RESUMO
Background: Recently two outcome instruments have been developed and validated for assessing cutaneous sarcoidosis in a live, in-person setting. Teledermatology is a rapidly growing field; yet, to date, no instrument has been validated for use in a remote setting, which could ultimately impact clinical trial design. Objective: To assess the interrater reliability of these outcome instruments for store-and-forward teledermatology. Methods: Seven sarcoidosis experts, including both pulmonologists and dermatologists, scored photographs of cutaneous sarcoidosis lesions in 13 patients utilizing the Cutaneous Sarcoidosis Activity and Morphology Index (CSAMI), the Sarcoidosis Activity and Severity Index (SASI) and the Physician Global Assessment (PGA). Interrater reliability was assessed for each instrument and was compared to results obtained from a prior study involving sarcoidosis experts evaluating the same patient population in an in-person setting. Results: Interrater reliability (presented as ICC [95%CI]) was poor for the CSAMI Activity scale (0.36 [0.16 - 0.65]) and the CSAMI Damage scale (0.17 [0.04 - 0.43]) and was fair for the Modified Facial SASI (0.59 [0.36 - 0.82]) and the PGA (0.47 [0.23 - 0.74]). All results were inferior to those obtained from the prior studies validating these instruments for in-person use. Conclusions: Given the superiority of these instruments when utilized in person, it is recommended to have an on-site sarcoidosis expert evaluate cutaneous sarcoidosis lesions whenever possible. (Sarcoidosis Vasc Diffuse Lung Dis 2017; 34: 165-169).
Assuntos
Neoplasias Hematológicas/epidemiologia , Pacientes Internados , Encaminhamento e Consulta/estatística & dados numéricos , Dermatopatias/diagnóstico , Dermatopatias/epidemiologia , Adulto , Distribuição por Idade , Idoso , Comorbidade , Bases de Dados Factuais , Dermatologia/normas , Dermatologia/tendências , Feminino , Neoplasias Hematológicas/diagnóstico , Hospitais Universitários , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Medição de Risco , Distribuição por SexoRESUMO
The clinical differential diagnosis of erythroderma is extensive and includes both benign and malignant causes. The authors present an exceptional case of erythroderma secondary to pre-B-cell lymphoblastic leukemia cutis, with diagnostic findings on biopsy.
Assuntos
Dermatite Esfoliativa/patologia , Infiltração Leucêmica/patologia , Leucemia-Linfoma Linfoblástico de Células Precursoras B/complicações , Pele/patologia , Idoso , Dermatite Esfoliativa/etiologia , Dermatite Esfoliativa/metabolismo , Evolução Fatal , Feminino , Humanos , Infiltração Leucêmica/etiologia , Pele/químicaRESUMO
The adverse effects of pegylated liposomal doxorubicin are primarily cutaneous. The majority of these cutaneous side effects manifest as palmar-plantar erythrodysesthesia, whereas few reports of other cutaneous reactions exist in the literature. The authors report a case of an exuberant follicular eruption in a patient treated with pegylated liposomal doxorubicin. This case also highlights the potential mechanism underlying this unusual histological and clinical reaction.
Assuntos
Antibióticos Antineoplásicos/efeitos adversos , Doxorrubicina/análogos & derivados , Toxidermias/etiologia , Toxidermias/patologia , Adulto , Cistadenocarcinoma Seroso/tratamento farmacológico , Doxorrubicina/efeitos adversos , Feminino , Humanos , Recidiva Local de Neoplasia/tratamento farmacológico , Neoplasias Ovarianas/tratamento farmacológico , Polietilenoglicóis/efeitos adversosAssuntos
Lúpus Eritematoso Cutâneo/tratamento farmacológico , Lúpus Eritematoso Cutâneo/patologia , Segurança do Paciente , Talidomida/análogos & derivados , Administração Oral , Biópsia por Agulha , Relação Dose-Resposta a Droga , Esquema de Medicação , Feminino , Seguimentos , Humanos , Imuno-Histoquímica , Lenalidomida , Masculino , Estudos Prospectivos , Índice de Gravidade de Doença , Talidomida/uso terapêutico , Fatores de Tempo , Resultado do TratamentoRESUMO
E-cadherin, a member of the cadherin family of transmembrane adhesion receptors, is critical for cutaneous barrier function, as it promotes keratinocyte and Langerhans cell adhesion in the epidermis. Recent murine models of chronic inflammation identified new E-cadherin expressing subsets of mononuclear phagocytes, including alternatively activated macrophages and selected inflammatory dendritic cells. It has been shown in vitro that expression of E-cadherin by murine macrophages promotes their homotypic aggregation and fusion to multinucleated giant cells (MNGCs), a signature cell type of granulomatous inflammation. The purpose of this study was to assess E-cadherin expression on histiocytes and giant cells in cutaneous granulomas in humans. E-cadherin expression was evaluated by immunohistochemistry of formalin-fixed paraffin-embedded skin biopsies of foreign body granulomas (n = 21) and sarcoidosis (n = 21). The results showed consistent membranous E-cadherin staining pattern on mononucleated histiocytes and MNGCs in both granuloma types. These E-cadherin expressing histiocytes are distinct from dermal Langerhans cells because they lacked CD1a expression. Our findings suggest that E-cadherin expressing mononuclear histiocytes are likely precursors for MNGCs in cutaneous granulomas and may play a critical role in disease pathogenesis.
Assuntos
Caderinas/análise , Células Gigantes/química , Granuloma de Corpo Estranho/metabolismo , Histiócitos/química , Sarcoidose/metabolismo , Dermatopatias/metabolismo , Pele/química , Antígenos CD , Antígenos CD1/análise , Biomarcadores/análise , Biópsia , Células Gigantes/patologia , Granuloma de Corpo Estranho/patologia , Histiócitos/patologia , Humanos , Imuno-Histoquímica , Sarcoidose/patologia , Pele/patologia , Dermatopatias/patologiaRESUMO
IMPORTANCE: A validated scoring system is essential to assess the effect of therapeutic interventions on a disease. The instrument introduced here captures sarcoidosis disease activity in a reliable, reproducible manner, which will help standardize clinical trial outcomes and allow comparative efficacy studies in the future and may help lead to more robust data regarding the effect of different treatments on cutaneous sarcoidosis. OBJECTIVE: To assess the reliability and convergent validity of the Cutaneous Sarcoidosis Activity and Morphology Instrument (CSAMI) and Sarcoidosis Activity and Severity Index (SASI) for evaluating cutaneous sarcoidosis outcomes. DESIGN AND SETTING: Cross-sectional study evaluating cutaneous sarcoidosis disease severity using CSAMI, SASI, and Physician's Global Assessment (PGA) as reference in the dedicated cutaneous sarcoidosis clinic of a teaching hospital. PARTICIPANTS: Eight dermatologists evaluating cutaneous sarcoidosis in 11 patients. INTERVENTION: Evaluation using the study instruments. MAIN OUTCOMES AND MEASURES: Primary outcomes included interrater and intrarater reliability and convergent validity; secondary outcomes, correlation with quality-of-life measures and time required for completion. RESULTS: All instruments demonstrated good to excellent intrarater reliability. Interrater reliability was excellent for CSAMI Activity scores (intraclass correlation coefficient, 0.82 [95% CI, 0.66-0.94]) and fair to poor for CSAMI Damage scores (0.42 [0.21-0.72]), modified Facial SASI (0.40 [0.17-0.72]), and PGA scores (0.40 [0.18-0.70]). CSAMI Activity and Damage scores and modified Facial SASI all demonstrated convergent validity with statistically significant correlations with PGA scores. Trends for correlations were seen between CSAMI scores and specific Skindex-29 quality-of-life domains. Although CSAMI required longer time to complete than SASI, both were scored within adequate time for use in clinical trials. CONCLUSIONS AND RELEVANCE: CSAMI appears to be a reliable and valid outcome instrument to measure cutaneous sarcoidosis and may capture a wide range of body surface and cutaneous morphologic types. This instrument can be adopted into clinical practice and clinical trials to allow physicians to assess the intensity of their patients' cutaneous sarcoidosis disease activity. Widespread use of one metric for disease severity assessment can help standardize the evaluation of the effect of various treatments on the disease. Future research is necessary to demonstrate its sensitivity to change and to confirm its correlation with quality-of-life measures.