Summary 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.

The DLQI banding concept provides a further tool to assess the impact of biologics on HRQoL of patients with psoriasis. Based on retrospective application of DLQI bands to published RCT data, infliximab, followed by etanercept, showed the greatest improvement in the overall HRQoL paralleled by a 75% improvement in the Psoriasis Area and Severity Index. However, some publications did not provide absolute baseline DLQI values, making interpretation of data and comparison between the agents difficult. Side-to-side comparative studies between biologics and between biologics and nonbiological psoriasis treatments will aid evidence-based psoriasis management decisions in the future.

Summary 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.

This study provides the longest available follow-up data of children and adults with DOK on oral retinoid therapy. Such information is essential for clinicians and their patients with DOK embarking on life-long treatment with retinoids.

In psoriasis there is a relationship between the type of management decision and the patient-rated QOL. However, there is a very wide score scatter for each management decision, challenging the appropriateness of some clinical decisions.

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Allergens used for patch testing in the hairdressing series vary between dermatology centres in the UK. The aim of our study is to ascertain the hairdressing allergens currently in use and their test results in several dermatology centres in the UK. Data were obtained from databases in 9 dermatology departments. The allergens with positive results and current/past relevance were included in a new hairdressing series based on collective experience, for wider use and further evaluation.

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