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1.
Pediatr Transplant ; 28(1): e14618, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37786978

RESUMO

BACKGROUND: There is a well-documented risk of secondary cutaneous malignancies following allogeneic hematopoietic stem cell transplant (HSCT), but data on risk in pediatric populations are limited. The objective of this study is to perform a systematic review of reported features and outcomes of skin cancers in pediatric allogeneic HSCT recipients. METHODS: MEDLINE, EMBASE, CINAHL, Cochrane, and Web of Science were systematically searched (Prospero CRD42022342139). Studies reporting cutaneous cancer outcomes were included if the age at transplant was ≤19 years. Titles, abstracts, and full-text articles were screened in duplicate. RESULTS: Out of 824 citations that were screened, 12 articles were selected for analysis. The final sample included 67 pediatric HSCT recipients, comprising 65 allogeneic transplant recipients and 2 cases of HSCT with an unknown donor type. The median age at transplant and skin cancer diagnosis were 7.4 and 13 years, respectively. Out of the 67 pediatric HSCT recipients, some patients developed more than one lesion, resulting in 71 lesions. The most common skin cancer type was cutaneous squamous cell carcinoma (32 lesions), followed by basal cell carcinoma (25 lesions). The median latency period between HSCT and skin cancer diagnosis ranged from 0 to 29 years. Identified risk factors for skin cancers included younger age at the time of transplant, exposure to total body irradiation, prolonged post-transplant immunosuppression, graft versus host disease, and sunburn. CONCLUSION: Skin cancers are reported in pediatric allogeneic HSCT recipients, and the risk appears to be increased. More data are needed to better characterize this risk.


Assuntos
Carcinoma de Células Escamosas , Doença Enxerto-Hospedeiro , Transplante de Células-Tronco Hematopoéticas , Neoplasias Cutâneas , Humanos , Criança , Adulto Jovem , Adulto , Neoplasias Cutâneas/etiologia , Carcinoma de Células Escamosas/etiologia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Doença Enxerto-Hospedeiro/complicações , Transplante Homólogo/efeitos adversos , Progressão da Doença
2.
Pediatr Dermatol ; 2024 Jul 04.
Artigo em Inglês | MEDLINE | ID: mdl-38961790

RESUMO

Our study aims to synthesize existing evidence of dupilumab for alopecia areata (AA) in pediatric patients with atopic dermatitis (AD). We searched MEDLINE and Embase on March 1, 2024, using keywords related to AD, AA, dupilumab, and pediatric patient populations per PRISMA-ScR guidelines. A mean SALT score reduction of 42.6 following dupilumab treatment (p < .01) over an average of 3.21 months, and a mean reduction of Investigator Global Assessment (IGA) levels of 2.14 units (p < .01) demonstrates the efficacy of dupilumab in pediatric AA when there is concurrent AD. Our findings in combination with dupilumab's favorable safety profile in pediatric AD makes it an appealing option for AA treatment, however, a greater understanding of the underlying mechanisms, optimal pediatric patient selection criteria, long-term efficacy, and safety profile of dupilumab in this context is warranted.

3.
Pediatr Dermatol ; 41(1): 16-22, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-37864376

RESUMO

BACKGROUND/OBJECTIVE: Stiff skin syndrome (SSS) is a rare disorder characterized by "rock hard" indurated skin affecting different body parts. The localized variant poses a diagnostic challenge, as it is frequently mistaken for other inflammatory connective tissue disorders. The aim of this study is to provide insightful clinical, radiologic and diagnostic data that might prove useful for the evaluation, management and treatment of pediatric patients with segmental SS. METHODS: This single-center cohort study included patients ≤18 years diagnosed with localized SSS from 1988 to 2021 in a quaternary pediatric healthcare center in Toronto, Canada. Data included demographics, clinical, histopathologic and radiologic features, treatments, and clinical course. Data were summarized with descriptive statistics (mean, standard deviation, medians, interquartile ranges [IQRs]) and frequencies. RESULTS: A total of 11 patients were included. The sclerotic changes were measured clinically and radiologically, by a total of 16 imaging studies: 13 magnetic resonance imaging (MRI) and 3 ultrasound. MRI readings showed abnormal high signal intensity of the affected tissue correlating with the anatomical site of involvement in all cases, specifically, in the shoulder/pelvic girdle with limb extension. Shear wave ultrasound elastography (SWE) demonstrated higher values within the dermis compared to the control site. CONCLUSION: The presence of segmental sclerotic changes that affects the pelvic/shoulder girdle with extension to the extremities, in the absence of inflammation on biopsy and abnormal signaling intensity on imaging is suggestive of SSS. Skin SWE is a feasible, noninvasive, and objective instrument to evaluate and monitor sclerotic changes overtime, it could be potentially extrapolated to other pediatric skin sclerotic conditions.


Assuntos
Contratura , Dermatopatias Genéticas , Humanos , Criança , Estudos Retrospectivos , Estudos de Coortes , Centros de Atenção Terciária
4.
Pediatr Dermatol ; 41(1): 5-11, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-37906120

RESUMO

BACKGROUND: Dupilumab is approved for moderate-severe atopic dermatitis (AD) in patients aged ≥6 months by the US Food and Drug Administration and Health Canada; however, there are little real-world data because providers have limited practical experience with this recently approved therapy. OBJECTIVES: To describe the real-world effectiveness and safety in patients aged <12 years with moderate-severe AD currently receiving or previously having received dupilumab. METHODS: A multicenter retrospective study was conducted at six Canadian sites. Cases were divided into Group 1 ≤2 years old, Group 2 >2 to <6 years old, and Group 3 ≥6 to <12 years old. Medical history and details of dupilumab treatment were collected. The primary outcome was to measure the improvement in eczema area and severity index. Secondary outcomes examined included the children's dermatology life quality index/infant's dermatitis quality of life, peak pruritus numerical rating scale, and delay to dupilumab access for patients who were considered off-label for dupilumab due to their age. RESULTS: Sixty three pediatric patients (37 males) with moderate-to-severe AD were included; the mean age was 6.4 years old (range: 2-11) when dupilumab treatment was started. Overall, 75% (36/48) achieved EASI-75% and 71% (34/48) achieved EASI-90. EASI-75 and EASI-90 were achieved in 90% (17/19) and 73% (12/19) in patients <6 years old, and 76% (22/29) and 59% (17/29) in patients >6 years old, respectively. No serious adverse events were reported. CONCLUSIONS: Dupilumab is safe and effective for patients under the age of 12. However, even for experienced providers, access to the medication was challenging.


Assuntos
Anticorpos Monoclonais Humanizados , Dermatite Atópica , Criança , Pré-Escolar , Humanos , Masculino , Canadá , Dermatite Atópica/tratamento farmacológico , Método Duplo-Cego , Qualidade de Vida , Estudos Retrospectivos , Índice de Gravidade de Doença , Resultado do Tratamento , Feminino , Lactente
5.
J Cutan Med Surg ; : 12034754241253195, 2024 May 28.
Artigo em Inglês | MEDLINE | ID: mdl-38807451

RESUMO

INTRODUCTION: Rosacea is a chronic inflammatory skin condition affecting approximately 5.5% of the global population. Patients present heterogeneously with a mix of features in the central facial region, of which papules and pustules are considered to be a major feature. The identification of effective treatments for reducing inflammatory lesions in rosacea can alleviate the psychosocial burden that many rosacea patients experience, including reduced self-esteem, anxiety, and social withdrawal. The objective of this systematic review is to determine the effectiveness of topical and systemic therapies in reducing lesion count in rosacea patients. METHODS/RESULTS: Medline, Embase, and Cochrane CENTRAL databases were searched, resulting in the inclusion of 43 clinical trials reporting on a total of 18,347 rosacea patients. The most well-studied treatments include ivermectin, metronidazole, azelaic acid, minocycline, and doxycycline. Oral isotretinoin was the most effective treatment in reducing inflammatory lesions and may be recommended for severe recalcitrant cases of rosacea. CONCLUSIONS: Several topical and systemic therapies have demonstrated efficacy in reducing inflammatory lesion count in rosacea patients, with mechanisms of action centred around suppressing inflammation and killing Demodex folliculorum mites. Additional research is required to determine effective combination therapies in rosacea.

6.
J Am Acad Dermatol ; 89(5): 1015-1021, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37451624

RESUMO

BACKGROUND: Infantile hemangiomas (IHs) of the anogenital region remain poorly characterized. OBJECTIVE: To examine the distribution, ulceration rate, and associated congenital anomalies of anogenital IHs. METHODS: Retrospective study at 8 tertiary referral centers. RESULTS: A total of 435 infants with an IH of the anogenital region were enrolled (of which, 319 [73%] were girls). Congenital anomalies were present in 6.4% (n = 28) of infants with an anogenital IH. Segmental or partial segmental anogenital IHs ulcerated in 72% (n = 99 of 138) of infants, whereas 45% (n = 133 of 297) of focal anogenital IHs experienced ulceration (P < .001). In a multivariable logistic regression analysis, segmental or partial segmental morphology (adjusted odds ratio [aOR], 2.70; 95% CI, 1.60-4.64), mixed type (aOR, 3.44; 95% CI, 2.01-6.07), and perianal (aOR, 3.01; 95% CI, 1.53-6.12) and buttocks location (aOR, 2.08; 95% CI, 1.17-3.76) had increased odds of ulceration. Segmental or partial segmental IHs of the genitalia were confined to distinct anatomic territories and were predominantly distributed unilaterally, with a linear demarcation at the perineal raphe. LIMITATIONS: Possible selection bias, given recruitment at tertiary referral centers. CONCLUSION: This study improves our understanding of high-risk features of anogenital IHs and demonstrates that genital segmental or partial segmental IHs develop within distinct anatomic territories.

7.
J Cutan Med Surg ; 27(6): 621-627, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37823355

RESUMO

INTRODUCTION: The need for pediatric dermatology services is increasing across Canada. In parallel, the complexity of treatment with novel targeted therapeutics has increased. Currently, there is no accredited and limited non-accredited fellowship training access to pediatric dermatology in Canada. HYPOTHESIS: Understanding the current state of pediatric dermatology training in Canada will provide insight into opportunities for strategic improvement. METHODS: A survey was distributed to 44 pediatric dermatology providers. In addition, a review of the burden of pediatric skin disease and education/training in Canada was performed. RESULTS: Thirty-four specialists responded to the survey (77% response rate). One third of current pediatric dermatology providers are over 50 years old and half of these (15%) plan to retire within the next 5 years. Half of respondents were dermatologists, 35% were pediatricians, and 11% were double boarded. Almost all respondents practiced in an academic setting (94%). Most had further fellowship training in pediatric dermatology (82.4%) but only 57% achieved this training in Canada, due to lack of accredited or non-accredited funded fellowship positions. CONCLUSION: There is a high and growing need for pediatric dermatology specialty care in a diverse range of settings. The current provider population and training programs are insufficient to meet current and future demands. We highlighted solutions to close this gap between supply and demand including increased double board certification in Pediatrics and Dermatology, a protected pediatric stream within existing Dermatology residency training programs and accredited fellowships in Pediatric Dermatology for both dermatologists and pediatricians.


Assuntos
Dermatologia , Internato e Residência , Humanos , Criança , Pessoa de Meia-Idade , Dermatologia/educação , Canadá , Recursos Humanos , Inquéritos e Questionários
8.
Pediatr Transplant ; 26(1): e14146, 2022 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-34562053

RESUMO

BACKGROUND: The most frequently reported malignancies after solid organ transplant are cutaneous, but data on the risk in pediatric populations varies across studies. OBJECTIVES: To perform a systematic review including reported features and outcomes of skin cancers in pediatric solid organ transplant recipients. METHODS: EMBASE and MEDLINE were systematically searched (Prospero CRD42020201659). RESULTS: The review summarizes data from 20 studies on 337 patients, with a median age ranging from 15.0 to 19.5 years as reported in 4 studies, who developed skin malignancies after pediatric solid organ transplantation. Median ages at transplant and skin cancer diagnosis ranged from 1.5 to 17.0 years and 15.3 to 33.5 years, respectively. Squamous cell carcinoma (SCC) was most commonly reported (218 cases), followed by basal cell carcinoma (BCC) (91 cases), melanoma (18 cases), and unspecified keratinocyte carcinomas (2 cases). The median latency period between transplantation and cancer diagnosis ranged from 2.2 to 21.0 years. Overall, 4 studies reported 17 cases of metastasis in total, and recurrence was reported in one case. Six deaths were reported in one study related to SCC and melanoma metastases. The incidence rate of skin cancer after pediatric transplantation per 100 person-years of follow-up was 2.1 based on 5 studies. CONCLUSION: The most frequent post-transplant malignancy in pediatric organ transplant recipients was SCC.


Assuntos
Carcinoma/etiologia , Melanoma/etiologia , Transplante de Órgãos , Complicações Pós-Operatórias , Neoplasias Cutâneas/etiologia , Adolescente , Carcinoma/epidemiologia , Criança , Pré-Escolar , Humanos , Incidência , Lactente , Melanoma/epidemiologia , Complicações Pós-Operatórias/epidemiologia , Neoplasias Cutâneas/epidemiologia
9.
J Cutan Med Surg ; 26(2): 176-180, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-34587768

RESUMO

BACKGROUND: There is currently at least 1 biologic (adalimumab) approved in North America for treatment of Hidradenitis Suppurativa in the pediatric population. However, no reviews or clinical trials have specifically analyzed the effectiveness and safety data of biologic use in this population. The objective of this systematic review is to identify and summarize the outcomes of biologic therapy in pediatric patients with HS. METHODS: MEDLINE and EMBASE databases were used to conduct the search on Sept 18, 2020. RESULTS: The 15 included studies consisted of 26 patients, with the mean age of 15 ± 2.3 years. Females accounted for 53.8% (n = 14/26) of cases. The mean duration of HS prior to biologic initiation was 3.5 ± 2.9 years, with the majority having Hurley Stage II. The 26 patients received 34 biologics in total: 85.3% treated with TNF alpha inhibitors (adalimumab n = 17, infliximab n = 10, etanercept n = 1, unspecified n = 1), 5.9% with IL-12/23 inhibitors (ustekinumab n = 2), 5.9% with IL-1 inhibitors (i.e., anakinra n = 2) and 2.9% received IL-23 inhibitors (i.e., guselkumab n = 1) biologics. Of the 26 patients, 23.1% (n = 6/26) experienced complete resolution (CR), 73.1% (n = 19/26) experienced partial resolution (PR), and 3.8% (n = 1/26) had no resolution outcomes reported. The time to resolution of HS lesions after biologic initiation ranged from 10 days to 11.5 months (mean: 5.1 months). No adverse events were reported in the studies. CONCLUSION: Although anti-TNF alpha were the most common biologics used for HS in pediatric cases, large-scale trials specific to pediatric patients with HS are needed to confirm these findings.


Assuntos
Produtos Biológicos , Hidradenite Supurativa , Adalimumab/efeitos adversos , Adolescente , Produtos Biológicos/efeitos adversos , Criança , Feminino , Hidradenite Supurativa/tratamento farmacológico , Humanos , Infliximab/uso terapêutico , Inibidores do Fator de Necrose Tumoral
10.
J Am Acad Dermatol ; 85(1): 38-45, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-33689776

RESUMO

BACKGROUND: The distribution of pediatric-onset morphea and site-based likelihood for extracutaneous complications has not been well characterized. OBJECTIVE: To characterize the lesional distribution of pediatric-onset morphea and to determine the sites with the highest association of extracutaneous manifestations. METHODS: A retrospective cross-sectional study was performed. Using clinical photographs, morphea lesions were mapped onto body diagrams using customized software. RESULTS: A total of 823 patients with 2522 lesions were included. Lesions were more frequent on the superior (vs inferior) anterior aspect of the head and extensor (vs flexor) extremities. Linear morphea lesions were more likely on the head and neck, whereas plaque and generalized morphea lesions were more likely on the trunk. Musculoskeletal complications were more likely with lesions on the extensor (vs flexor) extremity (odds ratio [OR], 2.0; 95% confidence interval [CI], 1.2-3.4), whereas neurologic manifestations were more likely with lesions on the anterior (vs posterior) (OR, 2.8; 95% CI, 1.7-4.6) and superior (vs inferior) aspect of the head (OR, 2.3; 95% CI, 1.6-3.4). LIMITATIONS: Retrospective nature and the inclusion of only patients with clinical photographs. CONCLUSION: The distribution of pediatric-onset morphea is not random and varies with body site and within individual body sites. The risk stratification of extracutaneous manifestations by body site may inform decisions about screening for extracutaneous manifestations, although prospective studies are needed.


Assuntos
Transtornos da Cefaleia/epidemiologia , Doenças Musculoesqueléticas/epidemiologia , Esclerodermia Localizada/epidemiologia , Convulsões/epidemiologia , Idade de Início , Criança , Pré-Escolar , Estudos Transversais , Eletroencefalografia/estatística & dados numéricos , Feminino , Transtornos da Cefaleia/diagnóstico , Transtornos da Cefaleia/etiologia , Humanos , Imageamento por Ressonância Magnética/estatística & dados numéricos , Masculino , Doenças Musculoesqueléticas/diagnóstico , Doenças Musculoesqueléticas/etiologia , Fotografação , Estudos Retrospectivos , Medição de Risco/estatística & dados numéricos , Esclerodermia Localizada/complicações , Esclerodermia Localizada/diagnóstico , Convulsões/diagnóstico , Convulsões/etiologia , Pele/diagnóstico por imagem
11.
Pediatr Dermatol ; 38(5): 1086-1093, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-34402108

RESUMO

BACKGROUND: Paradoxical psoriasis occurs in pediatric patients following treatment with biologic agents. These presentations are not well described, and optimal treatment strategies have not been established. OBJECTIVE: To describe the reported rates, demographic characteristics, clinical presentation, and treatment options for TNF-α inhibitor-induced psoriasis. METHODS: Systematic review of published cases and cohort studies of paradoxical psoriasis induced by biologic agents, with specific reference to TNF-α inhibitors. RESULTS: We identified 4564 pediatric patients treated with TNF-α inhibitors, of whom 210 (4.6%) developed paradoxical psoriasis. Infliximab was the drug most likely to induce psoriasis (8.3%), followed by adalimumab (3.3%). Individual-level data were acquired from 129 individuals with a mean age of 13.6 years (SD: 4.0); 45.0% were male. The scalp was the most commonly affected area (47.5%), followed by the ears (30.8%). Most (63.3%) patients were continued on TNF-α inhibitor therapy. Among those who switched TNF-α inhibitors, only 32.0% had complete clearance of their skin lesions. Among patients who were switched to a non-TNF-α inhibitor, 81% had complete clearance of their paradoxical psoriasis. LIMITATIONS: Data were acquired from retrospective studies including case reports and case series. CONCLUSION: TNF-α inhibitor-induced psoriasis is a common adverse effect; however, most patients can continue their original therapy and be managed with skin-directed topical or systemic medications. If a patient requires medication discontinuation, switching to a new TNF-α inhibitor is unlikely to lead to resolution of their skin lesions.


Assuntos
Psoríase , Adalimumab/efeitos adversos , Adolescente , Criança , Humanos , Infliximab/efeitos adversos , Masculino , Psoríase/induzido quimicamente , Psoríase/tratamento farmacológico , Psoríase/epidemiologia , Estudos Retrospectivos , Fator de Necrose Tumoral alfa
12.
Pediatr Dermatol ; 38(6): 1532-1534, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-34647362

RESUMO

En coup de sabre form of morphea often affects the scalp with thickening, sclerosis, dyspigmentation, and scarring alopecia. Traditionally, it has been thought that the alopecia is not responsive to treatment and permanent. This report presents two cases with extensive, apparent scarring alopecia that improved with medical treatment.


Assuntos
Esclerodermia Localizada , Alopecia/diagnóstico , Alopecia/etiologia , Humanos , Esclerodermia Localizada/complicações , Esclerodermia Localizada/diagnóstico , Esclerodermia Localizada/tratamento farmacológico
13.
Pediatr Dermatol ; 38(2): 364-370, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33742457

RESUMO

BACKGROUND/OBJECTIVE: In spring 2020, high numbers of children presented with acral pernio-like skin rashes, concurrent with the coronavirus disease 2019 (COVID-19) pandemic. Understanding their clinical characteristics/ infection status may provide prognostic information and facilitate decisions about management. METHODS: A pediatric-specific dermatology registry was created by the Pediatric Dermatology COVID-19 Response Task Force of the Society for Pediatric Dermatology (SPD) and Pediatric Dermatology Research Alliance (PeDRA) and was managed by Children's Hospital of Philadelphia using REDCap. RESULTS: Data from 378 children 0-18 years entered into the registry between April 13 and July 17, 2020 were analyzed. Data were drawn from a standardized questionnaire completed by clinicians which asked for demographics, description of acral lesions, symptoms before and after acral changes, COVID-19 positive contacts, treatment, duration of skin changes, laboratory testing including SARS-CoV-2 PCR and antibody testing, as well as histopathology. 229 (60.6%) were male with mean age of 13.0 years (± 3.6 years). Six (1.6%) tested positive for SARS-CoV-2. Pedal lesions (often with pruritus and/or pain) were present in 96%. 30% (114/378) had COVID-19 symptoms during the 30 days prior to presentation. Most (69%) had no other symptoms and an uneventful course with complete recovery. CONCLUSIONS AND RELEVANCE: Children with acral pernio-like changes were healthy and all recovered with no short-term sequelae. We believe these acral changes are not just a temporal epiphenomenon of shelter in place during the spring months of the first wave of the COVID-19 pandemic and may be a late phase reaction that needs further study.


Assuntos
COVID-19 , Dermatologia/tendências , Pediatria/tendências , Dermatopatias/epidemiologia , Adolescente , Criança , Humanos , Masculino , Pandemias , Philadelphia , Sistema de Registros
14.
Adv Skin Wound Care ; 2021 Mar 12.
Artigo em Inglês | MEDLINE | ID: mdl-33797425

RESUMO

GENERAL PURPOSE: Forthcoming. TARGET AUDIENCE: This continuing education activity is intended for physicians, physician assistants, nurse practitioners, and nurses with an interest in skin and wound care. LEARNING OBJECTIVES/OUTCOMES: Forthcoming. ABSTRACT: This review article focuses on the pathogenesis, clinical features, and diagnostic testing of the common pathologies that can manifest as chilblains-like lesions. These differentials include COVID toes, Raynaud phenomenon, acrocyanosis, critical limb ischemia, thromboangiitis obliterans, chilblains associated with lupus erythematosus, and idiopathic chilblains. The authors present a helpful mnemonic, ARCTIC, to assist clinicians in recognition and diagnosis.

15.
Adv Skin Wound Care ; 34(7): 348-354, 2021 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-34125725

RESUMO

GENERAL PURPOSE: To familiarize wound care practitioners with the differential diagnoses of chilblains-like lesions that could be associated with the complications of COVID-19. TARGET AUDIENCE: This continuing education activity is intended for physicians, physician assistants, nurse practitioners, and nurses with an interest in skin and wound care. LEARNING OBJECTIVES/OUTCOMES: After participating in this educational activity, the participant will:1. Identify the population most often affected by COVID toes.2. Select the assessments that help differentiate the various conditions that cause chilblains-like lesions.3. Choose appropriate treatment options for the various conditions that cause chilblains-like lesions.


This review article focuses on the pathogenesis, clinical features, and diagnostic testing of the common pathologies that can manifest as chilblains-like lesions. These differentials include "COVID toes," Raynaud phenomenon, acrocyanosis, critical limb ischemia, thromboangiitis obliterans, chilblains associated with lupus erythematosus, and idiopathic chilblains. The authors present a helpful mnemonic, ARCTIC, to assist clinicians in recognition and diagnosis.


Assuntos
COVID-19/diagnóstico , Pérnio/diagnóstico , Dermatopatias/diagnóstico , COVID-19/complicações , Pérnio/patologia , Pérnio/virologia , Diagnóstico Diferencial , Dedos/patologia , Humanos , Dermatopatias/patologia , Dermatopatias/virologia , Avaliação de Sintomas , Dedos do Pé/patologia
16.
Pediatr Dermatol ; 37(3): 461-466, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32058621

RESUMO

BACKGROUND/OBJECTIVES: Stevens-Johnson syndrome and toxic epidermal necrolysis represent important sources of potential mortality and morbidity in children. There is a need for more clinical data in this population to determine whether specific treatments preferentially improve outcomes. METHODS: This was a single-center retrospective review of children admitted with drug-induced Stevens-Johnson syndrome, toxic epidermal necrolysis or Stevens-Johnson syndrome/toxic epidermal necrolysis overlap at a tertiary care pediatric institution in North America from 2008 to 2018. Patients without a dermatology assessment and diagnosis were excluded. Demographic, clinical, and treatment information were abstracted and reviewed for all included patients. RESULTS: Sixteen patients were identified, 43% female (7/16), with a mean age at presentation of 10.4 ± 5.2 years. Antibiotics were implicated in 56.3% of patients (9/16) and anticonvulsants in 31.3% (5/16). Sulfamethoxazole-trimethoprim was the triggering antibiotic in 31.3% of patients. The majority of patients were treated with intravenous immunoglobulin alone (50%, 8/16) or intravenous immunoglobulin with steroids (25%, 4/16). Etanercept was added to intravenous immunoglobulin and corticosteroid in a 2-year-old patient, resulting in clinical stabilization and halting of epidermolysis. No patients died. Clinical sequelae were noted in five patients, including ocular complications (n = 4), labial adhesions (n = 1), and persistent skin dyspigmentation (n = 3). CONCLUSIONS: Our results highlight that sulfamethoxazole-trimethoprim is an important cause of Stevens-Johnson syndrome-toxic epidermal necrolysis in children. Mortality was reassuringly low, but ocular sequelae were an important cause of morbidity. More data are needed to help determine whether specific treatments including etanercept may provide mortality or morbidity benefit in pediatric populations.


Assuntos
Síndrome de Stevens-Johnson , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Imunoglobulinas Intravenosas , Masculino , América do Norte , Estudos Retrospectivos , Síndrome de Stevens-Johnson/diagnóstico , Síndrome de Stevens-Johnson/tratamento farmacológico , Síndrome de Stevens-Johnson/etiologia , Atenção Terciária à Saúde
17.
Pediatr Dermatol ; 37(1): 246-247, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31830321

RESUMO

Acquired capillary malformations are rare vascular anomalies composed of dilated capillaries in the skin. We present a pediatric case of an acquired capillary malformation as a novel presentation of the PIK3CA-related overgrowth syndromes. Using next-generation sequencing, we identified a PIK3CA p.Val344Met mutation within the acquired capillary malformation with possible prognostic and therapeutic significance.


Assuntos
Capilares/anormalidades , Classe I de Fosfatidilinositol 3-Quinases/genética , Pele/irrigação sanguínea , Malformações Vasculares/genética , Adolescente , Biópsia , Capilares/patologia , Feminino , Humanos , Mutação , Pele/patologia , Malformações Vasculares/patologia
18.
Pediatr Dermatol ; 37(3): 484-489, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32110851

RESUMO

BACKGROUND: Neonatal lupus erythematosus (NLE) results from cross-placental transfer of maternal autoantibodies. Neonates can present with cardiac, cutaneous, hepatobiliary, hematologic, and neurologic complications from antibody-mediated organ toxicity. Scant evidence exists on long-term clinical characteristics and outcomes of patients with neonatal lupus. OBJECTIVES: To characterize the autoantibody profile, laboratory, and clinical features of patients with NLE. MATERIALS/METHODS: This was a single-site retrospective cohort study of patients at the Children's Hospital of Philadelphia with NLE. Data were collected on clinical, laboratory, and autoantibody profile at time of presentation, as well as long-term complications. RESULTS: Thirteen patients were included. Congenital cardiac findings were reported in 3 patients, with 1 having persistent cardiac sequelae. Cardiac manifestations were correlated with anti-Ro/SSA positivity in our cohort. Two patients had neurologic findings, with good long-term outcomes. Cutaneous findings were present in all patients, and many resolved without topical steroid treatment. Hematologic and hepatobiliary findings were common, but uncomplicated, with complete resolution by 6 months after initial presentation in all. Maternal rheumatologic disease, treatment, and race were not associated with systemic manifestations. CONCLUSIONS: Patients born to mothers with positive anti-Ro/SSA titers may benefit from routine cardiac monitoring in utero and at birth. Routine EEG or head ultrasound monitoring in patients who are autoantibody positive for NLE may be unnecessary. Information regarding long-term outcomes in NLE can be used to guide familial counseling and the use of serial laboratory testing.


Assuntos
Lúpus Eritematoso Cutâneo , Lúpus Eritematoso Sistêmico , Anticorpos Antinucleares , Criança , Feminino , Humanos , Recém-Nascido , Lúpus Eritematoso Cutâneo/diagnóstico , Lúpus Eritematoso Cutâneo/tratamento farmacológico , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/congênito , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Philadelphia , Gravidez , Estudos Retrospectivos
19.
J Cutan Med Surg ; 24(1): 73-78, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31691587

RESUMO

Pityriasis rubra pilaris (PRP) is an uncommon, inflammatory, papulosquamous skin disease. Treatment of PRP is challenging as the disease is often refractory to conventional therapies, such as retinoids and methotrexate. There has been an increasing number of studies reporting the successful use of biologic therapy in patients with PRP; however, the data on the efficacy and safety are limited. Our objective was to evaluate the existing evidence for utilizing biologics, whether alone or in combination with established systemic therapies, in patients with treatment-resistant PRP. We systematically reviewed evidence within Medline and Pubmed databases between January 1, 2000, to March 31, 2019. Articles consisted of patients diagnosed with PRP who have failed to respond sufficiently to first-line systemic therapies, or who had comorbidities that precluded their use. In total, 363 unique articles were identified, 56 of which were considered relevant to the clinical question. Of the 56 articles highlighted, 35 met the inclusion criteria and were limited to case series and case studies. Therapy with biologics was found to be successful for both monotherapy (81.1% [27/33]) and when used in combination with existing systemic therapies (87.5% [14/16]). The existing evidence suggests that biologics may be regarded as a tool for PRP treatment alone or in combination therapy with existing treatments, although large-scale randomized clinical trials are necessary to better assess their efficacy and safety.


Assuntos
Produtos Biológicos/uso terapêutico , Pitiríase Rubra Pilar/tratamento farmacológico , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Humanos , Pitiríase Rubra Pilar/metabolismo
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