Treatment of pseudoxanthoma elasticum-like papillary dermal elastolysis with nonablative fractional resurfacing laser resulting in clinical and histologic improvement in elastin and collagen.

OBJECTIVE: Pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE) is a rare skin condition seen predominantly in elderly females. These asymptomatic lesions are brought to the dermatologist's attention due to patient's displeasure in their appearance. We report a case of a 28-year-old female with PXE-PDE on the right neck treated with nonablative fractional resurfacing (NAFR). CASE: The patient on examination had yellow-to-skin-colored papules that coalesced into a cobblestone-appearing plaque on her lateral neck. The area was treated with NAFR to improve texture and cosmesis. After three treatments, the patient noted at least a 50% improvement of appearance and texture in the affected skin. Adverse effects were mild without development of serious side effects, such as scarring and/or permanent dyspigmenation. CONCLUSION: Established therapies for elastin disorders like PXE-PDE have yielded unsatisfactory results by patient and clinician standards. Positive results with NAFR, illustrated in our PXE-PDE case, further provides a role for NAFR in elastin disorders.

Acquired brachial cutaneous dyschromatosis in a 60-year-old male: a case report and review of the literature.

Acquired brachial cutaneous dyschromatosis is an acquired pigmentary disorder that has been described in only 20 patients but likely affects many more. This case of a man with acquired brachial cutaneous dyschromatosis is unique as most reports are in women. We report the case of a 60-year-old male who presents with an asymptomatic eruption characterized by hyperpigmented and telangiectatic macules coalescing into patches on the bilateral extensor aspects of the forearms which is consistent clinically and histopathologically with acquired brachial cutaneous dyschromatosis. Given its presence in patients with clinical evidence of chronic sun exposure and its histopathological finding of solar elastosis, acquired brachial cutaneous dyschromatosis is likely a disorder caused by cumulative UV damage. However, a possible association between angiotensin-converting enzyme inhibitors and acquired brachial cutaneous dyschromatosis exists. Further investigation is needed to elucidate both the pathogenesis of the disorder and forms of effective management. Treatment of the disorder should begin with current established treatments for disorders of dyspigmentation.

Characterization of clinical photosensitivity in cutaneous lupus erythematosus.

BACKGROUND: Photosensitivity (PS) in lupus erythematosus (LE) is frequently determined by patient report. OBJECTIVE: We sought to characterize self-reported PS in cutaneous LE (CLE). METHODS: The PS survey was used to classify subject responses into 5 phenotypes: direct sun-induced CLE flare (directCLE); general exacerbation of CLE (genCLE); polymorphic light eruption-like reactions (genSkin); general pruritus/paresthesias (genRxn); and sun-induced systemic symptoms (genSys). In all, 91 subjects with CLE alone or with CLE and systemic LE were interviewed. RESULTS: In all, 81% ascribed to 1 or more PS phenotypes. CLE-specific reactions (direct sun-induced CLE flare or general exacerbation of CLE) were reported by 86% of photosensitive subjects. Higher CLE disease activity (measured by CLE Disease Area and Severity Index activity scores) was suggestive of direct sun-induced CLE flare reactions (P = .09). In all, 60% of photosensitive subjects described CLE-nonspecific reactions: polymorphic light eruption-like rash and general pruritus/paresthesias. These phenotypes often co-occurred with CLE-specific reactions and were predicted by more systemic disease activity as measured by Physicians Global Assessment (PGA) scores in regression analyses (genSkin, P = .02) and (genRxn, P = .05). In all, 36% of subjects reported systemic reactions and higher PGA scores were predictive of the sun-induced systemic symptoms phenotype (P = .02); a diagnosis of systemic LE was not (P = .14). LIMITATIONS: PS was inferred from patient report and not directly observed. CONCLUSIONS: Characterization of self-reported PS in LE reveals that patients experience combinations of CLE-specific, CLE-nonspecific, and systemic reactions to sunlight. Sun-induced CLE flares are associated with more active CLE disease. Polymorphic light eruption-like, generalized pruritus/paresthesias, and systemic reactions are associated with more active systemic disease. Recognition of PS phenotypes will permit improved definitions of clinical PS and allow for more precise investigation into its pathophysiology.

Impact of smoking in cutaneous lupus erythematosus.

OBJECTIVE: To investigate cigarette smoking in cutaneous lupus erythematosus (CLE). 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.

Prevalence of self-report photosensitivity in cutaneous lupus erythematosus.

BACKGROUND: Little is known about the prevalence of self-reported photosensitivity (PS) and its effects on quality of life in a US cutaneous lupus population. OBJECTIVE: We sought to determine the prevalence of self-reported PS among a cutaneous lupus population and to examine its impact on quality of life. METHODS: A total of 169 patients with lupus were interviewed about PS symptoms and completed the modified Skindex-29+3, a quality-of-life survey. A complete skin examination was conducted and the Cutaneous Lupus Erythematosus Disease Area and Severity Index was completed. RESULTS: In all, 68% of patients reported some symptoms of PS. The PS group (those who reported a history of and current PS) scored worse on PS-related items of the modified Skindex-29+3 and had higher cutaneous disease activity as determined by the Cutaneous Lupus Erythematosus Disease Area and Severity Index. Patients with PS had worse symptoms and emotions and experienced significant functional impairments compared with patients who had cutaneous lupus without PS. LIMITATIONS: This study was done at a single referral center. CONCLUSIONS: Self-reported PS is very common among patients with cutaneous lupus and is associated with significant impairments related to symptoms, emotions, and daily functioning.

Quality of life in dermatomyositis.

BACKGROUND: Quality of life (QoL) for patients with inflammatory skin disease can be significant, but has been evaluated in just one study in dermatomyositis (DM). OBJECTIVE: We sought to examine the relationship between the Cutaneous Dermatomyositis Area (CDASI) and Severity Index, a DM-specific cutaneous severity instrument, and various QoL study instruments and to determine the impact of DM on QoL. METHODS: Skin-specific QoL instruments, the Skindex and the Dermatology Life Quality Index, and global medical QoL instruments, the Short Form 36 and the Health Assessment Questionnaire-Disability Index, were used. Pruritus was evaluated by a visual analog scale and a 0-to-10 scale in DM and cutaneous lupus erythematosus (CLE) populations, respectively. RESULTS: There was a significant correlation between the CDASI and all skin-specific QoL scores (lowest P = .0377). Using the Short Form 36, DM population was found to have significantly worse QoL scores than the general population with the exception of bodily pain (all subscore P values < .01). Furthermore, DM had a significantly lower vitality score, representing energy level, compared with CLE, hypertension, diabetes, and recent myocardial infarction scores (lowest P = .003). There was a significantly lower mental health score, representing overall mood, to all compared diseases except CLE and clinical depression (P values < .01 when significant). We found that DM produces more pruritus than CLE (P < .0001). LIMITATIONS: A larger patient population needs to be studied to further assess QoL in patients with DM. CONCLUSION: We conclude that DM has a large impact on QoL, even when compared with other diseases, and that DM skin disease activity correlates with a poorer QoL.

Response to antimalarial agents in cutaneous lupus erythematosus: a prospective analysis.

OBJECTIVE: To demonstrate response to antimalarial agents in patients with cutaneous lupus erythematosus (CLE) using activity scores from the Cutaneous Lupus Erythematosus Disease Area and Severity Index, a validated outcome measure. DESIGN: Prospective, longitudinal cohort study. SETTING: University cutaneous autoimmune disease clinic. PARTICIPANTS: A total of 128 patients with CLE who presented from January 2007 to July 2010 and had at least 2 visits with activity scores. INTERVENTION: Administration of antimalarial agents. MAIN OUTCOME MEASURES: Response was defined by a 4-point or 20% decrease in activity score. Response to initiation was determined by the difference between the scores before treatment and at the first visit at least 2 months after treatment. Response to continuation was determined by the difference between the scores at the first visit and the most recent visit while undergoing treatment. RESULTS: Of 11 patients who initiated treatment with hydroxychloroquine, 55% were responders (n = 6), showing a decrease in median (interquartile range [IQR]) activity score from 8.0 (3.5-13.0) to 3.0 (1.8-7.3) (P = .03). Of 15 patients for whom hydroxychloroquine failed, 67% were responders to initiation of hydroxychloroquine-quinacrine therapy (n = 10), showing a decrease in median (IQR) activity score from 6.0 (4.8-8.3) to 3.0 (0.75-5.0) (P = .004). Nine of 21 patients who continued hydroxychloroquine treatment (43%), and 9 of 21 patients who continued hydroxychloroquine-quinacrine (43%) were responders, showing a decrease in median (IQR) activity score from 6.0 (1.5-9.5) to 1.0 (0.0-4.5) (P = .01) and 8.5 (4.25-17.5) to 5.0 (0.5-11.5) (P = .01), respectively. CONCLUSIONS: The use of quinacrine with hydroxychloroquine is associated with response in patients for whom hydroxychloroquine monotherapy fails. Further reduction in disease activity can be associated with continuation of treatment with antimalarial agents.

Percutaneous transluminal angioplasty balloon inflation with syringes: who needs an inflator?

PURPOSE: High pressures are important in percutaneous transluminal angioplasty, particularly in hemodialysis access. Although many operators rely on commercially available inflators, small syringes can deliver high pressures, which might make inflators unnecessary. Recently introduced polycarbonate (PC) syringes may allow higher pressure delivery than traditional polypropylene (PP) ones, so the present study was performed to determine predictors of inflation pressures achievable with various syringe designs. MATERIALS AND METHODS: Forty-two subjects participated: 12 residents, 12 interventional radiology fellows, 12 interventional radiology attending physicians, and six others. Each subject made three attempts to deliver maximum pressure with use of a calibrated gauge for each syringe type (1-, 3-, 5-/6-, 10-, and 20-mL PP and PC syringes). A control experiment was conducted to assess fatigue effects. Age, sex, grip strength measured on a dynamometer, and physical parameters (height, weight, body mass index [BMI]) were collected for each participant. RESULTS: Pressures of 40 atm or greater were achieved by more than 90% of subjects with 1-mL PC syringes (mean, 47 atm +/- 7 [SD]). Female subjects generated lower mean pressures with every syringe type (P value range, <.0001 to .025); this effect was least pronounced with 1-mL PC syringes. Grip strength was highly correlated with pressure generation across syringe types. Control experiments showed no significant fatigue effect. With rare exceptions, age, BMI, level of training, height, and weight did not correlate with pressure generation. CONCLUSIONS: Irrespective of sex, grip strength, and BMI, 1-mL PC syringes can be used to generate pressures in excess of 40 atm. High correlation between grip strength and pressure generation may make it possible to predict the pressure an individual can generate with any syringe size or design.