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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 (

Background Toxicity is rarely considered in the differential diagnosis of conjunctivitis, but we present here a new form of toxic conjunctivitis with unusual clinical features. Between 2010 and 2013, a new clinical presentation of chronic conjunctivitis unresponsive to normal treatment was noted within a Primary Care Ophthalmology Service. Methods Retrospective review of case records and histopathology results. Results A total of 55 adult patients, all females, presented with epiphora and stickiness. They did not complain of itch and had had symptoms for an average of 9 months. Clinical examination showed bilateral moderate to severe upper and lower tarsal conjunctival papillary reaction, without corneal or eyelid changes and mild bulbar conjunctival hyperaemia in a third of cases. Biopsies were taken in 15 cases to exclude an atypical infection or lymphoma. Histologically, there was a variable superficial stromal lymphocytic infiltrate, involving the epithelium in more severe cases. The majority of the cells were CD3 positive T-lymphocytes and follicle formation was not noted. The clinical history in all cases included prolonged use of eye make- up and other facial cosmetic products. Clinical symptoms of epiphora settled with topical steroid drops, but the clinical signs of chronic tarsal inflammation persisted until withdrawal of the facial wipes thought to contain the inciting agent, though the exact nature of this remains unclear. Conclusion The presentation, appearances, histological features are consistent with a contact allergen-driven chronic conjunctivitis. Steroid treatment provided good relief of symptoms and patients were advised to avoid potential contact allergens. Management remains difficult. Further research into contact allergies of mucous membranes and identification of its allergens is required. Electronic supplementary material The online version of this article (doi:10.1186/s12886-016-0294-1) contains supplementary material, which is available to authorized users.

Summary Background. For some time, there has been a suspicion that the number of articles published by UK-based authors in dermatology has declined. This probably reflects a reduction in the publication output of dermatology departments generally. Methods. We identified articles with British authorship in the British Journal of Dermatology between 1970 and the present date, and compared the journal with the three most commonly cited dermatological journals: Archives of Dermatology, Journal of Investigative Dermatology and Journal of the American Academy of Dermatology. Later, we expanded this search to include a further 33 dermatological journals. Results. Despite an increase in the total number of published papers by the British Journal of Dermatology, there was a decline in the number of British-authored papers, from 97 (57%) in 1970 to 80 (22%) in 2005. The trend was also seen in the Journal of the American Academy of Dermatology, with 16 papers (5%) in 1989 and 7 (2%) in 2005. In Journal of Investigative Dermatology, British papers increased from 10 papers in 1975 to 17 in 2005, with a percentage decrease from 7% to 4%. Overall, despite an increase in the total number of publications in dermatological journals from 2745 in 1985–5034 in 2005, British publications increased from 271 in 1989 to only 289 in 2005, which represents a percentage decrease from 10% to 6%. Conclusions. Despite a three-fold increase in dermatology consultants and registrars in UK, a three-fold increase in dermatological journals and a four-fold increase in dermatological papers published, the overall number of British papers has remained static over the years.

Harlequin ichthyosis (HI) is a rare autosomal recessive skin disorder. No orthopaedic procedure has previously been described on a patient with HI. We report the case of a 17-year-old patient with HI who presented with bilateral juvenile idiopathic arthritis of the hips who underwent bilateral total hip replacements. Our standard operative and postoperative regime was followed and no complications occurred. One year after the second procedure our patient had a Oxford hip score of 43 and was very satisfied with the result.

Importance Epidermolysis bullosa (EB) pruriginosa is a rare variant of dystrophic EB. It may manifest late in life and is characterized by intense pruritus, resulting in a phenotype resembling acquired inflammatory dermatoses. Dermatopathology textbooks include hereditary forms of EB among the “cell-poor” list of subepidermal blistering disorders. Observations We report a case of dominant dystrophic EB pruriginosa with late-onset cutaneous manifestations. A biopsy specimen showed subepidermal blistering with prominent inflammatory cells, including numerous eosinophils. Unfamiliarity with the distinctive clinicopathologic features of EB pruriginosa led to an initial erroneous histopathologic diagnosis of an acquired autoimmune blistering disorder. Direct immunofluorescence study results were negative for immune reactants. A strong clinical suspicion of hereditary EB pruriginosa led to mutation analysis of COL7A1, which confirmed a novel, heterozygous nonglycine missense mutation. Subsequently, 2 other family members who had nail dystrophy were also correctly diagnosed as having dominant dystrophic EB, highlighting the clinical spectrum of the disorder and the intrafamilial variability in disease presentation. Conclusions and Relevance The clinical features of EB pruriginosa are becoming more widely recognized, but dermatologists, dermatopathologists, and histopathologists should be aware that inflammatory infiltrates and late presentation are potential pitfalls in correctly diagnosing this subtype of hereditary EB.

Harlequin ichthyosis (HI) is the most severe and frequently lethal form of recessive congenital ichthyosis. Although defects in lipid transport, protein phosphatase activity, and differentiation have been described, the genetic basis underlying the clinical and cellular phenotypes of HI has yet to be determined. By use of single-nucleotide–polymorphism chip technology and homozygosity mapping, a common region of homozygosity was observed in five patients with HI in the chromosomal region 2q35. Sequencing of the ABCA12 gene, which maps within the minimal region defined by homozygosity mapping, revealed disease-associated mutations, including large intragenic deletions and frameshift deletions in 11 of the 12 screened individuals with HI. Since HI epidermis displays abnormal lamellar granule formation, ABCA12 may play a critical role in the formation of lamellar granules and the discharge of lipids into the intercellular spaces, which would explain the epidermal barrier defect seen in this disorder. This finding paves the way for early prenatal diagnosis. In addition, functional studies of ABCA12 will lead to a better understanding of epidermal differentiation and barrier formation.

Given the long purported anecdotal association between rosacea and gastrointestinal disease, the discovery that Helicobacter pylori causes gastritis and duodenal ulcer disease has led to a hypothesized role for this organism in the aetiology of rosacea. We conducted a case-series study of 49 patients to assess the potential association between severity of rosacea and direct and serological evidence of H. pylori infection. Patients were classified by severity into non-inflammatory erythematotelangiectatic or inflammatory/papulopustular rosacea and were tested for current H. pylori infection and evidence of previous exposure. Positive 13C-urea breath test and ELISA tests were more likely to be observed in patients with inflammatory rosacea, although not statistically significantly so (OR = 3.0, p = 0.15 and OR = 2.9, p = 0.16, respectively). However, the proportion of patients who tested positive in both assays (versus negative in at least one) was even higher in the inflammatory rosacea group and neared statistical significance (OR = 4.5, p = 0.06). This pilot study provides sufficient evidence suggestive of a positive association between the severity of rosacea and the presence of H. pylori to warrant further research.

A case of chronic low back pain caused by a glomus tumor that persisted more than 30 years is presented.To emphasize the need to consider skin tumors in the differential diagnosis of low back pain.Chronic low back pain can be caused by a myriad of factors. There are six relatively common skin tumors, which present as painful lesions. As seen in the reported case, if they occur in the lumbar region, they can cause low back pain.A subject with low back pain underwent an excision biopsy of a localized area of tenderness where his symptoms were reproduced when light pressure was applied.Histology confirmed that a glomus tumor accounted for the subject's pain.Painful skin tumors may cause low back pain and need to be considered in the differential diagnosis of chronic low back pain.

Sir, Adverse cutaneous reactions to drugs are among the most frequent complications of drug therapy. The most severe reaction is toxic epidermal necrolysis (TEN), a dermatological emergency with extensive epidermal necrosis resembling partial thickness burns and associated with high morbidity and mortality. StevensJohnson syndrome (SJS) and TEN constitute a spectrum of disease involving less than 10% and more than 30% epidermal detachment, respectively (1). Severe forms of SJS could evolve to TEN and the same drugs can induce both disorders (1). It is often impossible to distinguish between them at an early stage, since the final extent of detached epidermis is their distinguishing feature. We report here a case of incipient TEN induced by lamotrigine, where cyclosporine was used early in the course of the disease and appeared to halt its progression.

STUDY DESIGN A case of chronic low back pain caused by a glomus tumor that persisted more than 30 years is presented. OBJECTIVE To emphasize the need to consider skin tumors in the differential diagnosis of low back pain. SUMMARY OF BACKGROUND DATA Chronic low back pain can be caused by a myriad of factors. There are six relatively common skin tumors, which present as painful lesions. As seen in the reported case, if they occur in the lumbar region, they can cause low back pain. METHODS A subject with low back pain underwent an excision biopsy of a localized area of tenderness where his symptoms were reproduced when light pressure was applied. RESULTS Histology confirmed that a glomus tumor accounted for the subject's pain. CONCLUSIONS Painful skin tumors may cause low back pain and need to be considered in the differential diagnosis of chronic low back pain.

Objective: To assess the clinical outcomes of 45 cases of harlequin ichthyosis and review the underlying ABCA12 gene mutations in these patients. Design: Multicenter, retrospective, questionnaire-based survey. Setting: Dermatology research institute. Participants: Patients with harlequin ichthyosis for whom we had performed ABCA12 mutation analysis. Main Outcome Measures: Referring physicians were asked to complete a questionnaire using the patients' notes, detailing the clinical outcome of the affected child. In each case, the causative ABCA12 mutation was identified using standard polymerase chain reaction and sequencing techniques. Results: Of the 45 cases, the ages of the survivors ranged from 10 months to 25 years, with an overall survival rate of 56%. Death usually occurred in the first 3 months and was attributed to sepsis and/or respiratory failure in 75% of cases. The early introduction of oral retinoids may improve survival, since 83% of those treated survived, whereas 76% who were not given retinoids died. Recurrent skin infections in infancy affected one-third of patients. Problems maintaining weight affected 44%. Three children developed an inflammatory arthritis, and developmental delay was reported in 32%. Mutation analysis revealed that 52% of survivors had compound heterozygous mutations, whereas all deaths were associated with homozygous mutations. Conclusions: Harlequin ichthyosis should be regarded as a severe chronic disease that is not invariably fatal. With improved neonatal care and probably the early introduction of oral retinoids, the number of survivors is increasing. Compound heterozygotes appear to have a survival advantage.

Women in Detroit can now get more than hot rollers and highlights at their weekly trip to the hair salon. In a campaign to cut high rates of hypertension, diabetes, and renal disease among the city's African-Americans, the National Kidney Foundation of Michigan is teaching stylists to spot suspicious symptoms and encourage their clients to see a doctor ( Detroit News , 9 September 1999). The scheme could go nationwide if it is successful, although it's still unclear how women will respond to being probed about their health while trapped under a hair dryer. The helicopter ambulance in Tromso, a rural area in north Norway, takes about 20 minutes to get airborne after an emergency call. Emergency medicine specialists estimate, however, that speeding up the take off would make little difference to the patients ( Prehospital Immediate Care 1999;3:136-9). An expert panel reviewed 65 cases over two years - 51 deaths and 14 survivors with ongoing problems - and concluded that shortening the response time by 15 minutes would have improved the outcome in only two patients; a 6% increase in life years gained across the board. Electroconvulsive therapy has …