Design  We conducted a randomized, controlled trial of the effect of an electronic text-message reminder system on adherence to sunscreen application. Adherence to daily sunscreen use was evaluated using a novel electronic monitoring device.

Setting  Participants were recruited from the general community.

Participants  Seventy participants constituted a volunteer sample from the general community. The inclusion criteria required participants to be 18 years or older, to own a cellular telephone with text-message features, and to know how to retrieve text messages.

Intervention  Half of the participants received daily text-message reminders via cellular telephone for 6 weeks, and the other half did not receive reminders. The text-message reminders consisted of 2 components: a "hook" text detailing daily local weather information and a "prompt" text reminding users to apply sunscreen.

Main Outcome Measure  The primary end point of the study was adherence to sunscreen application measured by the number of days participants applied sunscreen during the 6-week study period.

Results  All 70 participants completed the 6-week study. There were no statistically significant differences in baseline characteristics between the 2 study groups. At the end of the study period, the 35 participants who did not receive reminders had a mean daily adherence rate of 30.0% (95% confidence interval, 23.1%-36.9%). In comparison, the 35 participants who received daily text-message reminders had a mean daily adherence rate of 56.1% (95% confidence interval, 48.1%-64.1%) (P < .001). Among the participants in the reminder group, 24 (69%) reported that they would keep using the text-message reminders after the study, and 31 (89%) reported that they would recommend the text-message reminder system to others. Subgroup analysis did not reveal any significant demographic factors that predicted adherence.

Conclusions  Despite awareness of the benefits of sunscreen, adherence is low, even in this population, for whom adherence was knowingly monitored. Short-term data demonstrate that using existing cellular telephone text-message technology offers an innovative, low-cost, and effective method of improving adherence to sunscreen application. The use of ubiquitous communications technology, such as text messaging, may have implications for large-scale public health initiatives.

Trial Registration  clinicaltrials.gov Identifier: NCT00535769

Design  Prospective convenience sample accrued from 2 interdisciplinary sites.

Setting  Two interdisciplinary centers for vascular anomalies in Brussels, Belgium, and Caen, France

Participants  The study population comprised 280 patients with clinical data, Doppler ultrasonograms (for 251 patients), and coagulation parameter measurements.

Main Outcome Measure  Measurement of D-dimer levels.

Results  A VM was diagnosed in 195 of 280 patients (69.6%), and 83 of them had elevated D-dimer levels; the sensitivity of D-dimer dosage was 42.6% (95% confidence interval, 35.6%-49.5%). Among the 85 patients without VM, D-dimer levels were elevated only in 3 patients; the specificity of the dosage was 96.5% (95% confidence interval, 92.5%-100%).

Conclusions  Elevated D-dimer level is highly specific for VMs (pure, combined, or syndromic), and therefore this easy and inexpensive biomarker test should become part of the clinical evaluation of vascular anomalies. It can detect hidden VMs and help differentiate glomuvenous malformation (normal D-dimer levels) from other multifocal venous lesions. Elevated D-dimer level also differentiates a VM from a lymphatic malformation. Moreover, slow-flow Klippel-Trenaunay syndrome (capillaro-lymphatico-venous malformation with limb hypertrophy) can be distinguished from fast-flow Parkes Weber syndrome (capillary malformation with underlying multiple microfistulas and limb hypertrophy). For these reasons, D-dimer level measurement is a useful complementary tool for diagnosing vascular anomalies in everyday practice.

Design  Retrospective case series of dermoscopy images with globules.

Setting  Private dermatology practices.

Participants  Patients in dermatology practices.

Intervention  Observation only.

Main Outcome Measure  Association of globule types with malignant melanoma.

Results  The presence of large globules (odds ratio [OR], 5.25) and globules varying in size (4.72) or shape (5.37) had the highest ORs for malignant melanoma among all globule types and combinations studied. Classical globules (dark, discrete, convex, and 0.10-0.20 mm) had a higher risk (OR, 4.20) than irregularly shaped globules (dark, discrete, and not generally convex) (2.89). Globules connected to other structures were not significant in the diagnosis of malignant melanoma. Of the different configurations studied, asymmetric clusters have the highest risk (OR, 3.02).

Conclusions  The presence of globules of varying size or shape seems to be more associated with a diagnosis of malignant melanoma than any other globule type or distribution in this study. Large globules are of particular importance in the diagnosis of malignant melanoma.

Design  Records review.

Setting  A network of 15 primary care skin cancer clinics across Australia.

Participants  Fifty-seven physicians performing excisions of 9417 BCCs and SCCs in a single network of 15 primary care skin cancer clinics across Australia between 2005 and 2007.

Main Outcome Measures  Rates of incomplete excision according to physician, clinic, anatomic location of the lesion, and whether a previous biopsy had been performed.

Results  Four hundred forty-three of 6881 BCCs (6.4%) and 159 of 2536 SCCs (6.3%) were excised incompletely. Incomplete BCC and SCC excisions were more frequent on the head and neck (282 of 2872 excisions [9.8%] and 97 of 861 [11.3%], respectively) than elsewhere. Ears (74 of 388 excisions [19.1%]) and nose (78 of 546 [14.3%]) had the highest rates of incompletely excised BCCs, and ears (26 of 144 excisions [18.1%]) and forehead (20 of 157 [12.7%]) had the highest rates of incompletely excised SCCs. Of all BCC excisions, 67.3% were once-off excisions with no previous biopsy, and these excisions were more likely to be incomplete (odds ratio, 1.73; 95% confidence interval, 1.36-2.20) than those with a previous biopsy. There was, however, substantial variation in frequency of incomplete excision between clinics for BCC (ranging from 3.3% to 24.7%) and SCC (ranging from 0% to 17.2%) and between physicians within clinics (BCC ranging from 0% to 31.1%, and SCC ranging from 0% to 23.5%).

Conclusions  Overall frequency of incomplete excision is low and similar to that in other reports. However, high frequency in high-risk sites, low rates of previous biopsy, and substantial variation in performance between physicians and clinics suggests there is significant opportunity to further improve health outcomes.

Design  Multicenter, double-blind, randomized, placebo-controlled clinical trial.

Setting  Academic departments of dermatology in the United States.

Participants  Forty-five individuals with chronic and severe AA affecting 50% to 95% of the scalp hair and resistant to previous therapies.

Intervention  Alefacept, a US Food and Drug Administration–approved T-cell biologic inhibitor for the treatment of moderate to severe plaque psoriasis.

Main Outcome Measure  Improved Severity of Alopecia Tool (SALT) score over 24 weeks.

Results  Participants receiving alefacept for 12 consecutive weeks demonstrated no statistically significant improvement in AA when compared with a well-matched placebo-receiving group (P  = .70).

Conclusion  Alefacept is ineffective for the treatment of severe AA.

Design  Randomized, double-blind, placebo-controlled trial using the reduction mammoplasty wound-healing model.

Setting  University Medical Center Groningen.

Patients  Thirty women who underwent bilateral reduction mammoplasty.

Interventions  For 3 months, scar segments were treated with either topical calcipotriol or placebo.

Main Outcome Measures  Three weeks, 3 months, and 12 months postoperatively, the scars were evaluated and punch biopsy samples were collected for immunohistochemical analysis.

Results  No significant difference in the prevalence of hypertrophic scars was observed between the placebo- and calcipotriol-treated scars. Only scars with activated keratinocytes 3 weeks postoperatively became hypertrophic (P = .001).

Conclusions  Topical application of calcipotriol during the first 3 months of wound healing does not affect the incidence of hypertrophic scar formation. We observed a strong association between keratinocyte activation and hypertrophic scar formation.

Trial Registration  trialregister.nl Identifier: NTR1486

Design  Interfamilial and intrafamilial observational study.

Setting  Tertiary genetic and dermatology referral center.

Participants  Thirty-four individuals recruited from 2 large multigenerational families with CYLD mutations. Clinical details, history, and tumor maps were obtained from all participants; in 18, the information was corroborated by detailed clinical examination.

Main Outcome Measures  Tumor density, distribution and histologic findings, associated medical conditions, patient symptoms, and impact of disease on quality of life.

Results  The severity of penetrance and phenotype varied within families. Although an approximately equal female to male predisposition was noted, 5 women and 1 man (of 26 patients surveyed [23%]) had undergone total scalp removal. The average age at onset was 16 years (range, 8-30 years). Symptoms reported by affected patients included painful tumors (in 12 of 23 patients [52%] who answered the question), conductive deafness, and sexual dysfunction. Of the 26 surveyed patients, tumors were noted on the scalp in 21 (81%), on the trunk in 18 (69%), and in the pubic area in 11 (42%). Tumor mapping provided clinical evidence that correlated with hormonally stimulated hair follicles being particularly vulnerable to loss of heterozygosity and tumor induction.

Conclusions  The burden of disease at sites other than the head and neck appears to be underreported in the literature and greatly affects quality of life. Differentiation between the clinical diagnoses has little prognostic or clinical utility in genetic counseling, even within individuals from the same family. Thus, we suggest an encompassing diagnosis of "CYLD cutaneous syndrome." Finally, the clinical distribution of tumors suggests that hormonal factors may play an important role in tumor induction in these patients.

Observations  We describe a boy who was found to have XP after a severe burn following minimal sun exposure. His maternal uncle, now age 20 years, had been diagnosed with XP after a similar sunburn in infancy. The uncle has the typical skin pigmentary findings of XP along with severe progressive neurologic involvement. Although the infant's parents were not known to be blood relatives, the infant and his affected uncle proved to be compound heterozygotes for the same 2 frameshift mutations in the XPA DNA repair gene (c.288delT and c.349_353del). After the diagnosis of XP in the infant, genealogic investigation identified a common Dutch ancestor for both of his grandfathers 5 generations back.

Conclusions  Counseling families at risk for a rare inherited disease is not always straightforward. The sociocultural and demographic backgrounds of the families must be considered for evaluation of risk assessment.

Observations  A 33-year-old woman with cystic fibrosis developed a papular eruption on the flexural surfaces of the upper and lower extremities, which was initially treated with prednisone. A punch biopsy showed granulomatous inflammation and associated PXE-like changes. The combined histologic and clinical findings were most consistent with granuloma annulare. There was no family history of PXE or clinical manifestations of PXE. The rash gradually resolved itself over the next several months.

Conclusions  There are few publications that document PXE-like changes in association with various inflammatory skin conditions. Thus, the clinical significance of this finding remains uncertain. This case and previous reports are discussed in the context of current molecular and genetic knowledge. It is hoped that greater awareness of this phenomenon will promote further investigation and elucidation of the clinical and biologic significance of PXE-like changes observed in biopsies of inflammatory skin disorders.

Design  Blinded comparison.

Setting  The CF and dermatology clinics at St Louis Children's Hospital.

Participants  Forty-four individuals with CF from a CF clinic and 26 controls from a dermatology clinic.

Intervention  Participants were tested for AWP using 3 minutes of water immersion with room-temperature tap water.

Main Outcome Measure  The degree of AWP was scored from 0 (no wrinkling) to 4 (severe wrinkling) by 3 blinded physicians. For genotype-phenotype correlations, patients with CF were divided into those homozygous for the F508 mutation and those with other genotypes.

Results  The mean AWP score of the CF group was significantly higher than the mean score of the control group (1.5 vs 0.6; P < .001). Patients with CF who were homozygous for the F508 mutation (n = 27) had significantly higher scores than patients with CF who were not homozygous for the F508 mutation (n = 17) (1.7 vs 1.1; P = .02). The 17 patients with CF who were not homozygous for the F508 mutation still had higher scores than the control group (1.1 vs 0.6; P = .03). There was no correlation between sweat chloride concentrations measured at the time of diagnosis and AWP score.

Conclusions  Our results confirm the association between AWP and CF. Among patients with CF, greater AWP occurs in those who are homozygous for the F508 mutation.

Observation  Seven patients were included in this retrospective study, all of whom were treated with pulsed high-dose corticosteroids combined with low-dose methotrexate for at least 6 months. The outcome measure was an individual, nonvalidated clinical score. Overall, a significant decrease in the clinical score was observed, from a median score of 8 (range, 5 to 24) before treatment to 2 (range, 1 to 4) after treatment. Adverse effects observed during therapy were moderate and disappeared after the end of treatment. During the follow-up period of at least 3 months (mean, 4.7 [range, 3-8] months), none of the patients experienced a relapse of extragenital LS.

Conclusions  Patients with severe extragenital LS benefit from pulsed high-dose corticosteroids combined with low-dose methotrexate therapy. This combination therapy should be considered in generalized disease, especially disease that is refractory to conventional treatment.

Observations  Two patients who had previously been treated with Bio-Alcamid outside of the United States presented with different manifestations of inflammatory responses to the product. These reactions were challenging to treat.

Conclusions  Despite claims of safety, Bio-Alcamid and possibly other soft-tissue fillers available worldwide have the potential to cause adverse reactions. Physicians should be aware of the various presentations and treatment options for these reactions.

Observations  A 45-year-old woman with a history of multiple dysplastic nevi and lentigines was diagnosed as having familial GIST syndrome. Treatment with imatinib mesylate was started in an attempt to decrease the tumor load. Three months after treatment initiation, the patient noted a decrease in the number of pigmented lesions, lightening of the skin in her genital area, and graying of her terminal hair.

Conclusions  The potential association between a specific genetic mutation and pigmentation changes secondary to imatinib therapy may account for the variety in presentation of this potential side effect. Further genetic studies paired with melanocyte-specific or c-kit–specific stains of affected tissue are warranted to better understand the relationship between the genetic mutation and the effect of imatinib on pigmentation.

Design  Retrospective cohort of patients exposed to gadolinium-based contrast agents (GBCAs) at a single institution during an 8-year period (January 1, 1999, to December 31, 2006), and a case series study of patients with biopsy-proven NSF.

Setting  A primary, secondary, and tertiary health care center that treated more than 2.2 million outpatients and had 135 000 hospital admissions in 2007.

Patients  A total of 94 917 patients exposed to GBCAs; patients at risk for NSF (3779 patients on hemodialysis, 1694 patients with renal transplants, and 717 patients with liver transplants, a well-defined subgroup that includes patients at risk for reduced renal function); and 61 patients with a clinical diagnosis of NSF.

Main Outcome Measure  Risk estimate for NSF.

Results  The risk of development of NSF is 1.0% for patients who undergo hemodialysis (8 of 827), 0.8% for patients with renal transplantation (4 of 527), and 0% for patients with liver transplantation at our institution (0 of 327).

Conclusions  Despite the limitations, this study, which reviewed a large number of patients who underwent intravascular GBCA injections, demonstrates a 77-fold higher risk of NSF among patients who undergo hemodialysis and a 69-fold higher risk in patients with renal transplantation. This increased risk is thought to be associated with poor clearance of most GBCAs.

Design  Prognostic study of an inception cohort.

Setting  Academic research.

Patients  Between July 1, 1999, and July 31, 2002, all patients who had primary cutaneous or mucosal melanomas that have a Breslow depth of 1.5 mm or greater, ulceration, or Clark level IV or V, or had SLN biopsies.

Main Outcome Measures  By the use of quantitative reverse transcription–polymerase chain reaction, the expression of the following was analyzed in SLNs: 2 melanocytic differentiation antigens (tyrosinase [P17646] and melanoma antigen recognized by T cells [MART-1; Q16655]) and genes involved in angiogenesis (VEGF [NM_001025366] and VEGFR2 [AF035121]), lymphangiogenesis (VEGFC [NM_005429], VEGFR3 [X68203], LYVE1 [NM_016164], and PROX1 [002763]), and invasion (uPA [NM_002658], PAI1 [NM_00602], and EMMPRIN [L10240]). Outcome measures were the association of these melanocytic differentiation antigens and angiogenesis biomarkers with clinicopathologic characteristics of patients, and an evaluation of the prognostic value for relapse-free survival and overall survival.

Results  Ninety-one patients were included, with a median follow-up period of 41 months. Micrometastases were present in 15% (14 of 91) of patients. Tyrosinase (P < .001), MART-1 (P < .001), vascular endothelial growth factor 121 (VEGF₁₂₁) (P = .007), and PAI1 (P = .02) expression was significantly associated with micrometastasis. In univariate analysis, histologic findings and tyrosinase and MART-1 expression were significantly associated with relapse-free survival. Tyrosinase and MART-1 expression was associated with overall survival. A multiple Cox proportional hazards regression model identified negative histologic findings and tyrosinase expression that exceeded 27 copies/copy of TATA box-binding protein (third quartile) as significantly associated with an increased risk of relapse or death.

Conclusions  Quantitative assessment of melanocytic differentiation antigens in SLNs, which has prognostic value, is more specific than qualitative assessment. Prognosis may be more effectively predicted by the combination of quantitative assessment of melanocytic differentiation antigens in SLNs with histologic assessment. A significant association was found between the presence of micrometastases and the expression of angiogenesis biomarkers.

Design  Biochemical analyses of human skin biopsy specimens following microdermabrasion treatment in vivo.

Setting  Academic referral center.

Participants  Volunteer sample of 40 adults, aged 50 to 83 years, with clinically photodamaged forearms.

Intervention  Focal microdermabrasion treatment with diamond-studded handpieces of varying abrasiveness on photodamaged forearms and serial biopsies at baseline and various times after treatment.

Main Outcome Measures  Quantitative polymerase chain reaction, immunohistochemistry, and enzyme-linked immunosorbent assay were used to quantify changes in inflammatory, proliferative, and remodeling effectors of normal wound healing. Type I and type III procollagen served as the main outcome marker of dermal remodeling.

Results  Coarse-grit microdermabrasion induces a wound healing response characterized by rapid increase in induction of cytokeratin 16 and activation of the AP-1 transcription factor in the epidermis. Early inflammation was demonstrated by induction of inflammatory cytokines, antimicrobial peptides, and neutrophil infiltration in the dermis. AP-1 activation was followed by matrix metalloproteinase–mediated degradation of extracellular matrix. Consistent with this wound-healing response, we observed significant remodeling of the dermal component of the skin, highlighted by induction of type I and type III procollagen and by induction of collagen production enhancers heat shock protein 47 and prolyl 4-hydroxylase. Dermal remodeling was not achieved when microdermabrasion was performed using a medium-grit handpiece.

Conclusions  Microdermabrasion using a coarse diamond-studded handpiece induces a dermal remodeling cascade similar to that seen in incisional wound healing. Optimization of these molecular effects is likely the result of more aggressive treatment with a more abrasive handpiece.

Trial Registration  clinicaltrials.gov Identifier: NCT00111254

Design  Retrospective survey.

Setting  Academic pediatric dermatology practice.

Patients  Three hundred seventy-four patients with alopecia referred from July 1, 1997, to June 31, 2007.

Main Outcome Measures  Epidemiologic data for all forms of alopecia were ascertained, such as sex, age at onset, age at the time of evaluation, and clinical diagnosis. Patients with LAHS were further studied by the recording of family history, disease associations, hair-pull test or biopsy results, hair color, laboratory test result abnormalities, initial treatment, and involvement of eyelashes, eyebrows, and nails.

Results  Approximately 10% of all children with alopecia had LAHS. The mean age (95% confidence interval) at onset differed between patients with LAHS (2.8 [1.2-4.3] years) vs patients without LAHS (7.1 [6.6-7.7] years) (P < .001), with 3 years being the most common age at onset for patients with LAHS. All but 1 of 37 patients with LAHS were female. The most common symptom reported was thin, sparse hair. Family histories were significant for LAHS (n = 1) and for alopecia areata (n = 3). In 32 of 33 patients, trichograms showed typical loose anagen hairs. Two children had underlying genetic syndromes. No associated laboratory test result abnormalities were noted among patients who underwent testing.

Conclusions  Loose anagen hair syndrome is a common nonscarring alopecia in young girls with a history of sparse or fine hair. Before ordering extensive blood testing in young girls with diffusely thin hair, it is important to perform a hair-pull test, as a trichogram can be instrumental in the confirmation of a diagnosis of LAHS.

Design  Survey of residents in November 2003.

Setting  Four US residency programs.

Participants  Medical residents in family medicine, pediatrics, obstetrics and gynecology, and internal medicine and specialists.

Main Outcome Measure  Proportion of residents reporting their current skill level for the performance of the SCE.

Results  Of 454 surveys distributed, 342 residents completed the survey (75.3% response rate). Clinical training for the SCE during residency was infrequent. During residency, 75.8% were never trained in the SCE, 55.3% never observed an SCE, and 57.4% never practiced the examination. Only 15.9% of residents reported being skilled in the SCE. However, the conduct of 4 SCEs (or slightly more than 1 per each year of residency) was associated with manifold increases in self-reported skill levels.

Conclusions  Information now collected from 7 medical schools and 4 residency programs underscores the need for more supervised opportunities to enable physicians in training to perform an SCE during routine patient examinations.

Design  A dedicated Web platform was developed to train the participants and to allow independent distant evaluations of confocal images via the Internet.

Setting  Department of Dermatology, University of Modena and Reggio Emilia, Modena, Italy.

Participants  The study population was composed of 15 melanomas, 30 nevi, and 5 Spitz/Reed nevi. Six expert centers were invited to participate at the study.

Intervention  Evaluation of 36 features in 345 confocal microscopic images from melanocytic lesions.

Main Outcome Measure  Interobserved and intraobserved agreement, by calculating the Cohen statistics measure for each descriptor.

Results  High overall levels of reproducibility were shown for most of the evaluated features. In both the training and test sets there was a parallel trend of decreasing values as deeper anatomic skin levels were evaluated. All of the features, except 1, used for melanoma diagnosis, including roundish pagetoid cells, nonedged papillae, atypical cells in basal layer, cerebriform clusters, and nucleated cells infiltrating dermal papillae, showed high overall levels of reproducibility. However, less-than-ideal reproducibility was obtained for some descriptors, such as grainy appearance of the epidermis, junctional thickening, mild atypia in basal layer, plump bright cells, small bright cells, and reticulated fibers in the dermis.

Conclusion  The standard consensus confocal terminology useful for the evaluation of melanocytic lesions was reproducibly recognized by independent observers.

Observation  In this particular case, MDCT venography demonstrated the origin, course, and relationship to adjacent anatomical structures of aberrant vessels that configure the superficial venous system with an anatomically normal and patent deep venous system. At the end of the treatment, which consisted of 8 sessions of microfoam sclerotherapy within 12 months, a considerable reduction in the number and size of the percutaneously treated aberrant veins was observed. The obvious clinical improvement was objectively demonstrated with MDCT venography, which showed clear reduction in the number and size of treated veins. Further clinical investigation performed because of isolated migraine episodes related to the sclerotherapy session revealed that the patient had a patent foramen ovale. A transcranial Doppler examination during the procedure showed middle cerebral artery bubbles, which indicated right-to-left shunt. No cerebral damage was observed in a subsequent diffusion-weighted magnetic resonance examination.

Conclusions  Microfoam sclerotherapy is an effective treatment option in patients with Klippel-Trenaunay syndrome. MDCT venography allows diagnosis of the disease, planning of therapy, and assessment of response to treatment. Although foam-induced microembolism is a common phenomenon during sclerotherapy, in this report we demonstrate that polidocanol microfoam prepared with a low-nitrogen gas mixture is safe in a patient with a patent foramen ovale.

Observations  Of 27 patients diagnosed as having adenosquamous carcinoma, 19 were men and 5 were immunosuppressed. The mean age was 74 years. The majority of tumors were located on the face and scalp (19 of 27 [70%]) or upper extremity (4 of 27 [15%]). Squamous and glandular differentiation was characteristic. Thickness of the primary lesion ranged from 1.2 to 9.2 mm, with all tumors extensively invading the reticular dermis. Perineural invasion was seen in 4 of 27 primary cases (15%). Although 3 of 6 patients treated with Mohs micrographic surgery had subsequent locoregional recurrences, there was no evidence of distant metastasis after a mean of 2.3 years of patient follow-up.

Conclusions  Adenosquamous carcinoma is best considered as a locally aggressive high-risk subtype of cutaneous squamous cell carcinoma. Tumor thickness and perineural invasion are high-risk histopathological attributes, and immunosuppression is an important clinical risk factor. Although Mohs micrographic surgery may be the best initial treatment, locoregional recurrence is common.

Observations  We describe a 94-year-old woman with IgA pemphigus with a unique immunopathologic pattern. Direct immunofluorescence microscopy revealed IgA deposits throughout the entire epidermis, with stronger staining in the upper epidermis. The autoantibodies from this patient did not show IgA or IgG reactivity with desmogleins via immunoblotting or enzyme-linked immunosorbent assay. By indirect immunofluorescence by the use of COS7 cells transfected with desmocollin 1, 2, or 3, IgA autoantibodies in a serum sample from our patient clearly reacted with all of them.

Conclusions  The pathophysiology and autoantigen profile of bullous autoimmune diseases, especially pemphigus and its subforms, are more complex than previously believed. Because pemphigus seems to be a heterogeneous disorder, further studies are needed to evaluate the complexity of the disease.

Observations  Findings from polymerase chain reaction and high-resolution capillary electrophoresis for T-cell receptor gene rearrangement analysis showed that all 6 prospectively evaluated patients (100%) with nephrogenic systemic fibrosis had detectable clonal T cells in their peripheral blood. In contrast, only 4 of the 15 control patients (27%) with chronic renal failure and none of the 12 healthy individuals analyzed in this study had evidence for T-cell clonality using the same type of examination. Clonal T-cell–positive patients with systemic sclerosis have previously been reported to better respond to extracorporeal photopheresis. However, this was not the case in 2 of our patients with nephrogenic systemic fibrosis.

Conclusion  As in systemic sclerosis, clonally expanded T-cell populations could play a critical role in the pathogenesis of nephrogenic systemic fibrosis, probably as an in vivo–activated inflammatory response to gadolinium exposure.

Observations  Four patients with NSF were treated with UV-A1 phototherapy at a tertiary medical center from 2005 through 2007. To our knowledge, it is unique to this series that all patients were receiving hemodialysis before, during, and after therapy with UV-A1. All experienced improvement in the degree of induration, and 2 experienced improvement in mobility of the hands and legs. Total treatments ranged from 22 treatments (with a cumulative dose of 1855 J/cm²) to 50 treatments (total UV-A1 exposure, 3850 J/cm²). No adverse events were observed.

Conclusions  Although no patient had complete resolution of indurated plaques, the improvement was substantial. For 2 patients, it resulted in a resumption of hand and leg mobility. As a result, UV-A1 therapy may represent a treatment for NSF when kidney transplantation is not an option or is delayed. Limitations of this study include the lack of a controlled trial, lack of quantification of gadolinium levels within tissue, and the lack of a defined grading scale for NSF severity.

Design  Prospective cohort nested within a randomized controlled trial. Skin examinations in 3 consecutive years (2004, 2005, and 2006) included full-body counts of nevi, skin color and tanning measurement using colorimetry, and hair and eye color evaluation by comparison with charts. Telephone interviews of parents provided sun exposure, sun protection, and sunburn history.

Setting  Large managed-care organization and private pediatric offices in the Denver, Colorado, metropolitan area.

Participants  A total of 131 very-light-skinned white children without red hair and 444 darker-skinned white children without red hair born in Colorado in 1998.

Main Outcome Measures  Full-body nevus counts at ages 6 to 8 years.

Results  Among very-light-skinned white children, geometric mean numbers of nevi for minimally tanned children were 14.8 at age 6 years; 18.8 at age 7 years; and 22.3 at age 8 years. Mean numbers of nevi for tanned children were 21.2 at age 6 years; 27.9 at age 7 years; and 31.9 at age 8 years. Differences in nevus counts between untanned and tanned children were statistically significant at all ages (P < .05 for all comparisons). The relationship between tanning and number of nevi was independent of the child's hair and eye color, parent-reported sun exposure, and skin phototype. Among darker-skinned white children, there was no relationship between tanning and nevi.

Conclusions  Very-light-skinned children who tan (based on objective measurement) develop more nevi than children who do not tan. These results suggest that light-skinned children who develop tans may be increasing their risk for developing melanoma later in life.

Design  Cross-sectional study.

Setting  United States.

Participants  Employees of 3647 indoor tanning facilities were contacted by telephone. Data collectors (ie, confederates) posed as prospective, fair-skinned, 15-year-old female customers who had never tanned before.

Main Outcome Measures  Confederates asked respondents about their facility's practices related to parental consent, parental accompaniment, and allowable tanning session frequency.

Results  Approximately 87% of the facilities required parental consent, 14% required parental accompaniment, 5% said they would not allow the confederate to tan owing to her age, and 71% would allow tanning every day the first week of indoor tanning. In Wisconsin, which bans indoor tanning among those younger than 16 years, 70% of facilities would not allow the confederate to tan. Multivariate analyses indicated that facilities in states with a youth access law were significantly more likely to require parental consent (P <.001) and parental accompaniment (P <.001) than those in states without a youth access law. Law was not significantly related to allowable tanning frequency (P = .81).

Conclusion  We recommend that additional states pass youth access legislation, preferably in the form of bans.

Design  Retrospective population-based analysis.

Setting  Mortality records from the Centers for Disease Control and Prevention mortality database.

Participants  Mortality records from 1979 through 2002 for persons who died of bullous disease.

Main Outcome Measures  Age-adjusted mortality rates and trends for 4 bullous disease subgroups: toxic epidermal necrolysis, pemphigoid, pemphigus, and epidermolysis bullosa.

Results  The overall age-adjusted (to the 2000 US standard population) annual mortality rate from bullous diseases of the skin was 0.103 death per 100 000. The average mortality from bullous disorders was 0.098 per 100 000 in 1979 through 1982 and remained stable at 0.099 per 100 000 during the final 4 years of the study, 1999 through 2002. Pemphigoid had a significant increase in mortality from 1979 through 2002, while pemphigus demonstrated a significant decrease in mortality. The mortality rate for toxic epidermal necrolysis was much higher among blacks (0.192 death per 100 000) than whites (0.025 per 100 000) (P < .001), with a mortality rate ratio of 7.57 (95% confidence interval, 6.97-8.21).

Conclusions  Overall mortality from bullous diseases remained stable from 1979 through 2002, although an increasing mortality from pemphigoid and a decreasing mortality from pemphigus occurred during this period. A very large racial disparity in mortality from toxic epidermal necrolysis was observed.

Design  Prospective multicenter case-control study.

Setting  Three French university hospitals.

Patients  One hundred eight patients with LE and 216 control subjects.

Intervention  Standardized questionnaire evaluating cigarette smoking and alcohol consumption.

Main Outcome Measures  The statistical significance of smoking history and alcohol consumption as associated risk factors for LE by estimating matched case-control odds ratios and their 95% confidence intervals, using multiple conditional logistic regression and the Breslow-Day test to investigate differences in quantities of cigarette and alcohol consumption.

Results  Of the LE patients, 73.1% smoked compared with 49.5% of controls, (odds ratio, 2.77; 95% confidence interval, 1.63-4.76). There was no significant difference in alcohol consumption between LE patients and controls. Among the 79 LE patients who smoked, 72 (91.1%) had started smoking before the first manifestation of LE (mean delay between initiation of smoking and first signs of LE, 14.1 years). The LE patients smoked significantly more than controls did (11.7 vs 7.0 pack-years; P = .002). The prevalence of smoking among patients who met more than 4 American College of Rheumatology (ACR) criteria and/or with antinuclear DNA antibodies was lower than the prevalence in patients who met fewer than 4 ACR criteria or than the prevalence in controls (P < .001).

Conclusions  Cigarette smoking is associated with LE, but alcohol consumption is not. The risk conferred by cigarette smoking seems highest in patients who meet fewer than 4 ACR criteria and/or who do not have antinuclear DNA antibodies.

Design  Cross-sectional study.

Setting  Dermatology outpatient clinic of a university hospital.

Participants  Of 120 patients with morphea or eosinophilic fasciitis diagnosed between December 1, 1994, and July 15, 2007, who were enrolled in the study, only 74 completed questionnaires were suitable for data analysis.

Main Outcome Measures  Self-reported responses on the Impact of Chronic Skin Diseases on Daily Life scale measure psychological distress, specifically anxiety and depressed mood.

Results  Psychological functioning was generally impaired in patients with skin disease, particularly among patients with generalized morphea and eosinophilic fasciitis. Twenty-eight patients (38%) were at risk of depression or anxiety. Higher levels of psychological distress were significantly related to greater severity of skin disease; more pain and fatigue; impact of disease on daily life; more perceived stigmatization; illness cognitions of greater helplessness; and less acceptance and less perceived social support.

Conclusions  Physical and psychosocial aspects play a substantial role in the quality of life for patients with morphea. Physicians should be encouraged to assess the physical and psychosocial factors when treating patients with sclerotic skin diseases. This approach could improve quality of life and ultimately lead to improved dermatological treatment outcomes.

Observation  We describe a 17-year-old Afghani girl who had lived in the United States for 4 years and who presented with a 6-month history of pretibial ulcerations, 9.1-kg weight loss, abdominal pain, splenomegaly, and extreme fatigue. Histopathologic examination and culture with isoenzyme electrophoresis speciation of her skin lesions confirmed the presence of L tropica. In addition, results of serum laboratory and serological studies were highly suggestive of concomitant visceral involvement. The patient was treated with a 28-day course of intravenous pentavalent antimonial compound sodium stibogluconate with complete resolution of her systemic signs and symptoms and improvement of her pretibial ulcerations.

Conclusions  This is an exceptional case in that our patient presented with disease after an incubation period of years rather than the more typical weeks to months. In addition, this patient had confirmed cutaneous involvement, as well as strong evidence of viscerotropic disease caused by L tropica, a species that characteristically displays dermotropism, not viscerotropism.

Observations  Products containing benzoyl peroxide produced an orange-brown discoloration when mixed with either sulfacetamide or dapsone.

Conclusions  Knowledge of the chemical reaction between benzoyl peroxide and sulfacetamide and dapsone will help minimize the occurrence of this interaction on our patients' skin.

Observations  We describe 2 patients with drug-induced hypersensitivity syndrome (one end of a spectrum of severe drug eruptions) who subsequently developed cutaneous CMV ulcers at unusual sites, such as the trunk; this occurrence was immediately followed by gastrointestinal manifestations, which were fatal in 1 patient. To identify factors predictive of CMV disease, we retrospectively investigated the prevalence of CMV reactivation during drug-induced hypersensitivity syndrome in 18 patients. In this analysis, patients were divided into 2 groups depending on the positivity of CMV DNA in the blood.

Conclusions  Older and male patients with antecedent high human herpesvirus 6 DNA loads are at risk for CMV disease irrespective of corticosteroid administration. A rapid reduction in white blood cell numbers is also predictive of the onset of CMV disease.

Data Sources  Bibliographical searches were conducted in the MEDLINE, EMBASE, and China National Knowledge Infrastructure (CNKI) databases without any language limitations.

Study Selection  Studies were selected when the following criteria were met: the coexistence of a study group and a control group, the reliable and nonselective use of the reference standards for the diagnosis of LP and HCV, and the proportion of events (the prevalence of HCV in patients with LP or the prevalence of LP in patients with HCV).

Data Extraction  Three investigators independently assessed abstracts for relevant studies, and 2 investigators independently reviewed all eligible studies.

Data Synthesis  Sixty-three articles entailing 7 studies were included in the meta-analysis. For the primary outcome of prevalence of events, the meta-analysis showed that there existed an important association between HCV and LP. In the comparison of the prevalence of HCV exposure among patients with LP with that of control participants, the odds ratio (OR) was 5.4 (95% confidence interval [CI], 3.5-8.3); in the prevalence of LP among patients with HCV compared with the prevalence among control participants, the OR was 2.5 (95% CI, 2.0-3.1). The subgroup analyses with geographical stratification did not show a significant association in studies from South Asia (P = .21), Africa (P = .15), and North America (P = .09), and the subgroup analyses from stratification by LP type also did not show a significant association in the isolated cutaneous type (P = .17). When strict criteria were applied, the results of sensitivity analysis remained robust.

Conclusion  Hepatitis C virus infection is associated with a statistically significant risk for development of LP, suggesting that the presence of either HCV or certain types of LP may be used as a predictive marker of the other in certain geographical regions.

Design  Retrospective analytical case series.

Setting  Private dermatology practice in Florida, from July 2005 through October 2008.

Patients  Patients with 126 melanomas, of which 51 were invasive and 75 were melanomas in situ.

Main Outcome Measures  Proportion of melanomas detected as a result of patient complaint vs proportion determined by dermatologist-conducted full-body skin examination (FBSE). As a secondary analysis, we used logistic regression odds ratios (ORs) of association to examine whether dermatologist detection rather than patient complaint was associated with detecting thinner melanomas. A post hoc analysis was performed using a thickness cutoff of 1.0 mm to define a deep melanoma.

Results  Overall, 56.3% (95% confidence interval [CI], 47.6%-65.1%) of melanomas were found by the dermatologist and were not part of the presenting complaint. Of melanomas in situ, 60.0% (95% CI, 48.7%-71.3%) were dermatologist detected. Dermatologist detection was significantly associated with thinner melanomas, with an OR of 0.42 (P = .04). We found a significant association between thinner melanomas as a group (thickness <1 mm) and dermatologist detection, with a logistic regression OR of 5.0 (95% CI, 1.0-25.3).

Conclusions  Most melanomas detected in a general-practice dermatology setting were found as a result of dermatologist-initiated FBSE, not patient complaint. We found that dermatologist detection was associated with thinner melanomas and an increasing likelihood of the melanoma being in situ.

Design  Population-based prospective study (Vitamins and Lifestyle [VITAL] cohort).

Setting  Western Washington State.

Participants  A total of 69 671 men and women who self-reported (1) intake of multivitamins and supplemental antioxidants, including selenium and beta carotene, during the past 10 years and (2) melanoma risk factors on a baseline questionnaire.

Main Outcome Measure  Incident melanoma identified through linkage to the Surveillance, Epidemiology, and End Results (SEER) cancer registry.

Results  Cox proportional hazards regression models were used to estimate multivariable relative risks (RRs) and 95% confidence intervals (CIs) for multivitamin, supplemental selenium, and supplemental beta carotene use. After adjusting for melanoma risk factors, we did not detect a significant association between multivitamin use and melanoma risk in women (RR, 1.14; 95% CI, 0.78-1.66) or in men (RR, 1.09; 95% CI, 0.83-1.43). Moreover, we did not observe increased melanoma risk with the use of supplemental beta carotene (RR, 0.87; 95% CI, 0.48-1.56) or selenium (RR, 0.98; 95% CI, 0.69-1.41) at doses comparable with those of the SUVIMAX study.

Conclusion  Antioxidants taken in nutritional doses do not seem to increase melanoma risk.

Design  Retrospective study of patients seen between the years 2004 and 2007.

Setting  Tertiary care neurofibromatosis referral clinic at St Louis Children's Hospital.

Patients  The study population comprised 110 patients who presented with CALMs and no other diagnostic features of NF1. The median number of CALMs at initial presentation was 6, while the median age of the patients was 33 months. The median age at the last follow-up examination was 76.5 months.

Main Outcome Measures  Number and morphologic appearance of CALMs and diagnosis of NF1.

Results  Thirty-four of the children met diagnostic criteria for NF1 during the study period. Thirty-two children met criteria prior to age 72 months, and 2 children met criteria after 72 months. The mean number of CALMs at presentation in children eventually diagnosed as having NF1 (11.8 CALMs) was significantly higher than the mean number of CALMs in children not diagnosed as having NF1 (4.6 CALMs). Of the 44 children who had 6 or more typical CALMs at presentation, 34 children met criteria for NF1. Sixty-eight patients had CALMs described as "typical," while 42 patients had "atypical" CALMs. Only 2 patients with atypical CALMs met criteria for NF1.

Conclusion  The majority of patients with 6 or more CALMs will eventually meet diagnostic criteria for NF1, typically by age 6 years, and this likelihood increases with increasing number and typical morphologic appearance of CALMs.

Design  Retrospective inception cohort study.

Setting  Two departments in tertiary teaching hospitals.

Patients  All patients diagnosed as having dermatomyositis (DM) according to the criteria of Bohan and Peter seen during a 13-year period.

Main Outcome Measures  Cumulative incidence rates of herpesvirus infections using the Kaplan-Meier method and risk factors for herpesvirus infections during the first year of DM using Cox proportional hazards models.

Results  A total of 121 patients met the inclusion criteria (mean [SD] age, 52 [15] years; 85 were women [70%]). Seventy-six percent had primary dermatomyositis, and 24% had dermatomyositis associated with a malignant neoplasm. The mean (SD) duration of follow-up was 42 (33) months. During follow-up, 20 patients developed a total of 22 herpesvirus infections (16 developed herpes zoster infections). The incidence rates for herpesvirus and for herpes zoster infections were 49 and 33 episodes per 1000 patient-years, respectively. In multivariate analysis, a positive association was noted between the risk of herpesvirus infection and use of systemic corticosteroid therapy (hazard ratio [HR], 3.71 [95% confidence interval {CI}, 1.02-13.41]; P = .04), lymphocyte count lower than 6000/µL (HR, 3.55 [95% CI, 1.00-12.65]; P = .05), and creatine phosphokinase level higher than 300 U/L (HR, 4.81 [95% CI, 1.28-18.06]; P = .02). Dermatomyositis associated with a malignant neoplasm tended to be negatively associated with the risk of herpesvirus infection (HR, 0.16 [95% CI, 0.02-1.29]; P = .08).

Conclusions  The risk of serious herpesvirus infections in patients with DM is high. Educational strategies and studies evaluating the risk-to-benefit and the cost-to-benefit balances of a prophylaxis with valacyclovir hydrochloride in selected patients with DM are warranted.

Design  The Australian Longitudinal Study on Women's Health was designed to investigate multiple factors affecting the health and well-being of women over a 20-year period. Data from 3 surveys (conducted in 2000, 2003, and 2006) were analyzed. Multivariate longitudinal generalized estimating equation models, with and without time lag, were used to determine significant factors associated with skin disease (including anxiety, depressive symptoms, and stress).

Setting  An Australian community-based study.

Participants  Women, aged 22 to 27 years at the time of the first survey, were randomly selected from the Australian National Medicare database. Participant numbers for the surveys from the years 2000, 2003, and 2006 were 9688, 9081, and 8910, respectively.

Main Outcome Measures  Outcome measures were the scores from the Center for Epidemiologic Studies Depression Scale, the Perceived Stress Questionnaire for Young Women, and an item to elicit reporting of anxiety symptoms.

Results  Of 6630 women providing data on skin diseases on all 3 surveys, 8.0% (n = 523) reported having skin problems on all 3 occasions; 12.1% (n = 803) on 2 occasions; and 23.9% (n = 1582) on 1 occasion. On the 2000, 2003, and 2006 surveys, prevalence of skin problems was 24.2%, 23.9%, and 24.3%, respectively. In the generalized estimating equation models, depression symptoms and stress (but not anxiety) were significantly associated with skin problems (P < .005).

Conclusion  The findings of this relationship of depression and stress to skin disease may have considerable clinical implications, including implications for adjunctive psychological interventions in the management of patients with skin disease.

Observations  A 41-year-old man sought treatment for cutaneous and bone marrow sarcoidosis resulting in fatigue, anemia, and leukopenia refractory to conventional therapies and mycophenolate mofetil. We initiated combination immunosuppressive therapy with methotrexate sodium and mycophenolate mofetil, which resulted in a safe and prolonged quiescence of cutaneous disease and resolution of anemia and leukopenia throughout a 34-month follow-up period.

Conclusions  We present this case to highlight the growing body of evidence supporting combination immunosuppressive therapy to treat refractory sarcoidosis. In our patient, sarcoidal bone marrow involvement responded dramatically to a combined regimen of methotrexate and mycophenolate mofetil with no significant adverse effects, despite previously having been refractory to conventional agents and mycophenolate mofetil alone. This report provides evidence that combination immunosuppressive therapy is a potential treatment of refractory bone marrow sarcoidosis and highlights important issues about combined immunosuppressive therapy.

Observations  We describe a 39-year-old woman with XP who developed 38 primary melanomas along with 6 squamous cell carcinomas and 70 basal cell carcinomas over a 23-year period. During this time, a 3-fold management approach of total-body cutaneous examination, total-body photography, and dermoscopy was used in the care of the patient. The thickest melanoma had a Breslow thickness of 1.07 mm, and the mean Breslow thickness of her detected melanomas was 0.18 mm. The ratio of benign to malignant biopsied suspicious melanocytic lesions during 23 years of follow-up was 0.9:1. All melanomas were treated using wide local excision, and she had no evidence of local or in-transit metastases of any of her malignant neoplasms at the most recent follow-up examination.

Conclusion  Monthly follow-up using total-body cutaneous examinations, total-body photography, and dermoscopy is an important 3-fold secondary management technique for this unique patient, allowing early detection of her melanomas.

Observations  A 43-year-old woman with multiple sclerosis treated with subcutaneous interferon beta presented with right lower abdominal quadrant pain, fever, and an indurated McBurney point. An abdominal computed tomographic scan showed an inflammatory subcutaneous fat infiltration reaching the surface of the right lateral rectus muscle. The patient was brought to the operating room, where a laparoscopic appendectomy was performed. She returned a week later unimproved. The infiltration near a site of subcutaneous injection progressed with areas of liquefaction. Histologic examination of a deep cutaneous biopsy specimen revealed a septal panniculitis without vasculitis.

Conclusions  Panniculitides encompass various clinical syndromes characterized by inflammation of the fibrous septae, fatty lobules, or both components of the subcutaneous tissue. Interferon beta-1b should be considered among the list of putative agents.

Observations  Sixty-two consecutive patients with cGVHD following allogeneic hematopoietic stem cell transplantation were evaluated for sclerotic skin disease. Forty-four patients (71%) had cutaneous cGVHD, and 28 patients (45%) had evidence of sclerotic involvement based on physical examination findings. Fifteen patients agreed to undergo research magnetic resonance imaging to evaluate quantifiable changes in the dermis, subcutaneous tissue, and muscle. Among 15 patients, magnetic resonance imaging identified abnormalities in the skin in 7 (47%), subcutaneous fibrous septa in 13 (87%), deep fascia in 12 (80%), epimysium in 9 (60%), and muscle in 3 (20%).

Conclusions  Magnetic resonance imaging should be considered in the evaluation of patients with cGVHD suspected of having subcutaneous or fascial involvement. Additional studies are needed to validate this noninvasive modality for serial monitoring of disease activity and response to therapy.

Trial Registration  clinicaltrials.gov Identifier: NCT00331968

Observations  We describe the occurrence of cutaneous hyperpigmentation after photosensitivity in 2 patients who were treated with vandetanib. The pigmentation patterns were variable within and between patients. Biopsy specimens from different sites revealed variability in Perls and Fontana staining patterns.

Conclusions  These 2 cases highlight the unusual occurrence of cutaneous hyperpigmentation after vandetanib-associated photosensitivity, a reaction that demonstrates that medications are important causes of acquired photosensitivity and hyperpigmentation. Aggressive photoprotection may facilitate the resolution of diffuse hyperpigmentation. Dermatologists should endeavor to identify and report novel cutaneous adverse effects as new targeted therapies are developed.