RESUMO
BACKGROUND: Epidermolysis bullosa (EB), characterized by skin fragility and blistering, often requires hospitalization. Training for inpatient management of EB is limited, with no unified recommendations available in North America. OBJECTIVE: To develop consensus-derived best practices for hands-on inpatient management of EB in both the neonatal and postneonatal period. METHODS: A modified Delphi method (expert-based input via 2 surveys and a final review) was implemented. Available guidelines from EB Clinical Research Consortium centers were analyzed to determine areas of focus and formulate statements to be voted on by EB Clinical Research Consortium members, experienced EB nurses, and select family members. Study participants evaluated statements using a Likert scale: statements with at least 70% agreement were accepted; statements with 30% or more disagreement were rejected. RESULTS: Ten areas of focus were identified. Delphi participants included 15 dermatologists, 8 nurses, and 6 nonhealth care caregivers. Consensus was established on 103/119 neonatal statements and 105/122 postneonatal statements; no statements were rejected. Most recommendations applied to both age groups. LIMITATIONS: Recommendations may require adjustment based on individual patient's clinical context. CONCLUSION: Using the Delphi method, a consensus-derived resource for hospital-based health care professionals who manage patients with EB has been developed to improve the quality of inpatient care.
Assuntos
Consenso , Técnica Delphi , Epidermólise Bolhosa , Humanos , Recém-Nascido , Epidermólise Bolhosa/terapia , Hospitalização , Guias de Prática Clínica como Assunto , Lactente , Feminino , Dermatologia/métodos , Dermatologia/normas , MasculinoRESUMO
Sunscreen is an essential way to protect against photodamage from ultraviolet (UV) radiation. Despite the recognized benefits of sunscreen in preventing skin damage from UV light, its use varies across different patient groups. This cross-sectional, questionnaire-based study aims to uncover the sunscreen usage patterns, preferences, and barriers among non-Hispanic White (NHW) and skin of color (SOC) individuals. Our findings demonstrate that NHW individuals are more likely to wear sunscreen daily (31% NHW vs 25% SOC) and reapply sunscreen at least once a day (76% NHW vs 45% SOC) compared with SOC individuals. SOC individuals demonstrate a willingness to use sunscreen, but they face barriers such as cost (2% NHW vs 16% SOC), lack of knowledge in finding suitable products (22% NHW vs 41% SOC), and concerns about white cast (7% NHW vs 25% SOC). SOC individuals are less likely to know the difference between mineral and chemical sunscreen (49% NHW vs 29% SOC), less likely to learn about sunscreen from dermatologists (36% NHW vs 22% SOC), and more likely to prefer sunscreen from brands owned by people of color (13% NHW vs 47% SOC). In addition to analyzing the broader categories of NHW and SOC, subgroup analysis was conducted on specific subgroups, including Black, Asian, and Hispanic groups. Herein, we highlight differences in motivations, sunscreen preferences, sources of information, and knowledge levels about sun protection between NHW and SOC individuals. By uncovering the unique needs and challenges faced by SOC individuals, we aim to improve culturally competent patient education and promote effective sun protection practices across diverse populations. J Drugs Dermatol. 2024;23(6):456-462. doi:10.36849/JDD.8268.
Assuntos
Conhecimentos, Atitudes e Prática em Saúde , Preferência do Paciente , Protetores Solares , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Adulto Jovem , Estudos Transversais , Queimadura Solar/prevenção & controle , Protetores Solares/administração & dosagem , Inquéritos e Questionários , Raios Ultravioleta/efeitos adversos , Brancos , Negro ou Afro-Americano , Asiático , Hispânico ou LatinoRESUMO
Neonatal lupus erythematosus (NLE) is an autoimmune disease caused by the passive transfer of autoantibodies from mother to child during pregnancy. A rare complication of NLE is hemophagocytic lymphohistiocytosis (HLH), a potentially life-threatening hyperinflammatory state more commonly associated with other rheumatologic disorders. Herein, we describe a fatal case of NLE-associated HLH.
Assuntos
Lúpus Eritematoso Sistêmico , Linfo-Histiocitose Hemofagocítica , Recém-Nascido , Gravidez , Criança , Humanos , Feminino , Linfo-Histiocitose Hemofagocítica/diagnóstico , Linfo-Histiocitose Hemofagocítica/etiologia , Transmissão Vertical de Doenças Infecciosas , Lúpus Eritematoso Sistêmico/complicações , AutoanticorposRESUMO
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/microbiologiaRESUMO
BACKGROUND: Accurate diagnosis of epidermolysis bullosa (EB) has significant implications for prognosis, management, and genetic counseling. OBJECTIVE: To describe diagnostic testing patterns and assess diagnostic concordance of transmission electron microscopy (TEM), immunofluorescence mapping (IFM), and genetic analysis for EB. METHODS: A retrospective cohort included patients enrolled in the Epidermolysis Bullosa Clinical Characterization and Outcomes Database from January 1, 2004, to July 8, 2019. Tests concluding the same EB type (EB simplex, junctional EB, dominant dystrophic EB, and recessive dystrophic EB) were considered concordant; those concluding different EB types were considered discordant; and those with nonspecific/nondefinitive results were equivocal. RESULTS: A total of 970 diagnostic tests were conducted from 1984 to 2018 in 771 patients. Genetic analyses were performed chronologically later than IFM or TEM (P < .001). The likelihood of undergoing genetic analysis was greater for junctional EB and recessive dystrophic EB, and the same for dominant dystrophic EB as compared with EB simplex. TEM results in 163 patients were equivocal (55%), concordant (42%), and discordant (3%). IFM results in 185 patients were equivocal (54%), concordant (42%), and discordant (4%). LIMITATIONS: Retrospective design. CONCLUSIONS: Diagnostic testing has shifted in favor of genetic analysis. TEM and IFM frequently offer equivocal findings when compared to the specificity afforded by genetic analysis.
Assuntos
Epidermólise Bolhosa Distrófica , Epidermólise Bolhosa Simples , Epidermólise Bolhosa Juncional , Epidermólise Bolhosa , Epidermólise Bolhosa/diagnóstico , Epidermólise Bolhosa/genética , Epidermólise Bolhosa Distrófica/diagnóstico , Epidermólise Bolhosa Simples/diagnóstico , Imunofluorescência , Humanos , América do Norte , Estudos RetrospectivosRESUMO
Neonatal lupus erythematosus (NLE) is an autoimmune disease characterized by a periorbital erythematous rash. Although post-inflammatory hypopigmentation and telangiectasias are known possible sequelae, these features may be particularly noticeable in skin of color. Herein, we describe two infants with skin of color in whom periorbital hypopigmentation and telangiectasias were clues to the diagnosis of NLE.
Assuntos
Hipopigmentação , Lúpus Eritematoso Cutâneo , Lúpus Eritematoso Sistêmico , Telangiectasia , Anticorpos Antinucleares , Humanos , Hipopigmentação/diagnóstico , Lactente , Recém-Nascido , Lúpus Eritematoso Cutâneo/diagnóstico , Lúpus Eritematoso Sistêmico/congênito , Pigmentação da Pele , Telangiectasia/diagnóstico , Telangiectasia/etiologiaRESUMO
Multisystem inflammatory syndrome in children (MIS-C) is a syndrome associated with coronavirus disease 2019. Various phenotypes of MIS-C have been described including Kawasaki disease (KD). Although perineal desquamation is a known early sign of KD, to our knowledge, this rash has not yet been described in the KD phenotype of MIS-C. In this article, we report two patients in whom perineal desquamation was an early clue for the KD phenotype of MIS-C.
Assuntos
COVID-19/diagnóstico , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Períneo/patologia , Dermatopatias Infecciosas/patologia , Síndrome de Resposta Inflamatória Sistêmica/diagnóstico , Pré-Escolar , Diagnóstico Diferencial , Humanos , Lactente , Masculino , FenótipoRESUMO
BACKGROUND/OBJECTIVE: Herpes simplex virus (HSV) infection acquired in utero may present with non-vesicular dermatologic findings in affected newborns, which may pose a diagnostic dilemma. We aimed to describe and assess the range of non-vesiculobullous skin lesions that neonates with intrauterine HSV infection may manifest at birth. METHODS: We collected a multicenter case series and conducted a literature review of neonates with intrauterine HSV infection presenting with non-vesiculobullous cutaneous lesions. RESULTS: Twenty-two cases were reviewed, including six managed clinically by members of our team and 16 identified in the literature. Four (18%) were associated with twin pregnancies, and thirteen (59%) cases occurred in premature infants. Only four (18%) mothers had a documented history of HSV infection. Twelve (55%) cases resulted in poor outcomes, including long-term neurologic sequelae or death. Cutaneous manifestations included erosions, ulcerations, crusted papules or plaques, calcinosis cutis, excoriations, macules (erythematous, hypopigmented, or hyperpigmented), cutaneous atrophy, contractures, and bruising. About one-third of neonates developed new-onset vesicular lesions within a week of birth; in each of these cases, accurate diagnosis and therapy were delayed until appearance of vesicles. CONCLUSIONS: The range of dermatologic findings associated with intrauterine HSV is extremely broad, and the various morphologies present at birth likely reflect different stages of the ongoing evolution of an HSV infection that began in utero. Clinicians should have a low threshold for HSV testing in premature neonates born with atypical cutaneous lesions, since early detection and treatment of HSV may reduce morbidity and mortality from systemic complications.
Assuntos
Herpes Simples , Doenças do Recém-Nascido , Complicações Infecciosas na Gravidez , Anormalidades da Pele , Feminino , Herpes Simples/complicações , Herpes Simples/diagnóstico , Humanos , Recém-Nascido , Estudos Multicêntricos como Assunto , GravidezRESUMO
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 aureusRESUMO
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.
Assuntos
Exantema , Mucosite , Pneumonia por Mycoplasma , Criança , Família , Humanos , Mucosite/diagnóstico , Mycoplasma pneumoniae , Pneumonia por Mycoplasma/complicações , Pneumonia por Mycoplasma/diagnóstico , Pneumonia por Mycoplasma/tratamento farmacológicoRESUMO
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.
Assuntos
Detergentes/administração & dosagem , Epidermólise Bolhosa/terapia , Higiene da Pele , Administração Tópica , Adolescente , Adulto , Criança , Pré-Escolar , Cosméticos/administração & dosagem , Estudos Transversais , Bases de Dados Factuais , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Autocuidado , Adulto JovemRESUMO
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 TobagoRESUMO
BACKGROUND: Oral propranolol has been used to treat complicated infantile hemangiomas, although data from randomized, controlled trials to inform its use are limited. METHODS: We performed a multicenter, randomized, double-blind, adaptive, phase 2-3 trial assessing the efficacy and safety of a pediatric-specific oral propranolol solution in infants 1 to 5 months of age with proliferating infantile hemangioma requiring systemic therapy. Infants were randomly assigned to receive placebo or one of four propranolol regimens (1 or 3 mg of propranolol base per kilogram of body weight per day for 3 or 6 months). A preplanned interim analysis was conducted to identify the regimen to study for the final efficacy analysis. The primary end point was success (complete or nearly complete resolution of the target hemangioma) or failure of trial treatment at week 24, as assessed by independent, centralized, blinded evaluations of standardized photographs. RESULTS: Of 460 infants who underwent randomization, 456 received treatment. On the basis of an interim analysis of the first 188 patients who completed 24 weeks of trial treatment, the regimen of 3 mg of propranolol per kilogram per day for 6 months was selected for the final efficacy analysis. The frequency of successful treatment was higher with this regimen than with placebo (60% vs. 4%, P<0.001). A total of 88% of patients who received the selected propranolol regimen showed improvement by week 5, versus 5% of patients who received placebo. A total of 10% of patients in whom treatment with propranolol was successful required systemic retreatment during follow-up. Known adverse events associated with propranolol (hypoglycemia, hypotension, bradycardia, and bronchospasm) occurred infrequently, with no significant difference in frequency between the placebo group and the groups receiving propranolol. CONCLUSIONS: This trial showed that propranolol was effective at a dose of 3 mg per kilogram per day for 6 months in the treatment of infantile hemangioma. (Funded by Pierre Fabre Dermatologie; ClinicalTrials.gov number, NCT01056341.).
Assuntos
Antagonistas Adrenérgicos beta/administração & dosagem , Hemangioma/tratamento farmacológico , Propranolol/administração & dosagem , Administração Oral , Antagonistas Adrenérgicos beta/efeitos adversos , Relação Dose-Resposta a Droga , Método Duplo-Cego , Esquema de Medicação , Feminino , Humanos , Hipotensão/induzido quimicamente , Lactente , Masculino , Propranolol/efeitos adversos , Resultado do TratamentoRESUMO
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 , HumanosRESUMO
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/patologiaRESUMO
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 , CicatrizaçãoRESUMO
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.