Longitudinal Study of Pediatric Urticaria Pigmentosa

Abstract

Background/Objectives

Urticaria pigmentosa (UP) is the most common form of mastocytosis in children and is associated with systemic signs, symptoms, and triggers. To our knowledge, the effect of UP on children's quality of life has not been studied. The objective of the current study was to characterize the natural history, triggers, and complications of pediatric UP, identify prognostic indicators, and determine its effect on quality of life.

Methods

Between 2002 and 2007, children with three or more mastocytomas diagnosed by a pediatric dermatologist were recruited during visits at the Children's Hospital of Wisconsin Dermatology Clinic (Milwaukee, WI). Research visits were conducted every 3 years and telephone interviews yearly. The Children's Dermatology Life Quality Index was administered to subjects 4 years of age and older at enrollment. Laboratory test results were collected for subjects younger than 4 years at enrollment. Subjects were followed until UP resolution or study end in August 2015.

Results

The final cohort size was 43 subjects followed for a median of 8.1 years. Twenty-six subjects were followed through study completion. At age 12 years, 6 patients had disease resolution and 14 remained active. Patients who had disease resolution before age 12 years were more likely to be male and had fewer years of age and smaller lesions, fewer affected areas, and earlier onset. Common medications and anesthetics resulted in no serious reactions. Hymenoptera stings occurred in 51%, with no reports of anaphylaxis. No patient reported a severe effect on quality of life, with most indicating mild to no effect.

Conclusion

Severe complications are not common with historically identified triggers. Disease does not resolve before adolescence in most children. UP has a minimal effect on quality of life for most children.

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Use of Phototherapy in Children

Abstract

Background

Phototherapy is a well-recognized treatment in adults and children. Previous articles have reported success in treating recalcitrant skin disorders such as atopic dermatitis (AD), psoriasis, pityriasis lichenoides chronica, and vitiligo in children.

Methods

This was a retrospective review over an 18-month period from June 2012 to December 2013 of all children receiving phototherapy in a tertiary pediatric dermatology center.

Results

Seventy-five patients 3 to 17 years of age (mean 10.6 years; 35 male, 40 female) were included. Forty-eight (64%) patients had AD and 21 (28%) had psoriasis. Seventy received narrowband ultraviolet B (NBUVB) treatment and five received hand and foot psoralen and ultraviolet A (PUVA) treatment. All patients with AD were treated with NBUVB and four (8.3%) were also treated with hand PUVA. After phototherapy, 76% had documented clear to almost clear skin. At the 12-month follow-up, 52% of the patients with AD remained clear. All 21 patients with psoriasis underwent NBUVB phototherapy. The clearance rate after phototherapy was 86%. At the 12-month follow-up, 43% of the patients with psoriasis remained clear.

Conclusion

Phototherapy can reduce disease burden in individuals with severe AD and psoriasis and should be considered as a second-line therapy if standard topical regimens are unsuccessful.

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Issue Information

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Updates in wound healing: Mechanisms and translation

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6th Conference of the European Hidradenitis Suppurativa Foundation E.V.

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Clinical Snippets

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Photodynamic therapy with intradermal application of 5-aminolevulinic acid successfully improved tumor lesions of mycosis fungoides

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Dapsone and sulfasalazine combination therapy in dermatitis herpetiformis

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Idiopathic Pulmonary Hemosiderosis in a Child with Recurrent Macrophage Activation Syndrome Secondary to Systemic Juvenile Idiopathic Arthritis

Macrophage activation syndrome, a severe complication of systemic juvenile idiopathic arthritis and other inflammatory diseases, represents one of the most important rheumatological emergencies. Delayed diagnosis could lead to life-threatening complications. Pulmonary hemosiderosis has been classically characterized by a triad of anemia, hemoptysis, and lung infiltrates on chest radiogram. Although the majority of patients of pulmonary hemosiderosis are considered idiopathic, secondary hemosiderosis associated with known diseases could be seen. In this case report, we aimed to present gradually increased pulmonary manifestations due to pulmonary hemosiderosis with recurrent macrophage activation syndrome attacks in a child with systemic juvenile idiopathic arthritis.

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The effects of hydroporation on melasma with anti-aging cocktail

Summary

Background

Jet-M is a device for epidermal peeling and is used to deliver substances by spraying air and microdroplets. Previously, a case was treated with a mixed solution of copper-GHK, oligo-hyaluronic acid, Rhodiola extract, tranexamic acid, and β-glucan. The results showed significant improvement of aged skin.

Aims

This study was conducted to evaluate the effects of hydroporation on melasma with the formulation in a small group of volunteers.

Methods

Clinical effects were evaluated by both subjective and objective methods including melanin index (MI) and erythema index (EI) measurement.

Results

Clinically, pigmentation and erythema were relieved and also both MI and EI decreased. Histopathologic observation revealed that type IV collagen and procollagen were increased in the upper dermis. Furthermore, the number of p63-positive cells is increased along the basement membrane. These results all suggest that hydroporation with GHR formulation induced anti-aging effects by reconstruction of extracellular matrix.

Conclusion

These findings suggest that the treatment may have depigmenting effects and erythema decreasing effects by enhancing the microenvironment of the skin.

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Acne RA-1,2, a novel UV-selective face cream for patients with acne: Efficacy and tolerability results of a randomized, placebo-controlled clinical study

Summary

Background

General skincare measures such as the use of moisturisers and products containing adequate photoprotection are important components of acne patients' management to complement the pharmacological regimen. Acne RA-1,2 is a novel dermato-cosmetic product which contains selective photofilters and active ingredients against the multifactorial pathophysiology of acne.

Objectives

To evaluate the tolerability of Acne RA-1,2 and its effect on the clinical signs of acne.

Methods

This double-blind, placebo-controlled study randomized 40 adult patients with 10-25 comedones per half face to once-daily application of Acne RA-1,2 or placebo for 8 weeks. Evaluations after 4 and 8 weeks included the number of comedones, transepidermal water loss (TEWL), sebum production, and tolerability.

Results

In the Acne RA-1,2 group, there was a significant 35% decrease in the mean number of comedones from 26 at baseline to 17 at Week 8 (P<.001), a 7% significant reduction in TEWL (9.32 to 8.66 g/h/m²; P<.001), and a 24% significant reduction in sebum production (154.8 to 117.6 μg/cm²; P<.001). The reductions in TEWL and sebum production were significantly greater than those in the placebo group at Weeks 4 and 8 (P<0.05). There were no adverse events.

Conclusions

Acne RA-1,2 was well tolerated and effective at reducing comedones and sebum production and improving epidermal barrier function. These results suggest that Acne RA-1,2 is useful against acne-prone facial skin, particularly as it targets sebum production, which topical pharmacological acne therapies do not address.

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Eosinophilic Angiocentric Fibrosis as a Stenosing Lesion in the Subglottis

Subglottic Eosinophilic Angiocentric Fibrosis (EAF) is an extremely rare disease of an elusive aetiology. It is chronically progressive benign condition that causes narrowing of the subglottic region leading to dysphonia and airway compromise. The diagnosis is historical and imaging is nonspecific. We report a case xc of 56-year-old lady referred to our institution with globus sensation, hoarseness, and mild stridor. Incidental subglottic mass was found at time of diagnostic microlaryngoscopy and biopsy confirmed subglottic EAF. All laboratory investigations were unremarkable. Lesion was removed with laryngeal microdebrider and three courses of intravenous dexamethasone were administered. Patient's postoperative period was uneventful and had remained disease free for 1 year. To date, no consensus has been reached on the optimal treatment of subglottic EAF. We recommend regular follow-up to detect early recurrence.

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Lichen myxedematosus: toward established classification and diagnostic criteria

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Quantitative computed tomography imaging-based clustering differentiates asthmatic subgroups with distinctive clinical phenotypes

Publication date: Available online 29 January 2017
Source:Journal of Allergy and Clinical Immunology
Author(s): Sanghun Choi, Eric A. Hoffman, Sally E. Wenzel, Mario Castro, Sean Fain, Nizar Jarjour, Mark L. Schiebler, Kun Chen, Ching-Long Lin
BackgroundImaging variables including airway diameter, wall thickness and air-trapping have been found to be important metrics when differentiating severe asthmatics from nonsevere asthmatics and healthy subjects.ObjectiveThe objective of this study was to identify imaging-based clusters and to explore the association of the clusters with existing clinical metrics.MethodsWe performed an imaging-based cluster analysis using quantitative computed tomography-based structural and functional variables extracted from the respective inspiration and expiration scans of 248 asthmatics. The imaging-based metrics included a broader-set of multiscale variables such as inspiratory airway dimension, expiratory air-trapping and registration-based lung deformation (inspiration vs. expiration). Asthma subgroups derived from a clustering method were associated with subject demography, questionnaire, medication history, and biomarker variables.ResultsCluster 1 patients were early-onset younger nonsevere asthmatics with reversible airflow obstruction, who showed normal airway structure; Cluster 2 patients were a mix of nonsevere and severe asthmatics with marginal inflammation, who exhibited airway luminal narrowing without wall thickening. Cluster 3 and 4 patients were dominated by severe asthmatics. Cluster 3 patients were obese females with reversible airflow obstruction who exhibited airway wall thickening without airway narrowing. Cluster 4 patients were late-onset older males with persistent airflow obstruction, exhibiting significant air-trapping and reduced regional deformation. Clusters 3 and 4 patients also showed decreased lymphocyte and increased neutrophils, respectively.ConclusionsFour image-based clusters were identified and shown to be correlated with clinical characteristics. Such clustering serves to differentiate asthma subgroups which may be used as a basis for the development of new therapies.

Teaser

We identified four asthma subgroups using a cluster analysis composed of imaging variables, which were associated with clinical metrics. Identifying imaging-based clusters could enable practical cluster-based therapeutic interventions.


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Role of high flow nasal oxygen in the management of a case of anaesthesia related aspiration pneumonia

Regurgitation and pulmonary aspiration of gastric contents is a feared complication of anaesthesia resulting in serious sequelae including acute hypoxemic respiratory failure (AHRF). Early application of non-invasive positive pressure ventilation (NIV) decreases the rate of endotracheal intubation and ICU mortality in AHRF. However, NIV is often complicated by high airway pressures predisposing to mask leaks, discomfort and device intolerance [1]. Recently high-flow oxygen delivered through nasal cannula (HFNO) has emerged as an alternative to NIV.

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Cervical plexus block for perioperative analgesia during otoplasty

To the Editor:

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Pharmacokinetics and pharmacodynamics of cisatracurium in patients undergoing surgery with two hemodilution methods

To investigate the pharmacokinetics and pharmacodynamics of cisatracurium in patients undergoing surgery under acute normovolemic hemodilution (ANH) and acute hypervolemic hemodilution (AHH).

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Effects of stylet use during tracheal intubation on postoperative pharyngeal pain in anesthetized patients: A prospective randomized controlled trial

This study aimed to compare the impact of stylet application for tracheal intubation for postoperative pharyngeal pain or hoarseness in patients undergoing elective surgery.

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Subclinical atherosclerosis in Systemic Lupus Erythematosus: Comparable risk with Diabetes Mellitus and Rheumatoid arthritis

Publication date: Available online 29 January 2017
Source:Autoimmunity Reviews
Author(s): Maria G. Tektonidou, Evrydiki Kravvariti, George Konstantonis, Nicholas Tentolouris, Petros P. Sfikakis, Athanasios Protogerou
ObjectiveAlthough a high risk of clinical and subclinical atherosclerosis has been reported in systemic lupus erythematosus (SLE), it is not directly compared with that observed in other rheumatic and non-rheumatic high-cardiovascular (CVD) risk diseases, such as Rheumatoid Arthritis (RA) and Diabetes Mellitus (DM). Our objective was to evaluate the relative risk (RR) of subclinical atherosclerosis in SLE, RA and DM patients compared to healthy controls, and examine potential associations with traditional and disease-related CVD risk factors in SLE.MethodsWe examined for atherosclerotic plaques 460 individuals (92% female) without CVD history, using carotid and femoral artery ultrasound: 115 SLE patients and matched 1:1 for age and gender RA, DM, and control subjects. Multivariate models were used to determine relative risk estimates for the number of atherosclerotic plaques in patient groups versus controls, and associations of plaques with traditional CVD and disease-related factors in SLE.ResultsA nearly two-fold higher number of atherosclerotic plaques in the carotid and femoral arteries was detected in each of SLE, RA and DM groups compared to controls, after adjusting for the effect of traditional CVD risk factors (RR=1.80, 95% CI 1.05–3.08, p=0.033, RR=1.90 (1.11–3.26), p=0.019, RR=1.93 (1.14–3.28), p=0.015, respectively). In SLE patients, the number of atherosclerotic plaques was associated with age (p<0.001), smoking (p=0.016), hypertension (p=0.029), and cumulative corticosteroid dose (p=0.007).ConclusionThe relative risk of subclinical atherosclerosis in SLE was comparable to that found in RA and DM, indicating that SLE patients merit a similar diligence in CVD risk assessment and management measures.



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Efficacy of belimumab on renal outcomes in patients with systemic lupus erythematosus: A systematic review

Publication date: Available online 29 January 2017
Source:Autoimmunity Reviews
Author(s): S Sciascia, M Radin, J Yazdany, RA Levy, D Roccatello, M Dall'Era, Maria J Cuadrado
Both BLISS-52 and BLISS-76 international phase III trials in Systemic Lupus Erythematosus (SLE) met their primary outcomes; however, they were not designed to assess the efficacy of belimumab for the treatment of lupus nephritis (LN). LN is a frequent cause of SLE-associated morbidity and mortality, and emerging evidence suggests a potential therapeutic role for agents that target B lymphocyte stimulator (BLyS).We conducted a systematic review to identify data on the effect of belimumab on LN.A total of 2004 patients with SLE were identified from 11 studies. Three hundred and twenty-six patients had LN at baseline and 234 (71.8%) of those received belimumab. Thirteen patients out of 234 (5.5%) received belimumab for active LN. Due to the heterogeneous definitions of treatment response, clinical presentation and renal involvement, it was not possible to compare results using a single outcome parameter. However, the majority of these studies defined clinical response in terms of rates of renal flare, renal remission, and/or renal organ disease improvement. One hundred twenty-nine (55.1%) of the 234 patients with LN at baseline showed an improvement in renal parameters after treatment with belimumab. In patients with baseline proteinuria >0.2g/24h, (n=687), those receiving belimumab had a median reduction in proteinuria during follow-up as high as 38%. When focusing on patients with proteinuria ≥1g/24h (n=228), 70.7% of those treated with belimumab (n=157) achieved a renal response.In the pooled population of patients receiving belimumab, we found an overall annual renal flare rate of 1.7% [24/1448, mean observation time 1,1years (0,5–3)].Despite the limitations of the studies included in this analysis, available data are promising and provide preliminary support for targeting BlyS to induce or maintain a renal response. Further trials should examine whether belimumab (alone or following rituximab) represents an additional therapeutic option in the treatment of LN.



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Clinical follow-up predictors of disease pattern change in anti-Jo1 positive anti-synthetase syndrome: Results from a multicenter, international and retrospective study

Publication date: Available online 29 January 2017
Source:Autoimmunity Reviews
Author(s): Elena Bartoloni, Miguel A. Gonzalez-Gay, Carlo Scirè, Santos Castaneda, Roberto Gerli, Francisco Javier Lopez-Longo, Julia Martinez-Barrio, Marcello Govoni, Federica Furini, Trinitario Pina, Florenzo Iannone, Margherita Giannini, Laura Nuño, Luca Quartuccio, Norberto Ortego-Centeno, Alessia Alunno, Christopher Specker, Carlomaurizio Montecucco, Konstantinos Triantafyllias, Silvia Balduzzi, Walter Alberto Sifuentes-Giraldo, Giuseppe Paolazzi, Elena Bravi, Andreas Schwarting, Raffaele Pellerito, Alessandra Russo, Carlo Selmi, Lesley-Ann Saketkoo, Enrico Fusaro, Simone Parisi, Nicolò Pipitone, Franco Franceschini, Ilaria Cavazzana, Rossella Neri, Simone Barsotti, Veronica Codullo, Lorenzo Cavagna
ObjectiveArthritis, myositis and interstitial lung disease (ILD) constitute the classic clinical triad of anti-synthetase syndrome (ASSD). These patients experience other accompanying features, such as Raynaud's phenomenon, fever or mechanic's hands. Most ASSD patients develop the complete triad during the follow-up. In the present study we aimed to determine whether the subsequent appearance of accompanying features may suggest the development of triad findings lacking at the onset in anti-Jo1 positive ASSD patients.MethodsAnti-Jo1 positive patients presenting with incomplete ASSD (no >2 classic triad features) were assessed. Clinical characteristics and clusters of disease manifestations were retrospectively collected and analyzed in a large international multicenter cohort of ASSD patients.Results165 patients (123 women) with incomplete ASSD were identified. Ninety-five patients (57.5%) developed new classic triad manifestations after 15months median (IQR 9–51) and 40 (24%) developed new accompanying features after 19months median (IQR 6–56) from disease onset. During the follow-up, the ex-novo occurrence of triad features was observed in 32 out of 40 patients (80%) with new accompanying findings and in 63 out of 125 patients (50.5%) without new accompanying findings (p=0.002). In patients with at least one new accompanying feature the odds ratio for the occurrence of new triad manifestations was 3.94 with respect to patients not developing ex-novo accompanying findings (95% CI 1.68–9.21, p=0.002).ConclusionAnti-Jo1 ASSD patients with incomplete forms at disease onset are at high risk for the subsequent occurrence of lacking classic triad findings. Although all ASSD patients should be carefully assessed for the occurrence of new triad features, a closer follow-up should be considered in the subgroup of patients developing ex novo accompanying findings. These patients, indeed, have near four-fold increased risk for new classic triad manifestation occurrence with respect to patients not presenting ex novo accompanying findings.



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The dangerous liaison between pollens and pollution in respiratory allergy

Publication date: Available online 29 January 2017
Source:Annals of Allergy, Asthma & Immunology
Author(s): Giovanna Schiavoni, Gennaro D'Amato, Claudia Afferni
ObjectiveTo recapitulate the more recent epidemiologic studies on the association of air pollution with respiratory allergic diseases prevalence and to discuss the main limitations of current approaches used to establish a link between pollinosis and pollution.Data SourcesThrough the use of PubMed, we conducted a broad literature review in the following areas: epidemiology of respiratory allergic diseases, effect of pollution and climate changes on pollen grains, and immunomodulatory properties of pollen substances.Study SelectionStudies on short- and long-term exposure to air pollutants, such as gaseous and particulate materials, on allergic sensitization, and on exacerbation of asthma symptoms were considered.ResultsTrend in respiratory allergic disease prevalence has increased worldwide during the last 3 decades. Although recent epidemiologic studies on a possible association of this phenomenon with increasing pollution are controversial, botanic studies suggest a clear effect of several pollutants combined to climatic changes on the increased expression of allergenic proteins in several pollen grains. The current literature suggests the need for considering both pollen allergen and pollutant contents for epidemiologic evaluation of environmental determinants in respiratory allergies. We propose that a measure of allergenic potential of pollens, indicative of the increase in allergenicity of a polluted pollen, may be considered as a new risk indicator for respiratory health in urban areas.ConclusionBecause public greens are located in strict proximity to the anthropogenic sources of pollution, the identification of novel more reliable parameters for risk assessment in respiratory allergic diseases is an essential need for public health management and primary prevention area.



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Assessment of sleep disturbance in children with allergic rhinitis

Publication date: Available online 29 January 2017
Source:Annals of Allergy, Asthma & Immunology
Author(s): Kathleen Dass, Alexandra Julia Petrusan, Jennifer Beaumont, Phyllis Zee, Jin-Shei Lai, Anna Fishbein




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