Observation We report the clinical course and management in 2 women with metastatic melanomas harboring the BRAFV600E mutation, who developed panniculitis with arthralgia during therapy with selective oral BRAF inhibitors. Panniculitis with arthralgia was the acute presenting adverse effect in both patients. Painful, red, nodular lesions were located on the upper and lower extremities. Biopsy specimens of the nodules showed a mild, predominantly lobular neutrophilic panniculitis. Analgesic and anti-inflammatory treatment improved panniculitis and arthralgia in both cases. It was also necessary to reduce the BRAF inhibitor dose in 1 patient.
Conclusions During therapy with selective BRAF inhibitors, panniculitis with arthralgia represents a new adverse effect that can require dose reduction. In case of this adverse effect, treatment with nonsteroidal anti-inflammatory drugs, such as etoricoxib, should be initiated early to keep patients on treatment and to avoid drug discontinuation and tumor progression.
]]>Design Open-label, dose-escalation, phase I trial.
Setting Dermatology outpatient clinic and clinical research unit at a university teaching hospital.
Patients A total of 21 adult patients (11 male, 10 female) with early-stage (IA-IIA) refractory CTCL, mycosis fungoides type, treated with topical carmustine following intravenous O6-benzylguanine.
Intervention Treatment once every 2 weeks with 120 mg/m2 intravenous O6-benzylguanine followed 1 hour later by whole-body, low-dose topical carmustine starting at 10 mg, with 10-mg incremental dose-escalation in 3 patient cohorts. Cutaneous T-cell lymphoma lesional skin biopsy specimens were taken at baseline and 6 hours, 24 hours, and 1 week after the first O6-benzylguanine infusion for analysis of O6-alkylguanine-DNA alkyltransferase activity.
Main Outcome Measures Clinical response measured by physical examination and severity-weighted assessment tool measurements, safety data acquired by review of adverse events at study visits, and O6-alkylguanine-DNA alkyltransferase activity in treated lesion skin biopsy specimens.
Results A minimal toxic effect was observed through the 40-mg carmustine dose level with 76% of adverse events being grade 1 based on the National Cancer Institute Common Terminology Criteria for Adverse Events. Mean baseline O6-alkylguanine-DNA alkyltransferase activity in CTCL lesions was 3 times greater than in normal controls and was diminished by a median of 100% at 6 and 24 hours following O6-benzylguanine with recovery at 1 week. Clinical disease reduction correlated positively with O6-alkylguanine-DNA alkyltransferase activity at 168 hours (P = .02) and inversely with area under the curve of O6-alkylguanine-DNA alkyltransferase over 1 week (P = .01). Twelve partial responses and 4 complete responses were observed (overall response, 76% [95% CI, 0.55-0.89]). Five patients discontinued therapy owing to adverse events with a possible, probable, or definite relationship to the study drug.
Conclusion O6-benzylguanine significantly depletes O6-alkylguanine-DNA alkyltransferase in CTCL lesions and in combination with topical carmustine is well tolerated and shows meaningful clinical responses in CTCL at markedly reduced total carmustine treatment doses.
Trial Registration clinicaltrials.gov Identifier:
Design Descriptive study.
Setting Death certificate records from 26 states, representing approximately 45% of the US population as reported by the National Center for Health Statistics, with recorded educational level information and population data from the US Bureau of Census Current Population Survey.
Patients Recorded deaths from malignant melanoma in non-Hispanic whites reported from 1993 through 2007.
Main Outcome Measures Age-standardized mortality rates for melanoma were evaluated by educational attainment (a marker of socioeconomic status) among non-Hispanic whites (aged 25-64 years) from 1993 through 2007. Rate ratios assessed the time trend in age-adjusted death rates by sex and educational level. Mortality differentials in educational level were measured using the regression-based Relative Index of Inequality. All statistical tests were 2-sided.
Results Melanoma mortality declined significantly between 1993-1997 and 2003-2007 in men (RR [rate ratio], 0.916; 95% CI, 0.878-0.954; P < .001) and women (RR, 0.907; 95% CI, 0.857-0.957; P < .001). However, these declines occurred only among the most educated persons (≥13 years of education irrespective of sex), and nonsignificant increases were found among the least-educated individuals, specifically men (P = .17). As a result, the Relative Index of Inequality by education in melanoma mortality in 2003-2007 relative to 1993-1997 (baseline) widened by 51.7% in men and by 35.7% in women.
Conclusions Recent declines in melanoma mortality rates among non-Hispanic whites in the United States mainly reflect declines among the most-educated individuals. The widening disparities in melanoma mortality rates by education calls for early detection strategies to effectively target high-risk, less-educated, non-Hispanic white individuals.
]]>Design A crossover quasiexperimental study design comparing 3 sunscreen dispenser types.
Setting Children aged 5 to 12 years from public primary schools (grades 1-7) in Queensland, Australia.
Participants Children (n = 87) and their parents randomly recruited from the enrollment lists of 7 primary schools. Each child provided up to 3 observations (n = 258).
Intervention Children applied sunscreen during 3 consecutive school weeks (Monday through Friday) for the first application of the day using a different dispenser each week.
Main Outcome Measure Thickness of sunscreen application (in milligrams per square centimeter). The dispensers were weighed before and after use to calculate the weight of sunscreen applied. This was divided by the coverage area of application (in square centimeters), which was calculated by multiplying the children's body surface area by the percentage of the body covered with sunscreen.
Results Children applied their sunscreen at a median thickness of 0.48 mg/cm2. Children applied significantly more sunscreen when using the pump (0.75 mg/cm2) and the squeeze bottle (0.57 mg/cm2) compared with the roll-on (0.22 mg/cm2) (P < .001 for both).
Conclusions Regardless of age, primary schoolchildren apply sunscreen at substantially less than 1.00 mg/cm2, similar to what has been observed among adults. Some sunscreen dispensers seem to facilitate thicker application than others.
]]>Design Retrospective cohort study.
Setting Two tertiary melanoma referral centers.
Patients A total of 698 patients with invasive primary cutaneous melanoma in whom the SLN was identified between January 1, 2000, and June 30, 2010.
Main Outcome Measure Based on previous studies, a surrogate measure for GR in primary invasive melanoma was calculated as the ratio of Breslow thickness to time to melanoma development.
Results The SLN was positive in 20.2% of patients. Multivariate logistic regression analysis revealed that GR, Breslow thickness, and the presence of microscopic satellitosis were independently associated with SLN positivity. The probability of SLN positivity was 8.2% for slow-growth melanomas (<0.10 mm/mo) compared with 19.8% for intermediate-growth melanomas (0.10-0.50 mm/mo) and 37.7% for fast-growth melanomas (>0.50 mm/mo). Growth rate was not an independent predictive factor for survival.
Conclusion Growth rate of primary cutaneous melanoma, together with Breslow thickness and the presence of microscopic satellitosis, predicts the histological SLN positivity.
]]>Design Descriptive survey study.
Setting Office-based practicing physicians in the United States.
Participants One thousand dermatologists.
Main Outcome Measure Melanoma risk communication practices.
Results Of 974 eligible dermatologists, 406 completed the survey (response rate, 41.7%). Almost 85% of dermatologists reported that they often or always communicate risk to patients with melanoma about their first-degree relatives, and almost 80% reported that they often or always advise their patients with melanoma that their older children (≥18 years) may be at greater risk of skin cancer. However, less than 50% of dermatologists routinely offered to screen first-degree relatives who live nearby, while only 19.7% used medical record reminders to note communication of melanoma risk to family members. Most dermatologists reported no major barriers to melanoma risk communication. However, the presence of "any risk communication barrier" (time constraints, absence of guidelines, or lack of written material) was associated with reduced melanoma risk communication practices by dermatologists.
Conclusions The observed high rates of self-reported risk communication by dermatologists to patients with melanoma about their first-degree family members are encouraging. However, the reported low rates of actual screening of first-degree relatives warrant easy-to-administer office-based medical record reminders to facilitate and optimize screening of at-risk relatives.
]]>Design A cross-sectional survey study conducted between May 30, 2007, and December 4, 2007.
Setting The Emory University campus and surrounding locations in Atlanta, Georgia.
Participants Four hundred fifteen community and university-affiliated women.
Main Outcome Measures Self-reported use of sunless tanning products and UV radiation tanning methods.
Results Forty-eight percent of participants had used sunless tanning products, 70.6% had tanned in the sun, and 26.0% had used tanning beds at least once in the past year. Most participants (92.7%) believed that tanned skin is more attractive than untanned skin, and 79.2% reported feeling better about themselves when tan. Many sunless tanning product users reported decreased frequency of tanning in the sun (36.8%) or in tanning beds (38%) because of product use. Frequent users were more likely to have decreased their UV radiation exposure. Lighter complexion, frequent use of UV radiation tanning methods, feeling better about oneself when tan, and having a history of skin cancer were independently associated with sunless tanning product use.
Conclusions The desire for tanned skin remains strong despite growing awareness of the dangers of UV radiation exposure. In some women, sunless tanning product use is associated with decreased UV radiation tanning frequency, especially in women who use them repeatedly. Improvements in the appearance of sunless tanning product tans may allow wider acceptance by the public and further decreases in UV radiation tanning practices.
]]>Design Retrospective study.
Setting Tertiary referral center.
Patients Seventy-eight patients with calcinosis cutis and ACTD between 1996 and 2009.
Main Outcome Measures Clinical features, treatments, and outcomes of patients with calcinosis cutis in the clinical setting of ACTD.
Results Of 78 patients (mean age at onset of calcinosis cutis, 40.1 years), 64 (82%) were female. The following diseases were associated with calcinosis cutis: dermatomyositis (n = 30) with classic (n = 15), juvenile (n = 14), and amyopathic (n = 1) subtypes; systemic sclerosis with limited cutaneous scleroderma (n = 24); lupus panniculitis (n = 4); systemic lupus erythematosus (n = 2); mixed connective tissue disease (n = 4); overlap connective tissue disease (n = 6); undifferentiated connective tissue disease (n = 6); polymyositis (n = 1); and rheumatoid arthritis (n = 1). Therapy for calcinosis cutis consisted of medical treatment alone (n = 19), surgical therapy alone (n = 11), combined medical and surgical treatment (n = 17), no treatment (n = 30), and unknown (n = 1). Diltiazem hydrochloride was the most commonly used medical therapy, with 9 of 17 patients having a partial response. Twenty-eight patients had surgical excision of 1 or more lesions of calcinosis cutis: 22 had a complete response, 5 had a partial response, and 1 had no response.
Conclusions Dermatomyositis and systemic sclerosis were the most common ACTDs associated with calcinosis cutis. Although no treatment was uniformly effective, surgical excision of symptomatic lesions and medical treatment with diltiazem provided benefit for some patients.
]]>Data Sources MEDLINE, Cochrane, and PubMed databases.
Study Selection English-language prospective, retrospective, and case studies describing at least 4 patients with HS.
Data Extraction Data quality and validity were addressed by multiple reviewers using independent extraction.
Data Synthesis Studies were categorized as treatments using antibiotics, biological agents, laser surgery, excisional surgery, or miscellaneous modalities. Of 62 publications included in the review, 4 studies met criteria to be assigned the highest grade for quality of evidence.
Conclusions Shown to be effective treatments for HS were a clindamycin-rifampin combination regimen, a course of infliximab, monthly Nd:YAG laser sessions, and surgical excision and primary closure with a gentamicin sulfate–collagen sponge. Most therapies used to treat HS were supported by limited or weak scientific evidence. A treatment approach is presented based on the evidence and on clinical experience at the Follicular Disorders Clinic, Department of Dermatology, Henry Ford Hospital, Detroit, Michigan. This review emphasizes the need for large randomized controlled trials to evaluate treatment options for HS.
]]>Data Sources We performed a systematic search for all entries in PubMed, CINAHL, Cochrane Review, and EMBASE related to combination treatments for psoriasis through July 2010.
Study Selection We included randomized controlled trials that reported proportion of disease clearance or mean change in clinical severity score (or provided these data through communication with study authors) for efficacy of a combination treatment for psoriasis compared with 1 or more corresponding monotherapies.
Data Extraction Study data were extracted by 3 independent investigators, with disagreement resolved by consensus. The proportion of patients who achieved clearance, definition of clearance, means and standard deviations for baseline disease symptom score and final disease symptom score, and major design characteristics were extracted for each study.
Data Synthesis Combination treatments consisting of vitamin D derivative and corticosteroid, vitamin D derivative and UV-B, vitamin A derivative and psoralen–UV-A, vitamin A derivative and corticosteroid, vitamin A derivative and UV-B, corticosteroid and hydrocolloid occlusion dressings, UV-B and alefacept, and vitamins A and D derivatives were more effective than 1 or more monotherapies using the likelihood of clearance as the outcome. Blinding status and potency of the corticosteroid treatment used were significant sources of heterogeneity between studies.
Conclusions The results demonstrate the need for additional long-term trials with standardized outcome measures to evaluate the efficacy and adverse effects of combination therapies for psoriasis and highlight the possible effects of trial design characteristics on results.
]]>Design Randomized, multicenter, controlled, prospective phase 3 trial with blinded assessment of outcome measures by a single observer.
Setting Two hospital referral centers in Caen and Lyon, France.
Patients Random sampling of 119 patients with a nonhealing, sloughy wound 40 cm2 or smaller, less than 2 cm deep, and an ankle brachial index of 0.8 or higher.
Intervention During a 2-week hospital stay, patients received either maggot debridement therapy (MDT) or conventional treatment. At discharge, conventional dressings were applied and a follow-up visit occurred at day 30.
Main Outcome Measure Percentage of slough in wounds at day 15.
Results There was a significant difference between groups at day 8 (54.5% in the MDT group and 66.5% in the control group) (P = .04). The mean percentage of slough at day 15 was 55.4% in the MDT group and 53.8% in the control group (P = .78).
Conclusions Although MDT shows no significant benefit at day 15 compared with conventional treatment, debridement by MDT is significantly faster and occurs during the first week of treatment. Because there is no benefit in continuing the treatment after 1 week, another type of dressing should be used after 2 or 3 applications of MDT.
Trial Registration clinicaltrials.gov Identifier:
Observations We present a unique case of multiple pagetoid Spitz nevi initially diagnosed as multiple in situ melanomas. Germline karyotyping, CDK4 and CDKN2A sequencing, and comparative genomic hybridization of HRAS, BRAF, KRAS, RAF1, CDKN2A, Rb1, MAP2K1, MAP2K2, PTEN, and PTPN11 genes did not identify mutations in this case. Germline and somatic sequencing of BRAF exon 15 revealed no mutations at V600D/E/K. In addition, single-nucleotide polymorphism microarray analysis (330K) on lesional and normal skin revealed no genome-wide copy number changes or loss of heterozygosity.
Conclusions Clinicians should be aware of the occurrence of multiple pagetoid Spitz nevi to avoid morbidity associated with the misdiagnosis of multiple melanomas. The genetic mechanisms of pagetoid spread of melanocytes are not fully understood.
]]>Design Cross-sectional and 9-month prospective cohort.
Setting Urban university setting.
Participants University students.
Intervention Participants were asked to fill out a survey form, were swabbed for culture, and had a visual examination for acne.
Main Outcome Measure Report of pharyngitis.
Results In the cross-sectional study, 10 of the 15 students receiving oral antibiotics for acne reported an episode of pharyngitis in the previous 30 days, whereas 47 of the 130 students not receiving oral antibiotics, but who had acne, reported an episode of pharyngitis in the prior month. The unadjusted odds ratio (OR) (95% CI) associating current oral antibiotic use in acne patients with a self-reported episode of pharyngitis was 3.53 (95% CI, 1.14-10.95). In the cohort study, there were 358 female and 218 male participants; 36 (6.2%) received oral antibiotics for acne during the study, and 96 (16.6%) received topical antibiotics for acne. Using mixed model logistic regression, the OR was 4.34 (95% CI, 1.51-12.47) associating oral antibiotic use with pharyngitis. Less than 1% of participants were colonized by group A streptococcus, which was not associated with pharyngitis.
Conclusions Our studies show that that the odds of reporting pharyngitis is more than 3 times baseline in patients receiving oral antibiotics for acne vs those who are not receiving oral antibiotics. The true clinical importance of these findings needs to be evaluated further by prospective studies, but this finding is not associated with group A streptococcus.
]]>Design A 12-month randomized, double-blind, placebo-controlled trial.
Setting A tertiary referral dermatology center in Singapore.
Participants Patients newly diagnosed as having an immunobullous disease and deemed to require at least 6 months of systemic glucocorticoid therapy.
Interventions The patients were randomized to receive either oral alendronate sodium (10 mg/d) or a matching placebo for 12 months. All patients also received concurrent calcium with vitamin D, 2 tablets daily.
Main Outcome Measures Percent change in bone mineral density (BMD) at the lumbar spine and the femoral neck at 12 months.
Results A total of 29 patients (alendronate [n = 15], placebo [n = 14]) were evaluated. The percent change in BMD in the alendronate group was +3.7% and +3.5% at the lumbar spine and the femoral neck, respectively, whereas in the placebo group, it was –1.4% and –0.7% at the lumbar spine and the femoral neck, respectively. The increase in BMD observed in the alendronate group compared with the placebo group was statistically significant at both the lumbar spine (P = .01) and the femoral neck (P = .01). There was also a statistically significant decrease in serum heat-labile alkaline phosphatase levels after 12 months (–32.6%, P < .01) in the alendronate group but not in the placebo group. Adverse events were generally minor, and the frequency of occurrence did not differ significantly between both treatment groups (P = .59).
Conclusions There were statistically significant increases in BMD at both the lumbar spine (P = .01) and the femoral neck (P = .01) with alendronate therapy. It is imperative to use bisphophonate therapy in patients with immunobullous disorders who are receiving oral corticosteroids because it largely prevents the morbidity associated with low BMD.
]]>Design Prospective longitudinal cohort study.
Setting Urban cutaneous autoimmune disease clinic.
Participants A total of 218 individuals with CLE or systemic lupus erythematosus and lupus nonspecific skin disease seen between January 5, 2007, and July 30, 2010.
Main Outcome Measures Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) scores to assess disease severity and response to treatment and Skindex 29+3 scores to assess patient quality of life.
Results Current smokers with lupus erythematosus had higher median CLASI scores (9.5) than did never (7.0) and past (6.0) smokers with CLE (P = .02). Current smokers had higher median scores on all the Skindex 29+3 subsets. Current smokers taking hydroxychloroquine sulfate had higher quinacrine hydrochloride use than did nonsmokers (P = .04). Two to 7 months after enrollment, current smokers (median CLASI change, –3) treated with only antimalarial agents improved more than never (1) and past (0) smokers (P = .02). Eight months or more after enrollment, current smokers (CLASI change, 3.5) treated with antimalarial drugs plus at least 1 additional immunomodulator improved less than never (–1.5) and past (0) smokers (P = .04).
Conclusions Current smokers with lupus erythematosus had worse disease, had worse quality of life, and were more often treated with a combination of hydroxychloroquine and quinacrine than were nonsmokers. Never and past smokers showed greater improvement when treated with antimalarial agents plus at least 1 additional immunomodulator. Current smokers had greater improvement when treated with antimalarial drugs only.
]]>Design Using a novel monoclonal antibody against soluble adenylyl cyclase (sAC), various benign and malignant melanocytic proliferations were immunostained.
Setting Weill Medical College of Cornell University dermatopathology laboratory.
Main Outcome Measures The results were qualitative, not quantifiable.
Results The sAC immunostaining produced distinctive patterns that paralleled melanomagenesis. At one pole of the spectrum were benign nevi, including atypical nevi of special sites and recurrent nevi showing a distinct pattern of dotlike Golgi staining, while at the opposite pole was melanoma, in which many cells demonstrated an intense pannuclear expression pattern, often accompanied by loss of the Golgi expression pattern. Melanomas of lentigo maligna and acral lentiginous subtypes exhibited the most striking pannuclear expression, while nodular melanomas showed the least, although with supervening enhanced diffuse cytoplasmic expression. Loss of the Golgi expression pattern was a feature of malignant melanoma.
Conclusion The sAC expression pattern is complex but seems discriminatory, with distinctive and variable staining patterns according to the nature of the lesion biopsied.
]]>Design Prospective analysis of 12 patients with HS.
Setting Unité de Cancéro-Dermatologie, Nantes Hospital, Nantes; Service de Dermatologie, Poitiers Hospital, Poitiers; and Service de Dermatologie, Clinique de Courlancy, Reims, France
Patients Twelve patients (Hurley stage I or II) in whom the disease had progressed for at least 6 months and who had a minimum of 2 closed nodules in typical sites.
Main Outcome Measures Two biopsies were performed at baseline: one in a closed inflammatory nodule and one in healthy adjoining skin. Patients were treated for 3 months with zinc gluconate at a dosage of 90 mg/d. A new biopsy was then performed in the same nodule. Innate immunity markers (toll-like receptors 2, 3, 4, 7, and 9; intercellular adhesion molecule 1; interleukin [IL] 6 and 10; tumor necrosis factor; α melanocyte stimulating hormone; transforming growth factor β; β-defensin 2 and 4; and insulinlike growth factor 1) were studied by immunohistochemical analysis.
Results We observed significantly decreased expression (P < .001) of all the innate immunity markers studied except IL-10 in nonlesional and lesional HS skin. The downregulation of innate markers was significantly stronger in lesional HS skin compared with normal skin except for tumor necrosis factor. Three months of zinc treatment induced a significant increase in the expression of all the markers involved in innate immunity.
Conclusion Our study demonstrates for the first time, to our knowledge, that a deficiency of the main innate immunity markers in typical HS sites may explain the development of chronic inflammatory nodules in this disease.
]]>Observations We describe a 21-year-old man with recessive dystrophic epidermolysis bullosa carrying the homozygous c.6508C>T (p.Gln2170X) nonsense mutation who reported an unaffected skin patch on his neck where blisters never had occurred. Immunofluorescent type VII collagen staining was normal in 80% of the unaffected skin biopsy; however, it was strongly reduced in the affected skin. In the unaffected skin, the somatic nucleotide substitution c.6510G>T reverted the germline nonsense codon to tyrosine (p.Gln2170Tyr), thereby restoring functional protein production.
Conclusions Revertant mosaicism is considered rare in recessive dystrophic epidermolysis bullosa. However, it might be more common than previously anticipated because our patient is the third in whom revertant mosaicism was identified in a short period of time. The correction mechanism is different than that previously reported. Systematic examination of patients with recessive dystrophic epidermolysis bullosa, therefore, will likely reveal more patients with revertant patches. This is important because the natural gene therapy phenomenon may provide opportunities for revertant cell therapy.
]]>Design Population-based case-control study in Queensland, Australia.
Setting General community.
Participants Population-based sample of 49 patients with LMM and 141 with SSM (in situ or invasive) aged 14 to 86 years at diagnosis in 1979 and 1980 and 232 control subjects. Response rates were 97.1% in cases and 91.8% in controls.
Main Outcome Measures Risks of both subtypes in relation to phenotypic and environmental factors, estimated by multinomial logistic regression.
Results The number of solar lentigines was the strongest determinant for LMM (odds ratio [OR], 15.93; P < .001 for trend) and significantly weaker for SSM (4.61; P < .001 for trend; P = .04 for homogeneity). Skin cancer history was significantly associated with LMM (OR, 2.84) but not with SSM (1.33; P = .07 for homogeneity). In contrast, the number of nevi was the strongest determinant for SSM (OR, 23.22; P < .001 for trend) while significantly weaker for LMM (3.60; P = .02 for trend; P < .001 for homogeneity). Multiple lifetime sunburns almost tripled the risk for SSM, whereas no association was detected with LMM (P = .04 for homogeneity). Shared risk factors for both subtypes were the number of solar keratoses (P < .001 for trend for both) and sun-sensitive complexion (ie, light eye/hair colors, sunburn propensity, and freckling) (2-fold to 5-fold increased risks).
Conclusions A propensity to lentigines is a stronger predictor of LMM, whereas high nevus propensity is a stronger predictor of SSM. Skin cancer history appears to determine LMM risk only, whereas the number of lifetime sunburns determines SSM only. Prevention strategies could be tailored differently given these distinctive points of difference.
]]>Design Retrospective medical record review with prospective blinded histopathologic analysis.
Setting University-affiliated dermatology and dermatopathology practice.
Patients Fifty-nine patients with SCLE were divided into DI (n = 15) and non-DI (n = 44) groups.
Main Outcome Measures A dermatopathologist masked to the etiologic associations reviewed corresponding histopathologic specimens. For each patient, an eosinophil ratio was calculated as the mean eosinophil score (averaging eosinophil counts from 10 high-power histologic fields) divided by the intensity of inflammation. Eosinophil ratios for both groups were compared using the Mann-Whitney test.
Results No significant difference was found in mean eosinophil ratios in the DI vs non-DI groups (0.11 vs 0.004; P = .34). Mucin deposition was present in both populations and was not significantly different (P = .18). The inflammatory infiltrate was superficial and deep in 10 patients (67%) in the DI group vs 24 (55%) in the non-DI group. Periadnexal inflammation was observed in 12 patients (80%) in the DI group vs 37 (84%) in the non-DI group, and basal layer liquefaction with dyskeratosis was seen in 15 patients (100%) in the DI group and in 37 (84%) in the non-DI group.
Conclusions Tissue eosinophilia is not a differentiating histopathologic feature of DI-SCLE. Careful review of a patient's drug history in correlation with clinical findings remains the standard for identifying a drug as an etiologic or exacerbating factor in patients with SCLE.
]]>Design A prospective multicenter study.
Setting Four French academic dermatology departments: Strasbourg, Montpellier, Tenon Hospital Paris, and Henri Mondor Hospital Créteil.
Patients Patients were recruited from November 1, 2008, through June 30, 2010. Seventy-six patients with morphea and 101 age- and sex-matched controls, who underwent complete clinical examination, were enrolled.
Interventions A complete clinical examination and, if deemed necessary, a cutaneous biopsy.
Main Outcome Measure The frequency of genital LS.
Results There were 58 women and 18 men (a 3:1 ratio) with a median age of 59 years. Mean (range) age at diagnosis was 54 (13-87) years. Forty-nine patients had plaque morphea, 9 had generalized morphea, and 18 had linear morphea. Three patients (3%) in the control group and 29 patients (38%) with morphea had LS (odds ratio, 19.8; 95% CI, 5.7-106.9; P < .001). Twenty-two patients with plaque morphea (45%) and only 1 patient with linear morphea (6%) had associated genital LS.
Conclusions Genital LS is significantly more frequent in patients with morphea than in unaffected individuals. Forty-five percent of patients with plaque morphea have associated LS. Complete clinical examination, including careful inspection of genital mucosa, should therefore be mandatory in patients with morphea because genital LS bears a risk of evolution into squamous cell carcinoma and thus needs treatment with topical corticosteroids.
]]>Design Retrospective population-based cohort study.
Setting Original 9 registries of the Surveillance, Epidemiology, and End Results Program from 1978 to 2007.
Participants A total of 111 478 patients with histologically confirmed invasive melanoma.
Main Outcome Measure Proportion of ultimately fatal melanomas by subtype.
Results Among melanomas of known subtype, superficial spreading melanoma comprised 66% of incident melanomas and 46% of ultimately fatal melanomas; nodular melanoma comprised 14% of incident melanomas and 37% of ultimately fatal melanomas. For superficial spreading melanoma, overall incidence per 100 000 per year increased (from 4.28 to 6.63), ultimately fatal incidence remained stable (at 0.56 to 0.51), and 10-year relative survival increased (from 90.6% to 96.5%) when comparing successive 5-year intervals. In contrast, for nodular melanoma, the overall incidence (1.30-1.32), ultimately fatal incidence (0.46-0.44), and 10-year relative survival rate (61.8%-61.5%) remained stable. Epidemiologic trends of melanoma, not otherwise specified, were similar to superficial spreading melanoma. There was a strong negative correlation between the proportion of melanoma, not otherwise specified, among all melanomas, and the proportion of superficial spreading melanoma, among melanomas of known subtype (r = –0.80; P = .01), across the registries.
Conclusions Superficial spreading and nodular melanoma constitute similar proportions of ultimately fatal melanomas. Although incidence of and survival from superficial spreading melanoma have increased from 1978 to 2007, neither the incidence of nor survival from nodular melanoma has changed. Public health efforts should include a focus on nodular melanoma for maximum reduction of melanoma mortality.
]]>Design Wrinkle depth was assessed with image analysis on the forehead and the lateral canthus of human cadavers. The density of the retinacula cutis was measured in Azan-Mallory–stained skin sections obtained around the wrinkles.
Setting Gross Anatomy Section, Kagoshima University Graduate School of Medical and Dental Sciences.
Participants Fifty-five male and female cadavers (35-93 years old).
Main Outcome Measures The maximum depth of each wrinkle was used to represent the wrinkle's degree. In the skin sections, the density of the retinacula cutis was measured around the deepest point of each wrinkle in a 1-mm-wide area (the wrinkle-specific area) and a 10-mm-wide area that included the wrinkle (the wrinkle-inclusive area).
Results In both the wrinkle-specific and wrinkle-inclusive areas, the retinacula cutis densities became lower in the forehead and in the lateral canthus areas. When a wrinkle was shallow, the density was lower in the wrinkle-specific area than in the wrinkle-inclusive area. With wrinkle progression, the density difference between the wrinkle-specific and the wrinkle-inclusive areas gradually decreased until there was no apparent difference.
Conclusions Facial wrinkles seem to develop above sites of reduced lower retinacula cutis density. As a wrinkle develops, the density decreases in both the wrinkle-specific and the wrinkle-inclusive areas, whereas the density difference between those areas vanishes.
]]>Design Two-center randomized controlled trial with 2-year follow-up.
Setting Interventions were performed on ambulatory and hospitalized patients at 2 vein centers, a university dermatology department (EVLT-treated group), and a specialized vein clinic (HLS-treated group).
Patients Random sample of 400 patients with GSV insufficiency.
Interventions Patients were assigned (1:1) to EVLT or HLS of the GSV from September 2004 through March 2007; 185 and 161 patients (limbs), respectively, were treated per protocol.
Main Outcome Measures Clinically recurrent varicose veins after surgery (REVAS classification, primary study objective), duplex-detected saphenofemoral recurrence, clinical venous severity scoring (Homburg Varicose Vein Severity Score), hemodynamics (venous refilling time), quality of life (Chronic Venous Insufficiency Questionnaire 2), adverse effects, and visual analog scale–based evaluations of patients' satisfaction.
Results Clinically recurrent varicose veins after surgery were similarly observed in both groups: 16.2% (EVLT-treated group) vs 23.1% (HLS-treated group); P = .15. Duplex-detected saphenofemoral refluxes occurred significantly more frequently after EVLT (17.8% vs 1.3%; P < .001). Both treatments equally improved medical condition (Homburg Varicose Vein Severity Score) and disease-related quality of life. Endovenous laser treatment caused more adverse effects (phlebitic reaction, tightness, dyspigmentation) but revealed advantages concerning hemodynamics, recovery, and cosmetic outcome.
Conclusions Both EVLT and HLS are comparably safe and effective procedures to treat GSV incompetence. The significantly higher rate and the course of duplex-detected saphenofemoral recurrences after EVLT remain a matter of further investigations.
Trial Registration isrctn.org Identifier:
Design We conducted a retrospective analysis of 1500 patients evaluated via live interactive teledermatology between 2003 and 2005 at the University of California, Davis. We compared diagnoses and treatment plans between the referring physicians and the teledermatologists. Patients with 2 or more teledermatology visits within a 1-year period were assessed for changes in clinical outcomes.
Setting Academic medical center with an established teledermatology program since 1996.
Participants Medical records were evaluated for 1500 patients who underwent live interactive teledermatology consultation. Patients seen for more than 1 teledermatology visit were included in the clinical outcome assessment.
Intervention Live interactive teledermatology consultation.
Main Outcome Measures Changes in diagnosis, disease management, and clinical outcome.
Results Compared with diagnoses and treatment plans from referring physicians, the 1500 live interactive teledermatology consultations resulted in changes in diagnosis in 69.9% of patients and changes in disease management in 97.7% of patients. Among 313 patients with at least 2 teledermatology visits within 1 year, clinical improvement was observed in 68.7% of patients. Multivariate analysis showed that changes in diagnosis (P = .01), changes in disease management (P < .001), and the number of teledermatology visits (P < .001) were significantly associated with improved clinical outcomes.
Conclusions Live interactive teledermatology consultations result in changes in diagnosis and disease management in most consultations. The numbers of live interactive teledermatology visits and changes in diagnosis and disease management are significantly associated with improved clinical outcomes.
]]>Design Retrospective, cross-sectional investigation using National Ambulatory Medical Care Survey data.
Setting US ambulatory physician offices from 1979 through 2007.
Patients Children with psoriasis ages 0 (birth) to 18 years.
Main Outcome Measures Demographics, physician specialty, and medications prescribed.
Results There were an estimated 3.8 million visits for psoriasis over the study interval with a median of 123 420 visits per year. Dermatologists saw 63% of patients, pediatricians saw 17%, and internists, 14%. The numbers of visits were equal between sexes but ranged by age group: patients ages 13 to 18 years accounted for 47% of visits, those ages 8 to 12 years for 35%, and those ages 0 to 7 for 18%. Ninety-three percent of patients were white. Topical corticosteroids were the most commonly prescribed medications. Children 0 to 9 years old received equally potent corticosteroids as children 10 to 18 years old. Among all patients, the most prescribed medication was topical betamethasone; among those ages 0 to 9 years, tacrolimus; and among those ages 10 to 18 years, betamethasone. By physician specialty, the most prescribed medications were high-potency steroids for dermatologists and internists, and topical tacrolimus for pediatricians. Topical calcineurin inhibitors were not among the top 20 most prescribed medications by dermatologists, and systemic antipsoriatic agents were not among the top 20 most prescribed medications in any age group.
Conclusions Over the 28-year interval, outpatient visits for pediatric psoriasis were attended primarily by white children older than 8 years in equal number by sex. Dermatologists and pediatricians saw the majority, and treatment approach differed by physician specialty and patient age. Treatment guidelines for childhood psoriasis may help reduce treatment variability.
]]>Observations A 3-year-old Caucasian male with a history of ichthyosis since birth presented with generalized tonic seizures. Findings from a physical examination were remarkable for thin hair, retinitis pigmentosa, and poor dentition. Polarized light microscopic examination of all the hair samples demonstrated a banding pattern. Magnetic resonance imaging of the brain revealed left hemispheric polymicrogyria with decreased sulcal pattern and stable asymmetric dilation of the left lateral ventricle. Constitutional microarray revealed the typical approximately 1.5-Mb deletion of the steroid sulfatase gene.
Conclusions Steroid sulfatase deficiency is a cause of X-linked ichthyosis; however, our patient also had retinitis pigmentosa, seizures, and abnormal hair findings. The presence of abnormal hair with a banding pattern on polarized microscopy may be helpful for diagnosis; however, this pattern is not specific to this disease. In addition, to our knowledge, the presence of a malformation of cortical development has not been previously reported in patients with steroid sulfatase deficiency.
]]>Observations Twenty-one patients from 14 families with confirmed homozygous or compound heterozygous mutations in DOCK8 were evaluated. Clinical findings included dermatitis, asthma, food and environmental allergies, recurrent sinopulmonary infections, staphylococcal skin abscesses, and severe cutaneous viral infections. Malignant neoplasms, including aggressive cutaneous T-cell lymphoma, anal and vulvar squamous cell carcinomas, and diffuse large B-cell lymphoma, developed in 5 patients during adolescence and young adulthood.
Conclusions DOCK8 deficiency and Job's syndrome share several clinical features, including elevated serum IgE levels, dermatitis, recurrent sinopulmonary infections, and cutaneous staphylococcal abscesses. However, the presence of recalcitrant, widespread cutaneous viral infections, asthma, and food and environmental allergies, as well as the absence of newborn rash and coarse facies, favors the clinical diagnosis of DOCK8 deficiency. Rates of malignancy and overall mortality in patients with DOCK8 deficiency were higher than in those with Job's syndrome, highlighting the value of distinguishing between these conditions and the importance of close monitoring for neoplasia.
]]>Observations A 17-year-old girl was seen with features of almost unilateral focal dermal hypoplasia. These included left cleft hand, dental dysplasia, left mammary hypoplasia, deviation of the sacral line, raspberrylike papillomas in the perianal region, syndactyly of the second and third digits of the left foot, and linear streaks of dermal hypoplasia and pigmented lesions on her left hemibody.
Conclusions Mutation analysis of PORCN revealed a novel heterozygous mutation in exon 10, c.854-855insACCTGAC; [p.T285fsX316], resulting in a premature stop signal. Analysis of the X-chromosome inactivation status was performed on blood and skin DNA samples, showing random inactivation in blood and unaffected skin and skewed inactivation in affected skin, highlighting the role of X-chromosome inactivation in X-linked disease expression.
]]>Observations We describe a patient with nonscarring alopecia that was misdiagnosed as scarring alopecia due to difficulty in distinguishing between scarred tracts (follicular dropout) and long-persisting fibrovascular streamers. Polarized light microscopy permits us to distinguish follicular scars from fibrous streamers because the fibrous streamers are birefringent negative for collagen. The main advantages of polarized microscopy are that it is fast and cost free and can screen all sections within minutes; it is also easy to interpret for beginners because there is a built-in control of birefringent-positive dermal collagen.
Conclusion Polarized light can be used in the pathological evaluation of hair loss to distinguish between the follicular scars in scarring alopecia and the fibrovascular streamers in long-standing nonscarring alopecia.
]]>Design Retrospective population-based cohort study.
Setting The National Health Insurance Research Database of Taiwan from January 1, 1996, through December 31, 2008.
Participants A total of 12 720 patients with chronic urticaria, with long-term antihistamine use and no history of malignant tumors, autoimmune diseases, atopy, or allergic diseases.
Main Outcome Measure Relative cancer risk calculated by standardized incidence ratios.
Results There were 704 cancers among chronic urticaria patients. An increased risk of cancer (standardized incidence ratio, 2.2; 95% CI, 2.0-2.3), especially hematologic malignant tumor (4.1; 3.1-5.4), was observed. The relative risk of cancer varied by age and was highest among those aged 20 to 39 years in comparison with the general population. Most cancer cases were detected within the first year of diagnosis. The risk of non-Hodgkin lymphoma was greatest (standardized incidence ratio, 4.4; 95% CI, 3.0-6.1) among the hematologic cancers.
Conclusions Patients with chronic urticaria are at increased risk of cancer, especially hematologic malignant tumors. Further studies are needed to delineate the associations.
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