPD + 0·17 J cm(-2). The measured PUVA MPD was 0·48 of the handheld MPD, not 0·15 as predicted by the published PUVA action spectrum., Conclusions: The modified MPD tester is a convenient and safe method for PUVA MPD testing, overcoming many problems of the 'traditional method'. The difference between the PUVA and TL-10 lamps was lower than predicted from published studies. This suggests that formal re-evaluation of the erythema action spectrum for PUVA is now needed., (© 2013 British Association of Dermatologists.)

Photosensitivity to UVB is a prominent feature of a small number of congenital skin disorders. In addition UVB may also contribute to the action spectrum of a number of acquired photosensitivity syndromes. The gene mutations underlying the genetically inherited disorders have largely been identified and have provided insights into DNA repair pathways. The pathomechanisms for the acquired disorders are still largely undefined. Few therapeutic options are available so management of all these disorders still relies on rigorous photo-protection.

Background: Congenital erythropoietic porphyria (CEP) is an autosomal recessive photomutilating porphyria with onset usually in childhood, where haematological complications determine prognosis. Due to its extreme rarity and clinical heterogeneity, management decisions in CEP are often difficult., Objectives: To develop a management algorithm for patients with CEP based on data from carefully characterized historical cases., Methods: A single investigator collated data related to treatments and their outcomes in 29 patients with CEP from the U.K., France, Germany and Switzerland., Results: Six children were treated with bone marrow transplantation (BMT); five have remained symptomatically cured up to 11.5 years post-transplantation. Treatments such as oral charcoal, splenectomy and chronic hypertransfusion were either of no benefit or were associated with complications and negative impact on health-related quality of life. Lack of consistent genotype-phenotype correlation meant that this could not be used to predict disease prognosis. The main poor prognostic factors were early age of disease onset and severity of haematological manifestations., Conclusions: A management algorithm is proposed where every patient, irrespective of disease severity at presentation, should receive a comprehensive, multidisciplinary clinical assessment and should then be reviewed at intervals based on their predicted prognosis, and the rate of onset of complications. A BMT should be considered in those with progressive, symptomatic haemolytic anaemia and/or thrombocytopenia. Uroporphyrinogen III synthase genotypes associated with poor prognosis would additionally justify consideration for a BMT. Rigorous photoprotection of the skin and eyes from visible light is essential in all patients., (© 2012 The Authors. BJD © 2012 British Association of Dermatologists.)

Background: Congenital erythropoietic porphyria (CEP) is an autosomal recessive cutaneous porphyria caused by decreased activity of uroporphyrinogen III synthase (UROS). Its predominant characteristics include bullous cutaneous photosensitivity to visible light from early infancy, progressive photomutilation and chronic haemolytic anaemia. Due to its rarity and genetic heterogeneity, clinical phenotypes are unclear and its impact on health-related quality of life (HRQoL) has not been previously assessed., Objectives: To define comprehensively CEP phenotypes and assess their impact on HRQoL, and to correlate these factors with laboratory parameters., Methods: A single observer assessed patients with CEP from four European countries., Results: Twenty-seven unrelated patients with CEP, aged between 7.6 and 65 years, participated in the study. The patients came from the U.K. (17), France (4), Switzerland (4) and Germany (2). Additional data were obtained for two deceased patients. Newly characterized features of CEP include acute-onset cutaneous and noncutaneous symptoms immediately following sunlight exposure, and pink erythematous facial papules. There was a lack of consistent genotype-phenotype correlation in CEP. The main poor prognostic factors in CEP are the early age of disease onset and haematological complications., Conclusions: CEP is a multisystem disease; cutaneous, ocular, oral and skeletal manifestations also contribute to disease severity and impact on HRQoL, in addition to the haematological complications. The rarity of the disease can lead to delayed diagnosis. The lack of consistent genotype-phenotype correlation in CEP suggests a contribution to phenotype from other factors, such as environment, patients' photoprotective behaviour and genes other than UROS. There is currently an unmet need for multidisciplinary management of patients with CEP., (© 2012 The Authors. BJD © 2012 British Association of Dermatologists.)

Microneedle delivery of nucleic acids, in particular plasmid DNA (pDNA), to the skin represents a potential new approach for the clinical management of genetic skin diseases and cutaneous cancers, and for intracutaneous genetic immunisation. In this study excised human skin explants were used to investigate and optimise key parameters that will determine stable and effective microneedle-facilitated pDNA delivery. These include (i) high dose-loading of pDNA onto microneedle surfaces, (ii) stability and functionality of the coated pDNA, (iii) skin penetration capability of pDNA-coated microneedles, and (iv) efficient gene expression in human skin. Optimisation of a dip-coating method enabled significant increases in the loading capacity, up to 100μg of pDNA per 5-microneedle array. Coated microneedles were able to reproducibly perforate human skin at low (<1N) insertion forces. The physical stability of the coated pDNA was partially compromised on storage, although this was improved through the addition of saccharide excipients without detriment to the biological functionality of pDNA. The pDNA-coated microneedles facilitated reporter gene expression in viable human skin. The efficiency of gene expression from coated microneedles will depend upon suitable DNA loading, efficient and reproducible skin puncture and rapid in situ dissolution of the plasmid at the site of delivery., (Copyright © 2012 Elsevier B.V. All rights reserved.)

Background: There are limited data on the use of ustekinumab outside of clinical trials., Objectives: To assess the efficacy and safety of ustekinumab in patients with severe psoriasis attending 10 dermatology centres in the U.K. and Ireland., Methods: A retrospective case-note review of 129 patients with psoriasis treated with ustekinumab., Results: Baseline Psoriasis Area and Severity Index (PASI) was 22·9±10·1 (mean±SD). After 16weeks of treatment with ustekinumab PASI 75 (75% reduction in PASI) was observed in 63·0% (n=80/127) of patients, although four patients required concomitant therapy at the 16-week time point. Previous biologic use did show a small, non-significant trend towards treatment failure. A PASI 75 response was seen in 29·4% (n=5/17) of individuals weighing 90-100kg and treated with the standard 45mg ustekinumab dose compared with PASI 75 of 70·3%, 71·4%, 75·0% and 55·6% for weight groups <80, 80-90, 100-110 and >110kg, respectively (P=0·024). Ustekinumab therapy was well tolerated; serious adverse events were observed in 2·3% (n=3/129) of patients., Conclusions: Ustekinumab is a novel biologic agent for psoriasis. When used in everyday clinical practice it demonstrates high levels of short-term therapeutic efficacy with an acceptable short-term safety profile., (© 2011 The Authors. BJD © 2011 British Association of Dermatologists.)

A number of skin conditions are characterised by photosensitivity to UVA. Some of these are exclusively UVA-mediated conditions, while others include UVA in the action spectrum which also include UVB and/or visible light. This review aims to describe this diverse range of conditions for non-dermatologist scientists with an interest in this topic. As such, clinical details, including treatments, are brief and succinct. Recent advances in understanding the pathogenesis of these conditions is highlighted., (This journal is © The Royal Society of Chemistry and Owner Societies 2012)

Rosacea fulminans (RF) is a rare facial dermatosis characterized by the sudden onset of severe facial inflammation consisting of numerous pustules, cystic swellings and coalescing sinuses. The standard treatment is the retinoid drug isotretinoin in combination with systemic corticosteroids or with high-dose oral tetracycline antibiotics. We report three recent cases of RF in pregnancy with differing obstetric outcomes: an intrauterine death, a termination of pregnancy, and a normal vaginal delivery. The pathogenesis of RF is considered and therapeutic options in pregnancy are reviewed.

There is a significant gap in our fundamental understanding of early morphological and migratory changes in human Langerhans cells (LCs) in response to vaccine stimulation. As the vast majority of LCs studies are conducted in small animal models, substantial interspecies variation in skin architecture and immunity must be considered when extrapolating the results to humans. This study aims to determine whether excised human skin, maintained viable in organ culture, provides a useful human model for measuring and understanding early immune response to intradermally delivered vaccine candidates. Excised human breast skin was maintained viable in air-liquid-interface organ culture. This model was used for the first time to show morphological changes in human LCs stimulated with influenza virus-like particle (VLP) vaccines delivered via intradermal injection. Immunohistochemistry of epidermal sheets and skin sections showed that LCs in VLP treated skin lost their typical dendritic morphology. The cells were more dispersed throughout the epidermis, often in close proximity to the basement membrane, and appeared vertically elongated. Our data provides for increased understanding of the complex morphological, spatial and temporal changes that occur to permit LC migration through the densely packed keratinocytes of the epidermis following exposure to vaccine. Significantly, the data not only supports previous animal data but also provides new and essential evidence of host response to this vaccination strategy in the real human skin environment.

Background/purpose: We tested eye protection used for phototherapy patients. The study also established current practice concerning eye protection in a sample of UK phototherapy units., Methods: The ultraviolet (UV) transmission spectra of 30 'UV protective' contact lenses were measured at 5 nm intervals between 290 and 400 nm. Sunglasses, small UV goggles and UV visors were tested between 270 and 420 nm. We surveyed the use of eye protection during phototherapy in 78 UK phototherapy units., Results: All samples of sunglasses, eye protection goggles, visors and sunglasses comfortably passed previously published arbitrary limits of acceptability. Most contact lenses showed some protection in the UVB, but most had little or no UVA protection. Of 78 UK phototherapy units 21 (33%) use tinted goggles during UV exposures, two (3%) use a visor only, 28 (43%) use both and nine (14%) use clear plastic (probably polycarbonate) goggles., Conclusions: UV transmission for sunglasses and contact lenses is lower compared with samples tested 10 years ago. All samples of glasses, goggles and visors tested provided adequate protection in the UV range according to published arbitrary limits of acceptability. Most contact lenses did not provide significant UV protection in the UVA range.

Background: Erythropoietic protoporphyria (EPP) is a cutaneous porphyria caused by mutations in the ferrochelatase (FECH) or, less frequently, the delta-aminolaevulinate synthase 2 (ALAS2) gene. Predictive genetic counselling requires accurate molecular diagnosis and knowledge of patterns of inheritance., Objectives: To investigate the molecular epidemiology of EPP in the U.K., Methods: DNA samples from 191 unrelated patients resident in the U.K. were analysed for mutations in the FECH and ALAS2 genes and for the FECH IVS3-48 dimorphism., Results: Mutations were identified in 179 (94%) patients. Most (169; 94%) had a FECH mutation on one allele and were classified as having pseudodominant EPP (psdEPP); seven (4%) patients had FECH mutations on both alleles (autosomal recessive EPP) and three (2%) patients had ALAS2 mutations (X-linked dominant protoporphyria). The FECH IVS3-48C allele was strongly associated with psdEPP and with the absence of mutations at the FECH or ALAS2 loci. Fifty-six FECH mutations were identified, 19 being previously unreported. Missense mutations were predominant in autosomal recessive EPP (82%) but not in psdEPP (32%). One mutation (c.314 + 2T>G) was present in 41 (24%) of EPP families, most of whom appeared to be descended from a common ancestor resident in the north of England., Conclusions: These data define the prevalence and molecular epidemiology of each type of EPP in the U.K.

Erythropoietic protoporphyria (EPP) is an inherited disorder that results from partial deficiency of ferrochelatase (FECH). It is characterized clinically by acute photosensitivity and, in 2% of patients, liver disease. Inheritance is usually autosomal dominant with low penetrance but is recessive in about 4% of families. A cross-sectional study of 223 patients with EPP in the United Kingdom identified six individuals with palmar keratoderma. We now show that these and three additional patients, from six families, have an inherited subtype of EPP which is characterized by seasonal palmar keratoderma, relatively low erythrocyte protoporphyrin concentrations, and recessive inheritance. No patient had evidence of liver dysfunction; four patients had neurological abnormalities. Patients were hetero- or homoallelic for nine different FECH mutations; four of which were previously unreported. Prokaryotic expression predicted that FECH activities were 2.7-25% (mean 10.6%) of normal. Neither mutation type nor FECH activity provided an explanation for the unusual phenotype. Our findings show that palmar keratoderma is a clinical indicator of recessive EPP, identify a phenotype that occurs in 38% of reported families with recessive EPP that to our knowledge is previously unreported, and suggest that patients with this phenotype may carry a lower risk of liver disease than other patients with recessive EPP.

This detailed and user-friendly guideline for the diagnosis and management of vitiligo in children and adults aims to give high quality clinical advice, based on the best available evidence and expert consensus, taking into account patient choice and clinical expertise. The guideline was devised by a structured process and is intended for use by dermatologists and as a resource for interested parties including patients. Recommendations and levels of evidence have been graded according to the method developed by the Scottish Inter-Collegiate Guidelines Network. Where evidence was lacking, research recommendations were made. The types of vitiligo, process of diagnosis in primary and secondary care, and investigation of vitiligo were assessed. Treatments considered include offering no treatment other than camouflage cosmetics and sunscreens, the use of topical potent or highly potent corticosteroids, of vitamin D analogues, and of topical calcineurin inhibitors, and depigmentation with p-(benzyloxy)phenol. The use of systemic treatment, e.g. corticosteroids, ciclosporin and other immunosuppressive agents was analyzed. Phototherapy was considered, including narrowband ultraviolet B (UVB), psoralen with ultraviolet A (UVA), and khellin with UVA or UVB, along with combinations of topical preparations and various forms of UV. Surgical treatments that were assessed include full-thickness and split skin grafting, mini (punch) grafts, autologous epidermal cell suspensions, and autologous skin equivalents. The effectiveness of cognitive therapy and psychological treatments was considered. Therapeutic algorithms using grades of recommendation and levels of evidence have been produced for children and for adults with vitiligo.

All reported mutations in ALAS2, which encodes the rate-regulating enzyme of erythroid heme biosynthesis, cause X-linked sideroblastic anemia. We describe eight families with ALAS2 deletions, either c.1706-1709 delAGTG (p.E569GfsX24) or c.1699-1700 delAT (p.M567EfsX2), resulting in frameshifts that lead to replacement or deletion of the 19-20 C-terminal residues of the enzyme. Prokaryotic expression studies show that both mutations markedly increase ALAS2 activity. These gain-of-function mutations cause a previously unrecognized form of porphyria, X-linked dominant protoporphyria, characterized biochemically by a high proportion of zinc-protoporphyrin in erythrocytes, in which a mismatch between protoporphyrin production and the heme requirement of differentiating erythroid cells leads to overproduction of protoporphyrin in amounts sufficient to cause photosensitivity and liver disease.

Porphyria cutanea tarda (PCT), the most common of the porphyrias, is a mainly acquired disease of the liver, which manifests with bullous skin lesions. However, up to 20% of patients with PCT, usually those with chronic untreated disease, are reported to develop some sclerodermatous changes that may affect both light-exposed and nonexposed areas and that can be histologically indistinguishable from true scleroderma. A small number of patients with PCT has severe or generalized scleroderma, which is not necessarily due to coexistent systemic sclerosis. There are few reports in the literature that detail whether the severe sclerodermatous changes respond to control of the porphyria. We report a case of familial PCT with associated severe sclerodermatous changes causing scarring alopecia, cicatricial ectropion and skin thickening over the upper trunk. The scleroderma improved slightly over a 4-year follow-up period after treatment to normalize porphyrin excretion and prevent relapse.

Background: Vitamin D, produced by the action of sunlight on skin, is an important hormone for calcium homeostasis and has been implicated as tumour-protective agent. Some previous studies of photosensitive patients who actively avoid sunlight have failed to show convincing evidence of vitamin D insufficiency., Objectives: The aim of this study was to characterize the vitamin D status of a large cohort of patients with erythropoietic protoporphyria (EPP)., Methods: U.K. patients with EPP were recruited prospectively and seen locally by a single study investigator. A blood sample was taken for vitamin D assay. All blood analyses were performed in the same laboratory., Results: A cohort of 201 patients with known EPP was seen over a 7-month period between January and July. Thirty-four patients (17%) were deficient in vitamin D and 126 (63%) had insufficient vitamin D. Both insufficiency and deficiency were significantly associated with the total erythrocyte protoporphyrin concentration and inversely with the time in minutes to the onset of symptoms following sunlight exposure., Conclusions: This is the first report of significant levels of vitamin D deficiency and insufficiency in a large cohort of patients with a photodermatosis. Such individuals are at risk of associated adverse events. In future, clinicians should consider monitoring 25-hydroxyvitamin D levels and instigating oral supplementation or dietary advice if appropriate.

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.

Background: Some patients with psoriasis may require hospital admission to stabilize their condition, although the role of inpatient management is changing given recent advances in therapeutic options, emphasis on community-based care for chronic conditions and limited healthcare resources. There is a need for evidence-based national standards for inpatient management of psoriasis taking account of factors that predict length of stay., Objectives: To determine which factors predict length of stay for patients with psoriasis requiring inpatient hospital care with a view to setting evidence-based standards for inpatient psoriasis management., Methods: A multicentre service review was conducted on all psoriasis admissions over a 9-month period in four dermatology centres in the U.K. We collected data on admission, at discharge and, where possible, at 3 months following discharge. Psoriasis severity was assessed using four validated scoring systems, including Psoriasis Area and Severity Index (PASI) and Dermatology Life Quality Index. We also recorded length of stay and treatment details., Results: Length of stay varied widely between the four centres, but was similar in the two centres which received a high proportion of tertiary referrals for severe psoriasis (mean 19.7 days, range 1-78, analysis of variance P=0.002). Disease severity, measured by PASI, on admission (mean 15.7, interquartile range 8.3-20.8) was significantly higher in the tertiary centres (P<0.0001). However, there was no significant difference in PASI between centres on discharge. The admission PASI was significantly associated with length of stay (r=0.2, P=0.02). There was no significant correlation between other measures of disease severity and length of stay., Conclusions: Disease severity on admission for patients with psoriasis is greater in tertiary referral centres for psoriasis and is directly associated with length of stay. Length of stay should be used in conjunction with clinical measures such as PASI improvement to set national standards for quality in secondary care.