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1.
Pediatr Dermatol ; 41(2): 253-255, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38342506

RESUMO

The video app TikTok, a controversial platform, has increasingly been utilized for the dissemination of health-related topics. In this study, the quality of information on the top 50 most viewed TikTok videos on infantile hemangiomas (IHs) labeled #hemangioma was analyzed. The results showed that the videos were skewed toward more severe subtypes of IH, and most were directed at raising awareness about IH or combating stigmatizing attitudes toward birthmarks. Providers should be aware that social media applications such as TikTok are a commonly used venue for parents to cope and connect but may relay inaccurate or misleading information.


Assuntos
Hemangioma Capilar , Hemangioma , Mídias Sociais , Humanos , Estudos Transversais , Pais
2.
Pediatr Dermatol ; 40(5): 863-865, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37364926

RESUMO

The most common bacteria isolated from wound cultures in patients recorded in the Epidermolysis Bullosa Clinical Characterization and Outcomes Database (EBCCOD) are Staphylococcus aureus and Pseudomonas aeruginosa. Given the prevalence of P. aeruginosa in this patient population and prior research implicating P. aeruginosa's potential role in carcinogenesis, we sought to further analyze patients with recorded wound cultures positive for Pseudomonas aeruginosa in the EBCCOD. We provide a descriptive analysis of this subset of patients and highlight potential avenues for future longitudinal studies that may have significant implications in our wound care management for patients with epidermolysis bullosa.


Assuntos
Epidermólise Bolhosa , Pseudomonas aeruginosa , Humanos , Epidermólise Bolhosa/complicações , Epidermólise Bolhosa/microbiologia
3.
Pediatr Dermatol ; 40(1): 28-34, 2023 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-36127831

RESUMO

BACKGROUND/OBJECTIVES: We sought to describe the experience among members of the Hemangioma Investigator Group with pulsed dye laser (PDL) in the treatment of nonulcerated infantile hemangioma (IH) in pediatric patients in the pre- and post-beta-blocker era. METHODS: A multicenter retrospective cohort study was conducted in patients with nonulcerated IH treated with laser therapy. Patient demographics, IH characteristics, indications for/timing of laser therapy, as well as laser parameters were collected. Responses to laser therapy were evaluated using a visual analog scale (VAS). RESULTS: One hundred and seventeen patients with IH were treated with PDL. 18/117 (15.4%) had early intervention (defined as <12 months of life), and 99/117 (84.6%) had late intervention (≥12 months of life). In the late intervention group, 73.7% (73/99) had additional medical management of their IH. The mean age at PDL initiation for the late intervention group was 46.7 ± 35.3 months of life (range 12-172 months) with total number of treatments to maximal clearing of 4.2 ± 2.8 (range 1-17). Those who received propranolol prior to PDL received fewer sessions (1.1 fewer sessions, approaching significance [p = .056]).     On the VAS, there was a mean 85% overall improvement compared to baseline (range 18%-100%), with most improvement noted in erythema and/or telangiectasias. The incidence of adverse effects was 6/99 (6.1%). CONCLUSIONS: PDL is a useful tool in the treatment of IH, with notable improvement of telangiectasia and erythema and low risk of complications.   PDL is often introduced after the maximal proliferative phase.


Assuntos
Hemangioma Capilar , Hemangioma , Lasers de Corante , Humanos , Criança , Estudos Retrospectivos , Lasers de Corante/uso terapêutico , Hemangioma Capilar/radioterapia , Hemangioma Capilar/cirurgia , Hemangioma/radioterapia , Hemangioma/cirurgia , Hemangioma/etiologia , Antagonistas Adrenérgicos beta , Resultado do Tratamento
4.
Pediatr Dermatol ; 40(1): 90-95, 2023 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-36128651

RESUMO

INTRODUCTION: Paraneoplastic pemphigus (PNP) is a rare, often fatal, autoimmune blistering disease of the skin and mucous membranes. In children, PNP is frequently associated with Castleman disease (CD). This series describes five cases of PNP associated with CD. METHODS: Data were collected retrospectively from the medical records of patients with a diagnosis of PNP and CD from January 2013 to June 2022. Patients ≤22 years old with clinical and immunopathologic evidence of PNP were included; CD was diagnosed histopathologically. RESULTS: Two children, two adolescents, and one young adult (two males, three females) were included. The average age at disease presentation was 11.8 years (range: 7-22 years). Oral (n = 5) and anogenital (n = 3) mucositis were common. Four patients had "unicentric" CD (UCD); one patient had "multicentric" CD (MCD). Castleman tumors were in the retroperitoneum (n = 4) or axilla (n = 1). One patient had myasthenia gravis without thymoma. Three patients had bronchiolitis obliterans (BO). Three patients had complete resection of their CD; two had partial resection. Three patients remain alive with a median follow-up of 13 months (range: 12 months to 13 years); two are clinically stable with resolution of mucocutaneous lesions; one has persistent BO requiring ongoing ventilatory support. Patients who remain alive had UCD with complete resection; all deceased patients had partial resection and BO. CONCLUSION: Most patients had UCD, and the retroperitoneum was the most common location. Patients with MCD, incomplete resection, and BO died; patients with UCD and complete resection remain alive, even in the setting of BO. Consideration of PNP is critical when pediatric patients present with mucositis as PNP may be clinically indistinguishable from more common causes of mucositis.


Assuntos
Doenças Autoimunes , Bronquiolite Obliterante , Hiperplasia do Linfonodo Gigante , Mucosite , Síndromes Paraneoplásicas , Pênfigo , Masculino , Feminino , Adolescente , Adulto Jovem , Humanos , Criança , Adulto , Pênfigo/complicações , Pênfigo/diagnóstico , Hiperplasia do Linfonodo Gigante/complicações , Hiperplasia do Linfonodo Gigante/diagnóstico , Hiperplasia do Linfonodo Gigante/patologia , Mucosite/complicações , Estudos Retrospectivos , Bronquiolite Obliterante/etiologia , Síndromes Paraneoplásicas/diagnóstico , Síndromes Paraneoplásicas/etiologia , Síndromes Paraneoplásicas/patologia
5.
Pediatr Dermatol ; 39(5): 679-688, 2022 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-35918890

RESUMO

The evaluation of pediatric patients with subcutaneous nodules remains a diagnostic challenge. Pediatric dermatologists are regularly confronted with patients who have a nonspecific nodule. Though most masses that require evaluation are ultimately benign, the possibility of a more aggressive process, including borderline or malignant neoplasms, underscores the pivotal role of the pediatric dermatologist in recognizing these lesions. The aim of this review is to provide an overview of lumps and bumps that are important to recognize to prevent delay in diagnosis or treatment of a serious underlying condition. Clinical clues that may lead the pediatric dermatologist to have a higher index of suspicion for more aggressive lesions are reviewed. Suggestions for evaluation and workup, as well as tips for the difficult to discern lesion, are proposed.


Assuntos
Neoplasias Cutâneas , Criança , Diagnóstico Diferencial , Humanos , Neoplasias Cutâneas/diagnóstico
6.
Pediatr Dermatol ; 39(5): 718-726, 2022 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-35734850

RESUMO

BACKGROUND/OBJECTIVES: The COVID-19 pandemic prompted a rapid expansion in the use of telemedicine. This study aimed to assess the experiences of hemangioma specialists utilizing telemedicine during the COVID-19 pandemic to evaluate and manage infantile hemangiomas (IH), including perceived effectiveness of different modalities and barriers to care delivery. METHODS: Multicenter cross-sectional study asking providers to describe their experiences using telemedicine for initial evaluation of IH from March to September 2020. RESULTS: The study included 281 patients from 15 medical centers internationally. Median time from referral to evaluation was 17 days. Median physician confidence in performing evaluations via telemedicine was 95.0 (IQR 90.0-100.0). Most evaluations were performed via video communication with photographs or audio communication with photographs; when not initially available, photographs were requested in 51.4%. Providers preferred follow-up modalities that included photographs. CONCLUSIONS: Physicians with extensive expertise in managing IH are confident in their abilities to assess and manage IH via telemedicine including initiating treatment in patients without risk factors for beta-blocker therapy. There was a preference for hybrid modalities that included photographs. The data suggest that telemedicine can be effective for managing IH and may decrease wait times and improve specialist reach to underserved areas.


Assuntos
COVID-19 , Hemangioma Capilar , Hemangioma , Telemedicina , COVID-19/epidemiologia , Estudos Transversais , Hemangioma/diagnóstico , Hemangioma/terapia , Humanos , Pandemias
7.
J Am Acad Dermatol ; 87(3): 623-631, 2022 09.
Artigo em Inglês | MEDLINE | ID: mdl-32592878

RESUMO

BACKGROUND: Transition from pediatric to adult care is a critical component of health care for children with long-term needs. The characteristics of epidermolysis bullosa (EB) demand higher than average levels of provider support. There is consensus among health care professionals regarding the importance of transition; however, there is a scarcity of practical information regarding models for patients with EB. OBJECTIVE: To review transition of care programs in varying specialties. Highlight practical considerations to facilitate the development of programs for patients with EB and other complex dermatologic conditions. METHODS: Articles were identified via MEDLINE and EMBASE health literature databases and screened for relevance to transition of care. RESULTS: Various models for transition exist. A well-executed formal transition program, early introduction, interdisciplinary collaboration, and psychosocial support were themes associated with successful outcomes. LIMITATIONS: Transition of care programs that have not been described in the literature are not reflected in this review. CONCLUSIONS: Patients with EB have unique needs that affect transition and span expertise across traditional boundaries, such as dependency on others for daily skin care, failure to thrive, and risk of squamous cell carcinoma. Given the rarity of the disease, patients with EB will benefit from collaborative efforts to develop programs to optimize successful transition.


Assuntos
Epidermólise Bolhosa , Transição para Assistência do Adulto , Adulto , Criança , Consenso , Bases de Dados Factuais , Epidermólise Bolhosa/complicações , Epidermólise Bolhosa/terapia , Humanos , Transferência de Pacientes
8.
Dermatol Clin ; 40(1): 61-71, 2022 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-34799036

RESUMO

This article reviews the clinical findings of epidermal nevi and their associated syndromes and provides an update on their pathogenic genetic changes as well as targeted therapies detailed to date.


Assuntos
Nevo , Síndrome de Proteu , Neoplasias Cutâneas , Humanos , Nevo/genética , Neoplasias Cutâneas/genética
9.
JAMA Dermatol ; 2021 06 16.
Artigo em Inglês | MEDLINE | ID: mdl-34132754

RESUMO

Importance: A 2010 prospective study of 108 infants estimated the incidence of PHACE (posterior fossa malformations, hemangioma, arterial anomalies, cardiac defects, eye anomalies) syndrome to be 31% in children with facial infantile hemangiomas (IHs) of at least 22 cm2. There is little evidence regarding the associations among IH characteristics, demographic characteristics, and risk of PHACE syndrome. Objectives: To evaluate demographic characteristics and comorbidities in a large cohort of patients at risk for PHACE syndrome and assess the clinical features of large head and neck IH that may be associated with a greater risk of a diagnosis of PHACE syndrome. Design, Setting, and Participants: This multicenter, retrospective cohort study assessed all patients with a facial, head, and/or neck IH who were evaluated for PHACE syndrome from August 1, 2009, to December 31, 2014, at 13 pediatric dermatology referral centers across North America. Data analysis was performed from June 15, 2017, to February 29, 2020. Main Outcomes and Measures: The main outcome was presence or absence of PHACE syndrome. Data included age at diagnosis, sex, patterns of IH presentation (including size, segment location, and depth), diagnostic procedures and results, and type and number of associated anomalies. Results: A total of 238 patients (mean [SD] age, 2.96 [4.71] months; 184 [77.3%] female) were included in the analysis; 106 (44.5%) met the criteria for definite (n = 98) or possible (n = 8) PHACE syndrome. A stepwise linear regression model found that a surface area of 25 cm2 or greater (odds ratio [OR] 2.99; 95% CI, 1.49-6.02) and involvement of 3 or more locations (OR, 17.96; 95% CI, 6.10-52.85) to be statistically significant risk factors for PHACE syndrome. Involvement of the parotid gland (OR, 0.39; 95% CI, 0.18-0.85) and segment S2 (OR, 0.38; 95% CI, 0.16-0.91) was associated with a lower risk. Race and ethnicity may also be associated with PHACE syndrome risk, although more studies are needed. Conclusions and Relevance: This cohort study further described factors associated with both a higher and lower risk of PHACE syndrome. The presence of multiple anatomical sites and large surface area were associated with greater risk, whereas S2 or parotid IHs were associated with lower, but still potential, risk. These findings can help in counseling families and decision-making regarding evaluation of infants with large head and neck IHs.

10.
J Am Acad Dermatol ; 84(3): 654-660, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32603724

RESUMO

BACKGROUND: High-flow vascular stains (HFVS) are lesions that have the appearance of capillary malformations/port wine stains but are associated with increased arterial flow. OBJECTIVE: To identify features of HFVS that differentiate them from typical "slow-flow" port wine stains. METHODS: Retrospective multicenter cohort study of HFVS evaluated across 7 centers was conducted. HFVS were characterized by clinical features (warmth, thrill, rapid capillary refill), radiologic findings (fast flow), or mutations associated with capillary malformation-arteriovenous malformation syndrome. Investigators reviewed photographs. RESULTS: The study reviewed 70 patients with HFVS (47 multifocal and 23 solitary). Most were flat (77%), warm to the touch (60%), and red or pink-red in color (35%), with heterogeneous color saturation (73%) and well-defined borders (71%). Regional soft tissue swelling/overgrowth was common (47%). Head and neck location was most common (38%). Among 34 HFVS with photographic review over time, all demonstrated changes in appearance. LIMITATIONS: Retrospective design, recall bias, lack of standardized time points or visual analog scale, and image variability. CONCLUSION: Heterogeneity of stain color saturation, warmth to touch, peripheral pallor, and overgrowth/soft tissue swelling help distinguish HFVS from port wine stains. Darkening of color and increased border demarcation may develop over time. These findings raise suspicion for HFVS and provide an indication to assess for extracutaneous involvement.


Assuntos
Malformações Arteriovenosas/diagnóstico , Capilares/anormalidades , Mancha Vinho do Porto/diagnóstico , Adolescente , Malformações Arteriovenosas/genética , Criança , Pré-Escolar , Análise Mutacional de DNA , Diagnóstico Diferencial , Feminino , Humanos , Angiografia por Ressonância Magnética , Masculino , Mutação , Mancha Vinho do Porto/genética , Pele/irrigação sanguínea , Pele/diagnóstico por imagem , Ultrassonografia Doppler , Adulto Jovem
11.
Pediatr Dermatol ; 38(1): 119-124, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-33247481

RESUMO

BACKGROUND/OBJECTIVES: Patients with epidermolysis bullosa (EB) require care of wounds that are colonized or infected with bacteria. A subset of EB patients are at risk for squamous cell carcinoma, and bacterial-host interactions have been considered in this risk. The EB Clinical Characterization and Outcomes Database serves as a repository of information from EB patients at multiple centers in the United States and Canada. Access to this resource enabled broad-scale analysis of wound cultures. METHODS: A retrospective analysis of 739 wound cultures from 158 patients from 13 centers between 2001 and 2018. RESULTS: Of 152 patients with a positive culture, Staphylococcus aureus (SA) was recovered from 131 patients (86%), Pseudomonas aeruginosa (PA) from 56 (37%), and Streptococcus pyogenes (GAS) from 34 (22%). Sixty-eight percent of patients had cultures positive for methicillin-sensitive SA, and 47%, methicillin-resistant SA (18 patients had cultures that grew both methicillin-susceptible and methicillin-resistant SA at different points in time). Of 15 patients with SA-positive cultures with recorded mupirocin susceptibility testing, 11 had mupirocin-susceptible SA and 6 patients mupirocin-resistant SA (2 patients grew both mupirocin-susceptible and mupirocin-resistant SA). SCC was reported in 23 patients in the entire database, of whom 10 had documented wound cultures positive for SA, PA, and Proteus species in 90%, 50%, and 20% of cases, respectively. CONCLUSIONS: SA and PA were the most commonly isolated bacteria from wounds. Methicillin resistance and mupirocin resistance were reported in 47% and 40% of patients tested, respectively, highlighting the importance of ongoing antimicrobial strategies to limit antibiotic resistance.


Assuntos
Epidermólise Bolhosa , Infecções Estafilocócicas , Antibacterianos/uso terapêutico , Canadá , Epidermólise Bolhosa/complicações , Epidermólise Bolhosa/tratamento farmacológico , Humanos , Mupirocina , Estudos Retrospectivos , Infecções Estafilocócicas/tratamento farmacológico , Staphylococcus aureus
12.
Pediatr Dermatol ; 37(3): 412-418, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32298480

RESUMO

The COVID-19 pandemic has caused significant shifts in patient care including a steep decline in ambulatory visits and a marked increase in the use of telemedicine. Infantile hemangiomas (IH) can require urgent evaluation and risk stratification to determine which infants need treatment and which can be managed with continued observation. For those requiring treatment, prompt initiation decreases morbidity and improves long-term outcomes. The Hemangioma Investigator Group has created consensus recommendations for management of IH via telemedicine. FDA/EMA-approved monitoring guidelines, clinical practice guidelines, and relevant, up-to-date publications regarding initiation and monitoring of beta-blocker therapy were used to inform the recommendations. Clinical decision-making guidelines about when telehealth is an appropriate alternative to in-office visits, including medication initiation, dosage changes, and ongoing evaluation, are included. The importance of communication with caregivers in the context of telemedicine is discussed, and online resources for both hemangioma education and propranolol therapy are provided.


Assuntos
Betacoronavirus , Infecções por Coronavirus/epidemiologia , Hemangioma/terapia , Pneumonia Viral/epidemiologia , Neoplasias Cutâneas/terapia , Telemedicina , Antagonistas Adrenérgicos beta/uso terapêutico , COVID-19 , Infecções por Coronavirus/prevenção & controle , Infecções por Coronavirus/transmissão , Hemangioma/patologia , Humanos , Lactente , Recém-Nascido , Pandemias/prevenção & controle , Seleção de Pacientes , Pneumonia Viral/prevenção & controle , Pneumonia Viral/transmissão , SARS-CoV-2 , Neoplasias Cutâneas/patologia
13.
Pediatr Dermatol ; 37(1): 78-85, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31631401

RESUMO

BACKGROUND/OBJECTIVE: The pathogenesis of infantile hemangiomas (IH), PHACE, and LUMBAR syndromes remains unknown. We aim to describe histopathologic features of midline anomalies associated with IH, including patients with PHACE and LUMBAR syndromes. METHODS: A multicenter retrospective chart review was performed to identify patients with IH, PHACE, and LUMBAR syndrome with histopathologic specimens from sternal or midline anomalies. A total of 18 midline lesions from 13 patients were included. Out of 18, 14 midline lesions underwent both histopathologic and clinical review. Three hamartoma-like chin plaques and one supraumbilical raphe underwent only clinical review. RESULTS: All 13 patients had midline lesions and IH. Histopathologic diagnoses were as follows: rhabdomyomatous mesenchymal hamartoma (3), folliculosebaceous cystic hamartoma (1), fibroepithelial polyp (1), verrucous epidermal hyperplasia with vascular proliferation and fibroplasia (1), congenital midline cervical cleft (1), pericardium with fibrosis (1), fibrous components with increased collagen (1), atrophic skin/membrane (3), angiolipomatous mass with neural components (1), and lipomatous mass (1). Due to the retrospective nature of this study, it was not possible to obtain pathology slides for all midline lesions that had previously been biopsied or resected. We show clinically and histopathologically a new association between PHACE syndrome and rhabdomyomatous mesenchymal hamartoma (RMH), in addition to demonstrating the association between PHACE syndrome and chin hamartomas. We also display histopathologic findings seen in midline lesions resected from LUMBAR patients. CONCLUSION: Rhabdomyomatous mesenchymal hamartoma is thought to be related to aberrations of mesenchymal cells during development; therefore, this may provide clues to the pathogenesis of IH and related syndromes.


Assuntos
Coartação Aórtica/patologia , Anormalidades Congênitas/patologia , Anormalidades do Olho/patologia , Hamartoma/patologia , Hemangioma/patologia , Síndromes Neurocutâneas/patologia , Neoplasias Cutâneas/patologia , Anormalidades Múltiplas , Feminino , Humanos , Lactente , Masculino , Malformações do Sistema Nervoso/patologia , Estudos Retrospectivos , Anormalidades da Pele/patologia , Síndrome
14.
Pediatr Dermatol ; 36(1): 9-15, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30152555

RESUMO

Despite the availability of effective medications for the management of atopic dermatitis and xerosis, patients may use nonconventional therapies such as topical oils. Patients choose these treatments because of the perceived lower risk of natural products and the fear of potential adverse effects of topical steroids. We review the use of topical olive, coconut, and sunflower seed oil in the treatment of atopic dermatitis and xerosis with a focus on children Currently available evidence suggests that olive oil may exacerbate xerosis and atopic dermatitis. Further studies are needed to make definitive recommendations regarding the use of coconut and sunflower seed oil.


Assuntos
Dermatite Atópica/terapia , Óleos de Plantas/administração & dosagem , Administração Tópica , Adolescente , Adulto , Criança , Pré-Escolar , Humanos , Lactente , Óleos de Plantas/efeitos adversos
16.
JAMA Dermatol ; 155(2): 196-203, 2019 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-30586139

RESUMO

Importance: Children with epidermolysis bullosa (EB) comprise a rare population with high morbidity and mortality. An improved understanding of the clinical trajectory of patients with EB, including age at time of clinical diagnosis and major clinical events, is needed to refine best practices and improve quality of life and clinical outcomes for patients with EB. Objectives: To describe demographics, clinical characteristics, milestone diagnostic and clinical events (such as initial esophageal dilation), and outcomes in patients with EB using the Epidermolysis Bullosa Clinical Characterization and Outcomes Database and to determine what characteristics may be associated with overall EB severity and/or disease progression. Design, Setting, and Participants: This cohort study included data on patients with EB who were enrolled in the Epidermolysis Bullosa Clinical Characterization and Outcomes Database from January 1, 2011, to June 30, 2017; 17 participating EB centers in the United States and Canada contributed data to this study. Exposures: Type of EB, including recessive dystrophic epidermolysis bullosa (RDEB), junctional epidermolysis bullosa (JEB), dominant dystrophic epidermolysis bullosa (DDEB), and epidermolysis bullosa simplex (EBS). Main Outcomes and Measures: Demographic information, clinical characteristics (including age at onset of signs of EB and subsequent clinical diagnosis), types of diagnostic testing performed, and milestone clinical events for patients with RDEB. Results: Of 644 enrolled patients from 17 sites included in this study, 323 were male (50.2%), with a mean (SD) age of 14.4 (11.7) years; 283 (43.9%) had RDEB, 194 (30.1%) had EBS, 104 (16.2%) had DDEB, and 63 (9.8%) had JEB. Signs of disease were present at birth in 202 patients with RDEB (71.4%), 39 with JEB (61.9%), 60 with DDEB (57.7%), and 74 with EBS (38.1%). For those with signs of disease at birth, a clinical diagnosis was made at the time of birth in 135 patients with RDEB (67.0%), 31 with DDEB (52.6%), 35 with EBS, (47.3%) and 18 with JEB (46.2%). Patients with JEB had the highest rate of any confirmatory testing (51 of 63 [81.0%]), followed by RDEB (218 of 283 [77.0%]), DDEB (71 of 104 [68.3%]), and EBS (100 of 194 [51.5%]). For all types of EB, both electron microscopy and immunofluorescence microscopy were performed at younger ages than genetic analysis. Among 283 patients with RDEB, 157 (55.5%) had esophageal dilation, 104 (36.7%) had gastrostomy tube placement, 62 (21.9%) had hand surgery, 18 (6.4%) developed squamous cell carcinoma, and 19 (6.7%) died. Conclusions and Relevance: The findings suggest that diagnostic testing for EB is more common for patients with severe phenotypes. Earlier diagnostic testing may enable improved characterizations of patients so that appropriate counseling and clinical care may be offered, especially pertaining to milestone events for those with RDEB.


Assuntos
Epidermólise Bolhosa/epidemiologia , Epidermólise Bolhosa/genética , Epidermólise Bolhosa/patologia , Predisposição Genética para Doença/epidemiologia , Adolescente , Distribuição por Idade , Biópsia por Agulha , Canadá , Criança , Pré-Escolar , Estudos de Coortes , Bases de Dados Factuais , Progressão da Doença , Feminino , Seguimentos , Humanos , Imuno-Histoquímica , Incidência , Lactente , Masculino , América do Norte/epidemiologia , Estudos Retrospectivos , Medição de Risco , Índice de Gravidade de Doença , Distribuição por Sexo , Análise de Sobrevida , Adulto Jovem
18.
Pediatrics ; 138(4)2016 10.
Artigo em Inglês | MEDLINE | ID: mdl-27677573

RESUMO

Routine childhood immunizations have resulted in great reductions in vaccine-preventable infectious diseases. Vaccine-related adverse events, albeit rare, can be of significant consequence. Although anaphylaxis, or type I hypersensitivity, is recognized as a potential reaction after vaccination, delayed type hypersensitivity or type IV reactions are less so. We present a case of persistent subcutaneous nodules and sterile abscesses in the setting of delayed type hypersensitivity to aluminum, confirmed by patch testing and recurrence on re-exposure. We review sources of aluminum in common immunizations, principles for treatment, and strategies for management of future vaccinations for this patient.


Assuntos
Abscesso/etiologia , Alumínio/efeitos adversos , Hipersensibilidade Tardia/imunologia , Vacinas/efeitos adversos , Humanos , Lactente , Masculino , Testes do Emplastro
19.
Pediatrics ; 138(3)2016 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-27527799

RESUMO

BACKGROUND: There has been a dramatic increase in the off-label use of ophthalmic timolol maleate, a ß-blocker used for infantile hemangioma (IH) treatment as a topical counterpart to oral propranolol. Its safety and efficacy in a pediatric population with IH have not been evaluated in a large cohort. Our goal was to retrospectively assess timolol's effectiveness, discern characteristics associated with response, and document reported adverse events. METHODS: A multicenter retrospective cohort study of 731 patients treated with topical timolol was completed at 9 centers. Inclusion required an IH suitable for timolol in the treating physician's judgment and access to clinical details including photographs. Logistic regression analysis and descriptive statistics were performed. Primary outcome measures were efficacy assessed by using visual analog scales for color and for size, extent, and volume from review of digital photographs taken as standard of care. RESULTS: Most IHs were localized (80.1%) and superficial (55.3%). Risk of disfigurement was the most common indication for therapy (74.3%). Duration of therapy (P < .0001), initial thinness (P = .008), and subtype (P = .031) were significant predictors of response. Best response occurred in superficial IHs <1 mm thick. Fifty-three (7.3%) required subsequent therapy with systemic ß-blocker. Adverse events were mild, occurring in 25 (3.4%) patients. No cardiovascular side effects were documented. CONCLUSIONS: Timolol seems to be a well-tolerated, safe treatment option with moderate to good effectiveness, demonstrating best response in thin, superficial IHs regardless of pretreatment size. Timolol can be recommended as an alternative to systemic ß-blockers and watchful waiting for many patients.


Assuntos
Antagonistas Adrenérgicos beta/administração & dosagem , Hemangioma/tratamento farmacológico , Neoplasias Cutâneas/tratamento farmacológico , Timolol/administração & dosagem , Administração Oral , Administração Tópica , Antagonistas Adrenérgicos beta/efeitos adversos , Estudos de Coortes , Feminino , Hemangioma/patologia , Humanos , Lactente , Recém-Nascido , Masculino , Uso Off-Label , Propranolol/uso terapêutico , Estudos Retrospectivos , Neoplasias Cutâneas/patologia , Timolol/efeitos adversos , Escala Visual Analógica
20.
Pediatr Dermatol ; 33(2): 178-83, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-26863906

RESUMO

BACKGROUND: Twins have a higher-than-expected risk of infantile hemangiomas (IHs), but the exact reasons for this association are not clear. Comparing concordant and discordant twin pairs might help elucidate these factors and yield more information about IH risk factors. METHODS: A prospective cohort study of twin pairs from 12 pediatric dermatology centers in the United States, Canada, Argentina, and Spain was conducted. Information regarding maternal pregnancy history, family history of vascular birthmarks, zygosity (if known), and pregnancy-related information was collected. Information regarding twins (N = 202 sets) included birthweight, gestational age (GA), presence or absence of IHs, numbers and subtypes of IHs, presence of other birthmarks, and other medical morbidities. RESULTS: Two hundred two sets of twins were enrolled. Concordance for IH was present in 37% of twin pairs. Concordance for IH was inversely related to gestational age (GA), present in 42% of GA of 32 weeks or less, 36% of GA of 33 to 36 weeks, and 32% of GA of 37 weeks or more. Twins of GA of 34 weeks or less were more than two and a half times as likely to be concordant as those of GA of 35 weeks or more (odds ratio (OR) = 2.66, 95% confidence interval (CI) = 1.42-4.99; p < 0.01). In discordant twins, lower birthweight conferred a high risk of IH; of the 64 sets of twins with 10% or greater difference in weight, the smaller twin had IH in 62.5% (n = 40) of cases, versus 37.5% (n = 24) of cases in which the higher-birthweight twin was affected. Zygosity was reported in 188 twin sets (93%). Of these, 78% were dizygotic and 22% monozygotic. There was no statistically significant difference in rates of concordance between monozygotic twins (43%, 18/42) and dizygotic twins (36%, 52/146) (p = 0.50). In multivariate analysis comparing monozygotic and dizygotic twins, adjusting for effects of birthweight and sex, the likelihood of concordance for monozygotic was not appreciably higher than that for dizygotic twins (OR = 1.14, 95% CI = 0.52-2.49). Female sex also influenced concordance, confirming the effects of female sex on IH risk. The female-to-male ratio was 1.7:1 in the entire cohort and 1.9:1 in those with IH. Of the 61 concordant twin sets with known sex of both twins, 41% were female/female, 43% were female/male, and 16% were male/male. CONCLUSIONS: These findings suggest that the origin of IHs is multifactorial and that predisposing factors such as birthweight, sex, and GA may interact with one another such that a threshold is reached for clinical expression.


Assuntos
Doenças em Gêmeos , Hemangioma/genética , Peso ao Nascer , Estudos de Coortes , Feminino , Idade Gestacional , Humanos , Recém-Nascido , Masculino , Gravidez , Estudos Prospectivos , Fatores Sexuais
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