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1.
Pediatr Dermatol ; 37(3): 545-547, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32172537

RESUMO

Reactive infectious mucocutaneous eruption (RIME) was recently proposed to replace the term Mycoplasma pneumoniae (MP)-induced rash and mucositis to account for the fact that non-MP pathogens may also cause rash and mucositis. In this report, we describe a unique case of recurrent RIME featuring a total of three episodes. As two of the episodes demonstrated contemporaneous infection with MP and group A streptococcus or influenza B, this case lends further support to use of the term RIME. In addition, although RIME typically involves at least two mucous membranes, this case shows that recurrent episodes may fall into the rare exception in which mucositis is limited to one site.

2.
Pediatr Dermatol ; 37(2): 326-332, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31944391

RESUMO

BACKGROUND/OBJECTIVES: Epidermolysis bullosa (EB) comprises a group of inherited skin blistering diseases. There is currently no cure, and management includes skin protection and prevention of infection. To date, there has been no systematic investigation of home skin care practices among EB patients on a multicenter scale. METHODS: This cross-sectional, observational study included data collected from patients with EB enrolled in the Epidermolysis Bullosa Characterization and Clinical Outcomes Database (EBCCOD) who provided answers to a patient-directed questionnaire between January 1, 2017, and December 31, 2017. RESULTS: Of 202 respondents, 130 (64.4%) had dystrophic EB, 51 (25.2%) had EB simplex, 21 (7.4%) had junctional EB, 3 (1.5%) had Kindler syndrome, and 3 (1.5%) had an unspecified subtype. Seventy-eight patients reported cleansing in plain water only (39%). Of those who used an additive in their cleansing water, 75 (57%) added salt, 71 (54%) added bleach, 36 (27%) added vinegar, and 34 (26%) endorsed the use of an "other" additive (multiple additives possible). Reported concentrations of additives ranged widely from 0.002% sodium hypochlorite and 0.002% acetic acid solutions, which are thought to have negligible effects on microbes, to 0.09% sodium hypochlorite and 0.156% acetic acid, concentrations shown to be cytotoxic. One hundred eighty-eight patients answered questions regarding topical product use (93%). Of those, 131 reported topical antimicrobial use (70%). Mupirocin and bacitracin were the most commonly reported topical antibiotics (59, 58 [31.4%, 30.9%], respectively). CONCLUSIONS: These findings highlight the variety of skin care routines and frequent use of topical antimicrobials among EB patients and have potential implications for antibiotic resistance. The reported range of bleach and vinegar additives to cleansing water, including cytotoxic concentrations, emphasizes the need for clear and optimized skin cleansing recommendations.

4.
Pediatr Dermatol ; 36(1): e12-e16, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30338553

RESUMO

Infective dermatitis (ID) associated with Human T-cell leukemia virus type-1 (HTLV-1) is a rare form of severe superinfected eczema seen mostly in the Caribbean islands and Latin America. Although rapid response to antibiotic treatment is observed, patients should be monitored for development of complications associated with this retroviral infection, including T-cell leukemia/lymphoma (ATLL) and HTLV-1 associated myelopathy/tropical spastic paraparesis (HAM/TSP). Infective dermatitis is rarely seen in the United States and therefore may be under-recognized by physicians unfamiliar with this condition. Herein, we present an additional case report of an ID associated with HTLV-1 in an 11-year-old girl from Trinidad.


Assuntos
Infecções por HTLV-I/complicações , Dermatopatias Infecciosas/diagnóstico , Antibacterianos/uso terapêutico , Feminino , Glucocorticoides/uso terapêutico , Vírus Linfotrópico T Tipo 1 Humano/imunologia , Humanos , Pele/patologia , Dermatopatias Infecciosas/tratamento farmacológico , Dermatopatias Infecciosas/virologia , Trinidad e Tobago
5.
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
6.
Pediatr Dermatol ; 35(6): e416-e417, 2018 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-30152553

RESUMO

A 15-month-old boy presented with 1-4 cm, pink edematous plaques with overlying round erosions and hemorrhagic bullae in the setting of a gastrointestinal illness and was ultimately diagnosed with bullous-type Sweet syndrome. Despite appropriate treatment with oral steroids, the patient's cutaneous lesions healed with secondary anetoderma. This case should prompt practitioners to be aware of bullous-type Sweet syndrome and the possibility of lesions healing with postinflammatory scarring.


Assuntos
Anetodermia/etiologia , Síndrome de Sweet/diagnóstico , Anetodermia/patologia , Glucocorticoides/uso terapêutico , Humanos , Lactente , Masculino , Prednisolona/uso terapêutico , Pele/patologia , Síndrome de Sweet/complicações , Síndrome de Sweet/tratamento farmacológico , Cicatrização
7.
Pediatr Dermatol ; 35(4): 502-506, 2018 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-29691886

RESUMO

Langerhans cell histiocytosis is a rare group of disorders that results from the abnormal proliferation and accumulation of dendritic-derived cells in various organs of the body, such as the skin and bones. Hypopigmented macules are a rare cutaneous presentation of Langerhans cell histiocytosis that may pose a diagnostic dilemma when no other findings of Langerhans cell histiocytosis are present at the time of examination. We present 2 cases of the hypopigmented variant of Langerhans cell histiocytosis, including a case with histopathologic features of regression, and a review of the literature. These cases highlight the importance of including Langerhans cell histiocytosis in the differential diagnosis of an infant with hypopigmented macules and papules.


Assuntos
Histiocitose de Células de Langerhans/diagnóstico , Dermatopatias/diagnóstico , Diagnóstico Diferencial , Feminino , Glucocorticoides/uso terapêutico , Humanos , Hipopigmentação/etiologia , Lactente , Masculino , Pele/patologia , Dermatopatias/patologia
8.
J Cutan Pathol ; 45(1): 67-70, 2018 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-29023848

RESUMO

Folliculocystic and collagen hamartoma (FCCH) of tuberous sclerosis is a rare entity described in 2012 by Torrelo et al. with only 8 cases described, predominantly in males. It presents since birth or early infancy and in the majority of cases is associated with tuberous sclerosis. The hamartoma presents as an exophytic plaque and has distinctive histopathological features including hair follicles, intact or ruptured epidermal cysts, and an increased number of collagen fibers extending to the subcutaneous tissue. Herein we present an additional case of this rare entity in an 18-year-old female who met clinical criteria for tuberous sclerosis. The patient had an exophytic mass in the left temporal area for many years and wanted surgical excision due to its cosmetic appearance. Histopathology of the surgical specimen showed a hamartomatous lesion with multiple large intact epidermal cysts, hairs and increased thickened collagen. The patient has followed up for 1 year after the excision, with no recurrence. Additionally, we provide a literature review of known cases of FCCH as well as its clinical and histopathological differential diagnosis.


Assuntos
Hamartoma/etiologia , Hamartoma/patologia , Dermatoses do Couro Cabeludo/etiologia , Dermatoses do Couro Cabeludo/patologia , Esclerose Tuberosa/complicações , Adolescente , Feminino , Humanos
10.
Pediatr Dermatol ; 34(3): e137-e139, 2017 May.
Artigo em Inglês | MEDLINE | ID: mdl-28317165

RESUMO

Distinguishing benign acral nevi from small early acral melanomas may be challenging in certain cases. Dermoscopy is a noninvasive imaging technique that can help clinicians better visualize deeper lesion structures and thus more easily differentiate benign nevi from melanoma. We report the case of a 13-year-old girl with a changing dark brown to black macule with a central papular component on the volar surface of the right third finger. Dermoscopy revealed asymmetrically distributed irregular black blotches on a bluish-black background. Histopathology revealed a traumatized compound melanocytic nevus. Certain melanocytic nevi, although histologically benign, may not conform to the limited selection of reassuring benign dermoscopic patterns. Nevi in children are often dynamic and have a high likelihood of dermoscopic change.


Assuntos
Dermoscopia/métodos , Dedos/patologia , Melanoma/diagnóstico , Nevo Pigmentado/diagnóstico , Neoplasias Cutâneas/diagnóstico , Adolescente , Biópsia por Agulha , Diagnóstico Diferencial , Feminino , Seguimentos , Humanos , Imuno-Histoquímica , Melanoma/patologia , Nevo Pigmentado/patologia , Nevo Pigmentado/cirurgia , Lesões Pré-Cancerosas/diagnóstico , Lesões Pré-Cancerosas/cirurgia , Medição de Risco , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/cirurgia , Resultado do Tratamento
11.
Dermatol Online J ; 23(7)2017 Jul 15.
Artigo em Inglês | MEDLINE | ID: mdl-29469703

RESUMO

Pigmentation of the nail plate, or melanonychia, is typically a benign condition caused by melanocyte activation. Although rare, melanonychia may be the initial presentation of melanoma, thus all cases require an in-depth examination. Evaluation in pediatric patients can prove especially difficult as benign cases have a higher prevalence of atypia compared to adults. Lack of specific treatment guidelines in the pediatric population can make diagnosis and treatment challenging. We report a pediatric patient with melanonychia with atypical features that required significant evaluations and collaboration to ultimately reach a treatment plan.


Assuntos
Hiperpigmentação/patologia , Doenças da Unha/patologia , Criança , Humanos , Hiperpigmentação/cirurgia , Masculino , Doenças da Unha/cirurgia
12.
Pediatrics ; 139(1)2017 01.
Artigo em Inglês | MEDLINE | ID: mdl-27999114

RESUMO

Harlequin ichthyosis (HI) is the most severe phenotype of the autosomal recessive congenital ichthyoses. HI is caused by mutations in the lipid transporter adenosine triphosphate binding cassette A 12 (ABCA12). Neonates are born with a distinct clinical appearance, encased in a dense, platelike keratotic scale separated by deep erythematous fissures. Facial features are distorted by severe ectropion, eclabium, flattened nose, and rudimentary ears. Skin barrier function is markedly impaired, which can lead to hypernatremic dehydration, impaired thermoregulation, increased metabolic demands, and increased risk of respiratory dysfunction and infection. Historically, infants with HI did not survive beyond the neonatal period; however, recent advances in neonatal intensive care and coordinated multidisciplinary management have greatly improved survival. In this review, the authors combine the growing HI literature with their collective experiences to provide a comprehensive review of the management of neonates with HI.


Assuntos
Ictiose Lamelar/terapia , Terapia Intensiva Neonatal/métodos , Transportadores de Cassetes de Ligação de ATP/genética , Terapia Combinada , Análise Mutacional de DNA , Seguimentos , Humanos , Ictiose Lamelar/diagnóstico , Ictiose Lamelar/genética , Ictiose Lamelar/mortalidade , Lactente , Recém-Nascido , Comunicação Interdisciplinar , Colaboração Intersetorial , Fenótipo , Diagnóstico Pré-Natal , Prognóstico , Taxa de Sobrevida , Centros de Atenção Terciária
13.
Pediatr Dermatol ; 34(1): 33-38, 2017 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-27653748

RESUMO

BACKGROUND/OBJECTIVES: The past several decades have witnessed unprecedented advances in genomic technology, bringing genetic testing to the forefront of medical practice and moving us towards the practice of personalized medicine. Genetic testing has become an important aspect in preempting and successfully treating diseases in dermatology, yet difficulty remains in regards to obtaining genetic testing for patients. We conducted a survey for pediatric dermatologists in order to try to gauge and understand where difficulties lie in obtaining genetic testing and to analyze how best these issues can be resolved. METHODS: An 18-question survey was emailed to 480 dermatologists who have attended at least one of the last three annual Society for Pediatric Dermatology (SPD) meetings. RESULTS: Virtually all providers encountered at least one situation in which they required genetic testing for a patient (97.3% [n = 108]) and 37.4% indicated needing genetic testing more than six times per year. Of the respondents who had attempted to obtain genetic testing, half were unsuccessful in obtaining coverage more than 75% of the time (45% [n = 32]) and only 7.0% (n = 5) achieved success 75% to 100% of the time. The most common reasons for obtaining genetic testing included the need to provide an accurate diagnosis, followed by the need to provide prognostic information and appropriate medical management. CONCLUSION: The role of genetic testing in the practice of dermatology is expanding, yet obtaining coverage for genetic testing remains a challenge. We propose several solutions as to how this can be remedied.


Assuntos
Dermatologia/estatística & dados numéricos , Testes Genéticos/estatística & dados numéricos , Criança , Dermatologistas , Humanos , Pediatria , Inquéritos e Questionários
14.
Arch Dermatol Res ; 309(1): 1-10, 2017 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-27878387

RESUMO

The use of ionizing radiation is critical to cancer treatment and fluoroscopic procedures. However, despite efforts to minimize total radiation dose, many patients experience toxic cutaneous side-effects of ionizing radiation, ranging from mild erythema to subcutaneous fibrosis, telangiectasia formation, and ulceration. Extent of injury is highly variable among patients. Studying the genetic determinants of radiation injury can help develop protocols to reduce radiation toxicity, as well as drive research into effective modulators of the genes and gene products associated with radiation injury. Many studies in the past two decades have identified single-nucleotide polymorphisms that may be associated with susceptibility to cutaneous radiation injury, such as those in genes related to the following cellular responses to ionizing radiation: inflammation, DNA repair, oxidation and stress response, and cell-cycle and apoptosis. This review summarizes the current literature on potential major genes and polymorphisms, in the previously described damage response pathways, that are involved in susceptibility to cutaneous radiation injury. Potential pitfalls of current research and further avenues of discovery will be explored.


Assuntos
Polimorfismo de Nucleotídeo Único , Radiodermatite/genética , Apoptose/genética , Ciclo Celular/genética , Dano ao DNA , Estudos de Associação Genética , Marcadores Genéticos , Predisposição Genética para Doença , Genômica/métodos , Humanos , Mediadores da Inflamação/metabolismo , Estresse Oxidativo/genética , Fenótipo , Radiodermatite/diagnóstico , Radiodermatite/metabolismo , Radiografia Intervencionista/efeitos adversos , Radioterapia/efeitos adversos , Reprodutibilidade dos Testes , Fatores de Risco
16.
Dermatol Online J ; 22(6)2016 Jun 15.
Artigo em Inglês | MEDLINE | ID: mdl-27617597

RESUMO

Linear vesicles or papules in a newborn can be a presenting sign of incontinentia pigmenti (IP). In this report, we present two cases of neonates with cutaneous manifestations of incontinentia pigmenti. In one case, mild peripheral eosinophilia was noted. No extra-cutaneous manifestations were noted otherwise in both cases after complete ophthalmological and neurological evaluations. These cases serve as a reminder for clinicians to consider IP in newborns presenting with linear vesicles or papules.


Assuntos
Incontinência Pigmentar/patologia , Dermatoses da Perna/patologia , Feminino , Humanos , Incontinência Pigmentar/diagnóstico , Recém-Nascido , Dermatoses da Perna/diagnóstico
18.
Arch Dermatol Res ; 305(4): 269-82, 2013 May.
Artigo em Inglês | MEDLINE | ID: mdl-23377138

RESUMO

Sirtuins are a family of seven proteins in humans (SIRT1-SIRT7) that are involved in multiple cellular processes relevant to dermatology. The role of sirtuins in other organ systems is established. However, the importance of these proteins in dermatology is less defined. Recently, sirtuins gained international attention because of their role as "longevity proteins" that may extend and enhance human life. Sirtuins function in the cell via histone deacetylase and/or adenosine diphosphate ribosyltransferase enzymatic activity that target histone and non-histone substrates, including transcription regulators, tumor suppressors, structural proteins, DNA repair proteins, cell signaling proteins, transport proteins, and enzymes. Sirtuins are involved in cellular pathways related to skin structure and function, including aging, ultraviolet-induced photoaging, inflammation, epigenetics, cancer, and a variety of cellular functions including cell cycle, DNA repair and proliferation. This review highlights sirtuin-related cellular pathways, therapeutics and pharmacological targets in atopic dermatitis, bullous dermatoses, collagen vascular disorders, psoriasis, systemic lupus erythematosus, hypertrophic and keloid scars, cutaneous infections, and non-melanoma and melanoma skin cancer. Also discussed is the role of sirtuins in the following genodermatoses: ataxia telangiectasia, Cowden's syndrome, dyskeratosis congenita, Rubenstein-Taybi, Werner syndrome, and xeroderma pigmentosum. The pathophysiology of these inherited diseases is not well understood, and sirtuin-related processes represent potential therapeutic targets for diseases lacking suitable alternative treatments. The goal of this review is to bring attention to the dermatology community, physicians, and scientists, the importance of sirtuins in dermatology and provide a foundation and impetus for future discussion, research and pharmacologic discovery.


Assuntos
Dermatologia/tendências , Sirtuínas/fisiologia , Dermatopatias/fisiopatologia , Dermatopatias/terapia , Pesquisa Biomédica/tendências , Senescência Celular/fisiologia , Humanos , Envelhecimento da Pele/fisiologia , Terapêutica/tendências
19.
Clin Dermatol ; 29(6): 633-43, 2011.
Artigo em Inglês | MEDLINE | ID: mdl-22014985

RESUMO

Over the past several decades, improved technologies used in the care of hospitalized and outpatient pediatric populations have resulted in a decreased but still significant number of iatrogenic injuries. Children at the highest risk for cutaneous injury include those with the most immature skin barriers, such as neonates younger than 32 weeks of gestational age. Additional risk factors include low birth weight, increased length of hospital stay, and indwelling instrumentation. Also at risk are older children with compromised skin barriers owing to infectious disease (staphylococcal scalded skin syndrome), inflammatory disease (atopic dermatitis), drug eruptions, and inherited or acquired blistering disorders. This review highlights the presentation, course, and management of iatrogenic skin injury events in children.


Assuntos
Dermatologia/métodos , Doença Iatrogênica , Dermatopatias/etiologia , Pele/lesões , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Cuidado Pós-Natal/métodos , Gravidez , Lesões Pré-Natais/etiologia , Fatores de Risco , Dermatopatias/induzido quimicamente
20.
Int J Dermatol ; 49(4): 353-61, 2010 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-20465686

RESUMO

The genodermatoses encompass a range of inheritable skin diseases that may be associated with significant mortality and long-term morbidity. In the past, options for prenatal diagnosis of these diseases were limited to fetal skin biopsy. As a result of recent leaps made in genetics and molecular biology, DNA-based prenatal diagnosis is now available for an increasing number of genodermatoses, and newer non-invasive methods are being developed that have the potential for tremendous future impact in dermatology. Dermatologists caring for patients with genodermatoses should be aware of the options for screening and prenatal testing and partake in a multi-disciplinary approach to patient care.


Assuntos
Doenças Fetais/diagnóstico , Diagnóstico Pré-Natal/métodos , Dermatopatias Genéticas/diagnóstico , Epidermólise Bolhosa/diagnóstico , Feto/fisiologia , Humanos , Hiperceratose Epidermolítica/diagnóstico , Polimorfismo Genético , Diagnóstico Pré-Natal/tendências , Ultrassonografia Pré-Natal
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