RESUMEN
Extramammary Paget disease is a rare condition that most commonly affects the anogenital region in the elderly. The treatment of choice has been surgical excision of the affected area with adequate depth and lateral margins, criteria that cannot always be fulfilled, especially when the vulva, anal canal, or penis are involved. More recently radiotherapy has been suggested as a suitable treatment when surgical excision or other modalities are not appropriate. We report a case of anogenital extramammary Paget disease and the clinical response to treatment with radiotherapy. The aim of this article is to review relevant aspects of radiotherapy as a first-choice curative treatment in specific situations of anogenital extramammary Paget disease in situ.
Asunto(s)
Enfermedad de Paget Extramamaria/patología , Enfermedad de Paget Extramamaria/radioterapia , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/radioterapia , Anciano , Biopsia con Aguja , Femenino , Estudios de Seguimiento , Humanos , Inmunohistoquímica , Estadificación de Neoplasias , Enfermedad de Paget Extramamaria/diagnóstico , Perineo/patología , Dosificación Radioterapéutica , Enfermedades Raras , Medición de Riesgo , Neoplasias Cutáneas/diagnóstico , Resultado del Tratamiento , Vulva/patologíaRESUMEN
Specific studies on apremilast for nail psoriasis are lacking. Our objective was to evaluate the nail-specific patient-reported outcomes, clinical efficacy, ultrasound (US) parameters, and safety of apremilast for nail psoriasis. We conducted a prospective cohort study including adult patients with plaque and nail psoriasis with a fingernail Nail Psoriasis Severity Index (NAPSI) score of 12 or more. Patients were treated with apremilast 30 mg b.i.d. for 52 weeks. Forty-five patients were included. At week 52, the Nail Assessment in Psoriasis and Psoriatic Arthritis (NAPPA) Patient Benefit Index global weighted score was 2 or more in 52% of patients and NAPPA Quality of Life and fingernail NAPSI improved by 57% and 53%, respectively. US parameters improved from week 16 onwards. Target nail NAPSI improvements were higher for nail matrix scores (60%) than for nail bed scores (38%, p < 0.001). Baseline target nail bed NAPSI was associated with not achieving a target nail 50% reduction in NAPSI score at week 52 in the bivariate analysis (p = 0.024). Safety was consistent with the known apremilast profile. Results from apremilast therapy for 52 weeks in patients with psoriasis and predominant nail disease show significant improvements in nail-specific quality of life, clinical signs, and structural restoration on US, suggesting that apremilast may be considered in the treatment of nail psoriasis.
Asunto(s)
Enfermedades de la Uña , Psoriasis , Adulto , Humanos , Enfermedades de la Uña/diagnóstico por imagen , Enfermedades de la Uña/tratamiento farmacológico , Estudios Prospectivos , Psoriasis/diagnóstico por imagen , Psoriasis/tratamiento farmacológico , Calidad de Vida , Índice de Severidad de la Enfermedad , Talidomida/análogos & derivados , Resultado del Tratamiento , UltrasonografíaRESUMEN
Cobb syndrome is a rare non-inherited neurocutaneous disease, in which there are metameric cutaneous and spinal vascular malformations of the trunk. In cases of segmentally distributed multiple cutaneous vascular lesions, early diagnostic imaging of the spinal cord allows prompt intervention in order to reduce permanent neurological sequelae. We report case of Cobb syndrome in a 12-year-old boy and present a systematic review of the literature.
Asunto(s)
Síndromes Neurocutáneos/diagnóstico , Enfermedades Cutáneas Vasculares/diagnóstico , Columna Vertebral/irrigación sanguínea , Malformaciones Arteriovenosas/diagnóstico por imagen , Malformaciones Vasculares del Sistema Nervioso Central/diagnóstico por imagen , Niño , Humanos , Masculino , Síndromes Neurocutáneos/diagnóstico por imagen , Radiografía , Enfermedades Cutáneas Vasculares/diagnóstico por imagen , Columna Vertebral/diagnóstico por imagenRESUMEN
Actinic keratosis (AK) and field cancerization are increasing health problems insufficiently diagnosed by primary care physicians. The objective of this study was to assess the validity and reliability of teledermatology (TD) and teledermoscopy in the diagnosis of AK and field cancerization in a gatekeeper healthcare model. A prospective diagnostic test evaluation was done to assess the diagnostic concordance, accuracy, and performance parameters and the interobserver and intraobserver concordances of TD and teledermoscopy compared with dermatologists' face-to-face evaluation or histopathology. A total of 636 patients with 1,000 keratotic skin lesions were included. TD diagnostic concordance for AK and field cancerization evaluation was very high and superior to primary care physicians' diagnosis (92.4% vs. 62.4% and 96.7% vs. 51.8%, P < 0.001). TD sensitivity, specificity, and positive and negative predictive values for AK diagnosis and field cancerization were high (range = 82.2-95.0) and better than primary care physicians' diagnosis. Teledermoscopy yielded better results in diagnostic concordance, performance parameters, and AK subtypes. Intraobserver and interobserver agreement was >0.83. TD and, to a greater extent, teledermoscopy may be valid and reliable tools for the diagnosis of AK and field cancerization and may improve diagnosis and correct allocation and management in gatekeeper healthcare systems. It can be an alternative tool to training primary care physicians in direct diagnosis of these lesions.
Asunto(s)
Dermatología/métodos , Dermoscopía/métodos , Queratosis Actínica/diagnóstico , Neoplasias Cutáneas/diagnóstico , Telemedicina/métodos , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Masculino , Persona de Mediana Edad , Variaciones Dependientes del Observador , Estudios Prospectivos , Reproducibilidad de los ResultadosAsunto(s)
Dermatología/métodos , Diterpenos/administración & dosificación , Queratosis Actínica/tratamiento farmacológico , Piel/diagnóstico por imagen , Telemedicina , Administración Cutánea , Anciano , Anciano de 80 o más Años , Diterpenos/efectos adversos , Femenino , Humanos , Queratosis Actínica/diagnóstico , Queratosis Actínica/patología , Masculino , Reproducibilidad de los Resultados , Piel/efectos de los fármacos , Piel/patología , Resultado del TratamientoAsunto(s)
Anticuerpos Monoclonales/administración & dosificación , Psoriasis/diagnóstico , Psoriasis/tratamiento farmacológico , Centros Médicos Académicos , Adulto , Anticuerpos Monoclonales Humanizados , Relación Dosis-Respuesta a Droga , Esquema de Medicación , Femenino , Humanos , Inyecciones Subcutáneas , Masculino , Dosis Máxima Tolerada , Persona de Mediana Edad , Pronóstico , Estudios Prospectivos , Medición de Riesgo , Índice de Severidad de la Enfermedad , Resultado del TratamientoRESUMEN
BACKGROUND: Adalimumab is a fully human IgG1 monoclonal antibody that binds to tumor necrosis factor (TNF), a key proinflammatory cytokine involved in the pathogenesis of psoriasis. OBJECTIVE: A single-center, retrospective study was conducted to assess the efficacy and safety of adalimumab in patients with moderate to severe psoriasis in daily practice. METHODS: The medical records of 15 patients with moderate to severe psoriasis treated with adalimumab during a 1-year period were reviewed. Previous conventional systemic treatments or other biological agents were unsuccessful. All patients received subcutaneous injections of an initial dose of adalimumab (80 mg) at week 0 followed by adalimumab (40 mg) every other week. RESULTS: A 75% improvement in the Psoriasis Area and Severity Index (PASI 75) score was achieved by 80% of patients at week 24 and by 73.3% of patients at week 48. Moreover, 13.3% of patients were almost completely cleared (PASI 90) at week 48. At 24 weeks, adalimumab therapy increased significantly a patient's quality of life as assessed by the Dermatology Life Quality Index (DLQI) (p = 0.001). The Nail Psoriasis Severity Index (NAPSI) decreased from a mean (SD) of 18.9 (12.2) to 8.2 (4.7) (p = 0.001) at week 24. Palmoplantar psoriasis decreased from a mean score of 1.1 (1.3) to 0.5 (0.9) (p = 0.026) and scalp involvement from a mean of 2.5 (1.2) to 1.1 (1.0) (p = 0.002) at week 24. Out of 11 patients with pruritus at the pre-treatment visit, this symptom had completely disappeared in nine of them after 24 weeks of treatment. CONCLUSIONS: Treatment with adalimumab proved to be effective for the management of chronic moderate to severe plaque-type psoriasis in patients whose disease had been refractory to systemic conventional therapies or other biologic agents.