RESUMO
OBJECTIVES: This study aims to evaluate the characteristics and treatment response of patients with pityriasis lichenoides seen in the last 43 years in a pediatric dermatology service. METHODS: This was a retrospective, analytical, longitudinal study of patients under 15 years of age. The medical records were reviewed and data were presented as frequencies, means and variances. Student's t-test, Mann-Whitney test, Fisher's exact test, Pearson/Yates chi-square test and multivariate logistic regression model were used, with p < 0.05 considered. RESULTS: 41 patients were included, 32 (78.0%) with pityriasis lichenoides chronica (PLC), five (12.2%) with pityriasis lichenoides et varioliformis acuta (PLEVA) and four (9.8%) with clinical PLC without biopsy. The age range of school children and adolescents was 19 (46.3%) and 13 (31.7%) respectively and 27 (65.8%) were male. Two peaks of the highest frequency were observed between 2004 and 2006 (10 patients - 24.4%) and another between 2019 and 2021 (6 patients - 14.7%). There was remission in 71.9% (n = 23), with 56.6% (n = 17) of those who used antibiotic therapy and 80% (n = 4) of those who had phototherapy. The chance of remission was 13 times greater in patients with disease onset after 5 years of age. CONCLUSIONS: The clinical form most commonly found was PLC mainly in school children and adolescents. The frequency peaks coincided with infectious outbreaks. The remission rate was satisfactory with antibiotic therapy, but higher with phototherapy. Remission was greater in patients with disease onset after 5 years of age.
Assuntos
Pitiríase Liquenoide , Humanos , Pitiríase Liquenoide/terapia , Pitiríase Liquenoide/tratamento farmacológico , Pitiríase Liquenoide/patologia , Criança , Masculino , Feminino , Adolescente , Estudos Retrospectivos , Pré-Escolar , Estudos Longitudinais , Resultado do Tratamento , Lactente , FototerapiaRESUMO
BACKGROUND: Lymphomatoid papulosis (LyP) is a rare cutaneous T-cell lymphoproliferative disorder. Comprehensive data on LyP in the paediatric population are scarce. OBJECTIVES: To characterize the epidemiological, clinical, histopathological and prognostic features of paediatric LyP. METHODS: This was a retrospective multicentre international cohort study that included 87 children and adolescents with LyP diagnosed between 1998 and 2022. Patients aged ≤ 18â years at disease onset were included. LyP diagnosis was made in each centre, based on clinicopathological correlation. RESULTS: Eighty-seven patients from 12 centres were included. Mean age at disease onset was 7.0â years (range 3â months-18â years) with a male to female ratio of 2 : 1. Mean time between the onset of the first cutaneous lesions and diagnosis was 1.3â years (range 0-14). Initial misdiagnosis concerned 26% of patients. LyP was most often misdiagnosed as pityriasis lichenoides et varioliformis acuta, insect bites or mollusca contagiosa. Erythematous papules or papulonodules were the most frequent clinical presentation. Pruritus was specifically mentioned in 21% of patients. The main histological subtype was type A in 55% of cases. When analysed, monoclonal T-cell receptor rearrangement was found in 77% of skin biopsies. The overall survival rate was 100%, with follow-up at 5â years available for 33 patients and at 15â years for 8 patients. Associated haematological malignancy (HM) occurred in 10% of cases (n = 7/73), including four patients with mycosis fungoides, one with primary cutaneous anaplastic large cell lymphoma (ALCL), one with systemic ALCL and one with acute myeloid leukaemia. If we compared incidence rates of cancer with the world population aged 0-19â years from 2001 to 2010, we estimated a significantly higher risk of associated malignancy in general, occurring before the age of 19â years (incidence rate ratio 87.49, 95% confidence interval 86.01-88.99). CONCLUSIONS: We report epidemiological data from a large international cohort of children and adolescents with LyP. Overall, the disease prognosis is good, with excellent survival rates for all patients. Owing to an increased risk of associated HM, long-term follow-up should be recommended for patients with LyP.
Lymphomatoid papulosis is a very rare skin condition caused by an abnormal increase in white blood cells (called 'lymphocytes') in the skin. The condition rarely affects children, so most of the scientific data published about this disease focuses on adults. This study involved 12 academic dermatology centres in Europe, the Middle East and North America, and gathered data from about 87 children who presented with symptoms of lymphomatoid papulosis before the age of 19â years. The aim of this study was to better describe this disease in the paediatric population and discuss its treatment options and evolution. We found that the presentation of the disease in children is roughly the same as in adults. Safe and effective treatment options exist. The disease is not life threatening, but it requires investigation by a dermatologist, both to make a careful diagnosis and to monitor it as sometimes associated cancers that originate from blood cells can occur, mostly on the skin.
Assuntos
Papulose Linfomatoide , Neoplasias Cutâneas , Humanos , Papulose Linfomatoide/patologia , Papulose Linfomatoide/epidemiologia , Masculino , Estudos Retrospectivos , Criança , Feminino , Adolescente , Pré-Escolar , Lactente , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/mortalidade , Idade de Início , Prognóstico , Erros de Diagnóstico/estatística & dados numéricos , Pitiríase Liquenoide/epidemiologia , Pitiríase Liquenoide/patologia , Pitiríase Liquenoide/diagnóstico , Mordeduras e Picadas de Insetos/epidemiologia , Mordeduras e Picadas de Insetos/complicações , Molusco Contagioso/epidemiologia , Molusco Contagioso/patologia , Molusco Contagioso/diagnósticoRESUMO
A healthy 2-year-old girl presented with multiple asymptomatic subcutaneous nodules on both legs. Histologically demonstrated calcium deposition within the dermis and subcutaneous tissue consistent with calcinosis cutis. Laboratory abnormalities, underlying genetic conditions, and potential triggering factors were ruled out. The lesions resolved over an 18-month period without treatment, emphasizing the importance of the wait-and-see approach in idiopathic cases of calcinosis cutis.
Assuntos
Calcinose Cutânea , Calcinose , Dermatopatias , Neoplasias Cutâneas , Feminino , Humanos , Pré-Escolar , Calcinose/diagnóstico , Calcinose/patologia , Gordura Subcutânea/patologia , Dermatopatias/diagnóstico , Dermatopatias/patologiaAssuntos
Transtornos da Pigmentação , Púrpura , Dermatopatias Vasculares , Dermatopatias , Humanos , Transtornos da Pigmentação/complicações , Transtornos da Pigmentação/diagnóstico , Púrpura/complicações , Estudos Retrospectivos , Dermatopatias/complicações , Dermatopatias/diagnóstico , Dermatopatias Vasculares/complicaçõesRESUMO
A 4-year-old girl presented with congenital patches of scalp alopecia, which on physical examination, was consistent with blaschkolinear alopecic patches with mild epidermal atrophy. Similar atrophic hypopigmented patches were seen on the trunk and proximal extremities. With the clinical suspicion of Conradi-Hünermann-Happle syndrome, genetic testing was performed and revealed a mutation in the EBP gene. Despite characteristic cutaneous findings, no skeletal, ocular, or other anomalies were found on further evaluation.
Assuntos
Condrodisplasia Punctata , Anormalidades da Pele , Alopecia , Pré-Escolar , Condrodisplasia Punctata/diagnóstico , Condrodisplasia Punctata/genética , Olho , Face , Feminino , HumanosRESUMO
A growing number of skin lesions during the COVID-19 pandemic are being recognized. Acral ischemic lesions identical to chilblains are most typical in children and young adults. We report an infant girl, aged 1 month and 29 days, with a peculiar reticulated purpuric eruption on her soles, with positive immunohistochemistry for SARS-CoV-2 in the endothelia of dermal blood vessels. The patient had an excellent outcome without specific therapy.
Assuntos
COVID-19/complicações , Pé/irrigação sanguínea , Púrpura/virologia , Feminino , Humanos , Lactente , SARS-CoV-2RESUMO
BACKGROUND: Different skin manifestations of COVID-19 are being reported. Acral lesions on the hands and feet, closely resembling chilblains, have been recognized during the peak incidence of the COVID-19 pandemic. MATERIAL AND METHODS: A retrospective review of 22 children and adolescents with chilblain-like lesions seen over a short period of time in the Emergency Department of a children's hospital during the peak incidence of COVID-19 in Madrid, Spain. RESULTS: All patients had lesions clinically consistent with chilblains of the toes or feet, with three also having lesions of the fingers. Pruritus and mild pain were the only skin symptoms elicited, and only 10 had mild respiratory and/or GI symptoms. None had fever. Coagulation tests, hemogram, serum chemistry, and lupus anticoagulant were normal in all patients tested. One out of 16 tested cases had elevated D-dimer results, but without systemic symptoms or other laboratory anomalies. SARS-CoV-2 PCR tested in 19 cases was positive in just one case. Skin biopsies obtained in six patients were consistent with chilblains. On follow-up, all cases showed spontaneous marked improvement or complete healing. CONCLUSION: Acute chilblains were observed during COVID-19 pandemic in children and teenagers. It is a mildly symptomatic condition with an excellent prognosis, usually requiring no therapy. Etiopathogenesis remains unknown.
Assuntos
Betacoronavirus , Pérnio/diagnóstico , Pérnio/virologia , Infecções por Coronavirus/complicações , Infecções por Coronavirus/diagnóstico , Pneumonia Viral/complicações , Pneumonia Viral/diagnóstico , Adolescente , COVID-19 , Pérnio/terapia , Criança , Infecções por Coronavirus/terapia , Dermoscopia , Feminino , Pé , Humanos , Masculino , Pandemias , Pneumonia Viral/terapia , Estudos Retrospectivos , SARS-CoV-2 , Espanha , Avaliação de Sintomas , Fatores de Tempo , Resultado do TratamentoRESUMO
During examination of cases of chilblains in children and adolescents, we identified four patients who also showed skin lesions similar to erythema multiforme (EM). They had no other known triggers for EM. One of them had a positive PCR for SARS-CoV-2, while the other three were negative. Skin biopsies from two patients showed features not typical of EM, such as deep perivascular and perieccrine infiltrate and absence of necrosis of keratinocytes. Immunohistochemistry for SARS-CoV/SARS-CoV-2 spike protein showed granular positivity in endothelial cells and epithelial cells of eccrine glands in both biopsies. All patients had an excellent outcome, and had minimal or no systemic symptoms. The coincidence of EM, a condition commonly related to viruses, and chilblains in the setting of COVID-19, and the positivity for SARS-CoV/SARS-CoV-2 spike protein by immunohistochemistry strongly suggest a link between EM-like lesions and SARS-CoV-2.
Assuntos
Betacoronavirus , Infecções por Coronavirus/complicações , Infecções por Coronavirus/patologia , Eritema Multiforme/diagnóstico , Eritema Multiforme/virologia , Pneumonia Viral/complicações , Pneumonia Viral/patologia , Adolescente , COVID-19 , Criança , Feminino , Humanos , Masculino , Pandemias , SARS-CoV-2RESUMO
The term mid-face toddler excoriation syndrome (MiTES) has been proposed to describe a rare condition that arises in infancy as is characterized by self-inflicted excoriations. It is considered to be a milder variant of hereditary sensory-autonomic neuropathy (HSAN) type VIII. We present an 8-year-old boy with lesions on the mid-face that were admittedly self-induced and associated with an abnormally high pain threshold. The diagnosis and management of MiTES is reviewed.
Assuntos
Neuropatias Hereditárias Sensoriais e Autônomas/diagnóstico , Proteínas de Transporte/genética , Criança , Traumatismos Faciais/etiologia , Neuropatias Hereditárias Sensoriais e Autônomas/genética , Neuropatias Hereditárias Sensoriais e Autônomas/psicologia , Humanos , Masculino , Proteínas do Tecido Nervoso/genética , Comportamento Autodestrutivo/etiologiaRESUMO
Papillary hemangioma (PH) is a rare, benign, vascular tumor that usually appears on the scalp and face and is reported most frequently in adults. We present a pediatric case of PH and provide sonographic features that may assist in establishing the diagnosis.
Assuntos
Hemangioma/diagnóstico por imagem , Neoplasias Cutâneas/diagnóstico por imagem , Bochecha , Criança , Feminino , Hemangioma/patologia , Humanos , Neoplasias Cutâneas/patologia , UltrassonografiaAssuntos
Síndromes Neoplásicas Hereditárias/diagnóstico por imagem , Síndromes Neoplásicas Hereditárias/patologia , Neoplasias Cutâneas/diagnóstico por imagem , Neoplasias Cutâneas/patologia , Ultrassonografia/métodos , Administração Tópica , Biópsia/métodos , Criança , Diagnóstico Diferencial , Feminino , Testes Genéticos/métodos , Humanos , Imunossupressores/administração & dosagem , Imunossupressores/uso terapêutico , Masculino , Mães , Síndromes Neoplásicas Hereditárias/tratamento farmacológico , Sirolimo/administração & dosagem , Sirolimo/uso terapêutico , Neoplasias Cutâneas/tratamento farmacológico , Resultado do Tratamento , Ultrassonografia Doppler em CoresAssuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Dermatite Esfoliativa/tratamento farmacológico , Interleucina-17/antagonistas & inibidores , Ceratodermia Palmar e Plantar/tratamento farmacológico , Linfócitos T Auxiliares-Indutores/fisiologia , Dermatite Esfoliativa/imunologia , Insuficiência de Crescimento/tratamento farmacológico , Feminino , Humanos , Hipotricose/tratamento farmacológico , Lactente , Interleucina-17/imunologia , Ceratodermia Palmar e Plantar/imunologia , Contagem de Linfócitos , Unhas Malformadas/tratamento farmacológico , SíndromeRESUMO
Viral exanthems are frequent in children and are mostly self-limited. Early recognition and differentiation from other childhood illnesses are important to direct further investigations and treatment initiation. The clinical presentation of viral exanthems in children includes a polymorphic spectrum of skin eruptions ranging from classic viral exanthems to "atypical" presentations that can mimic nonviral diseases; thus, viral exanthems of childhood can be readily diagnosed on clinical grounds, but not rarely do they represent a diagnostic challenge. In this review, we focus on viral diseases in children that may be difficult to diagnose due to their clinical similarities with nonviral diseases, and we offer clues for the differential diagnosis and proper diagnostic testing in such cases.
Assuntos
Exantema/diagnóstico , Exantema/virologia , Pele/virologia , Acrodermatite , Angiomatose , Febre de Chikungunya , Criança , Pré-Escolar , Técnicas de Laboratório Clínico , Dengue , Diagnóstico Diferencial , Exantema/patologia , Feminino , Doença de Mão, Pé e Boca , Herpesvirus Humano 4 , Humanos , Hospedeiro Imunocomprometido , Lactente , Masculino , Testes Sorológicos , Pele/patologia , Zika virusRESUMO
BACKGROUND: The superficial lymphatic component of vascular malformations poses a significant treatment challenge. It is responsible for the majority of symptoms presented, and to date, there is no consensus regarding treatment. OBJECTIVE: To evaluate the effectiveness of topical rapamycin in treating superficial lymphatic malformations (LM). METHODS: A case series study was performed of patients with superficial LM, treated with topical rapamycin. The clinical characteristics of patients and the concentration and application mode of the drug were recorded. The changes in the signs and symptoms observed and associated adverse effects were noted and analyzed. RESULTS: The study population consisted of 11 patients of an average age of 10.5 years. All were treated with topical rapamycin: 6 patients with a 1% concentration, 1 with a 0.8% concentration, and 4 with a 0.4% concentration. Changes in the clinical appearance of the lesions were observed in all patients. The associated symptoms, present in 9 of 11 patients, improved in every case. The mean follow-up time was 16.1 months. LIMITATIONS: This study is retrospective, with a small sample size and considerable heterogeneity of lesions and treatment approaches. CONCLUSION: Treatment with topical rapamycin modifies the clinical appearance and alleviates symptoms of superficial LM.
Assuntos
Imunossupressores/uso terapêutico , Anormalidades Linfáticas/diagnóstico , Anormalidades Linfáticas/tratamento farmacológico , Sirolimo/uso terapêutico , Administração Tópica , Adolescente , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Masculino , Prognóstico , Estudos Retrospectivos , Índice de Gravidade de Doença , Resultado do TratamentoAssuntos
Soluções para Diálise/efeitos adversos , Eritema/induzido quimicamente , Exantema/induzido quimicamente , Icodextrina/efeitos adversos , Diálise Peritoneal , Adulto , Idoso , Feminino , Dermatoses da Mão/induzido quimicamente , Humanos , Falência Renal Crônica/terapia , Masculino , Prurido/induzido quimicamenteRESUMO
Mastocytosis is a heterogeneous group of disorders with a variable clinical course, ranging from indolent disease with normal life expectancy to highly aggressive disease. In the skin, mast cells may show a spindle-shape appearance or appear as round cells with wide, polygonal cytoplasm. In this study, we present a case series of 4 patients with cutaneous childhood-onset mastocytosis in whom skin mast cells showed striking nuclear pleomorphism with bilobed and multilobed nuclei. Such finding does not seem to represent a malignant phenotype of the disease in the skin, although the true biological significance and the potential prognostic impact remain to be determined.