Subject(s)
Hamartoma , Nevus , Skin Neoplasms , Humans , Nevus/diagnosis , Hamartoma/diagnosis , Skin Neoplasms/diagnosisABSTRACT
Childhood-onset psoriasis generally follows an indolent course but patients with moderate or severe disease may require systemic treatment. The aim of this study was to determine the relative proportion of children and young people aged up to 21 years with moderate to severe psoriasis in the BIOBADADERM registry and to analyze the characteristics of these patients, treatments used, and adverse events. Of the 3946 patients in the registry, 24 were aged 21 years or younger. They had mean age of 16.1 years on starting treatment. When the registry was started, they had a Psoriasis Area and Severity Index of 9.4 and 67% were being treated with a conventional systemic drug. Treatment was discontinued in 14 patients (58%) due to adverse events or a loss or lack of effectiveness. In conclusion, the BIOBADADERM registry shows that young people account for a small proportion of psoriasis patients receiving systemic treatment, and they are more likely to be treated using conventional systemic drugs.
Subject(s)
Biological Products , Psoriasis , Adolescent , Biological Products/therapeutic use , Child , Humans , Psoriasis/chemically induced , Psoriasis/drug therapy , Psoriasis/epidemiology , RegistriesABSTRACT
El comienzo de la psoriasis en la edad pediátrica, aunque generalmente leve, puede requerir tratamiento sistémico en las formas moderadas o graves de la enfermedad. El objetivo de este trabajo es analizar la frecuencia relativa, las características de los pacientes, el tratamiento empleado y los eventos adversos (EA) observados a partir del registro BIOBADADERM en niños y jóvenes con psoriasis moderada-grave. Del total de 3.946 pacientes del registro, se incluyen 24 pacientes menores de 21 años, con una edad media al inicio del tratamiento en BIOBADADERM de 16,1 años y un PASI medio de 9,4. El 67% de los pacientes estaba en tratamiento sistémico clásico al inicio del registro. Catorce pacientes (58%) suspendieron el tratamiento por pérdida o falta de eficacia o por EA. En conclusión, los datos del registro BIOBADADERM muestran que los menores representan un grupo muy pequeño dentro de los pacientes con psoriasis que reciben tratamiento sistémico y son manejados más frecuentemente con tratamientos clásicos (AU)
Childhood-onset psoriasis generally follows an indolent course but patients with moderate or severe disease may require systemic treatment. The aim of this study was to determine the relative proportion of children and young people aged up to 21 years with moderate to severe psoriasis in the BIOBADADERM registry and to analyze the characteristics of these patients, treatments used, and adverse events. Of the 3946 patients in the registry, 24 were aged 21 years or younger. They had mean age of 16.1 years on starting treatment. When the registry was started, they had a Psoriasis Area and Severity Index of 9.4 and 67% were being treated with a conventional systemic drug. Treatment was discontinued in 14 patients (58%) due to adverse events or a loss or lack of effectiveness. In conclusion, the BIOBADADERM registry shows that young people account for a small proportion of psoriasis patients receiving systemic treatment, and they are more likely to be treated using conventional systemic drugs (AU)
Subject(s)
Humans , Male , Female , Child , Adolescent , Young Adult , Psoriasis/epidemiology , Severity of Illness Index , Records , Spain/epidemiologyABSTRACT
Childhood-onset psoriasis generally follows an indolent course but patients with moderate or severe disease may require systemic treatment. The aim of this study was to determine the relative proportion of children and young people aged up to 21 years with moderate to severe psoriasis in the BIOBADADERM registry and to analyze the characteristics of these patients, treatments used, and adverse events. Of the 3946 patients in the registry, 24 were aged 21 years or younger. They had mean age of 16.1 years on starting treatment. When the registry was started, they had a Psoriasis Area and Severity Index of 9.4 and 67% were being treated with a conventional systemic drug. Treatment was discontinued in 14 patients (58%) due to adverse events or a loss or lack of effectiveness. In conclusion, the BIOBADADERM registry shows that young people account for a small proportion of psoriasis patients receiving systemic treatment, and they are more likely to be treated using conventional systemic drugs (AU)
El comienzo de la psoriasis en la edad pediátrica, aunque generalmente leve, puede requerir tratamiento sistémico en las formas moderadas o graves de la enfermedad. El objetivo de este trabajo es analizar la frecuencia relativa, las características de los pacientes, el tratamiento empleado y los eventos adversos (EA) observados a partir del registro BIOBADADERM en niños y jóvenes con psoriasis moderada-grave. Del total de 3.946 pacientes del registro, se incluyen 24 pacientes menores de 21 años, con una edad media al inicio del tratamiento en BIOBADADERM de 16,1 años y un PASI medio de 9,4. El 67% de los pacientes estaba en tratamiento sistémico clásico al inicio del registro. Catorce pacientes (58%) suspendieron el tratamiento por pérdida o falta de eficacia o por EA. En conclusión, los datos del registro BIOBADADERM muestran que los menores representan un grupo muy pequeño dentro de los pacientes con psoriasis que reciben tratamiento sistémico y son manejados más frecuentemente con tratamientos clásicos (AU)
Subject(s)
Humans , Male , Female , Child , Adolescent , Young Adult , Psoriasis/epidemiology , Severity of Illness Index , Records , Spain/epidemiologySubject(s)
Humans , Female , Adult , Nevus, Pigmented/diagnostic imaging , Exanthema , Skin Neoplasms/diagnostic imaging , DermoscopySubject(s)
Humans , Female , Adult , Nevus, Pigmented/diagnostic imaging , Exanthema , Skin Neoplasms/diagnostic imaging , DermoscopyABSTRACT
BACKGROUND: The 'rainbow pattern' was initially described as a highly specific dermoscopic feature of Kaposi sarcoma. Since then, it has been reported in many benign and malignant cutaneous tumours, including a few malignant melanomas (MMs). AIM: To determine the frequency and presentation of this dermoscopic pattern in primary cutaneous MMs in comparison to other cutaneous tumours. METHODS: The presence of a rainbow pattern was evaluated in a sample of 1100 dermoscopic images of different melanocytic and nonmelanocytic cutaneous neoplasms. RESULTS: The rainbow pattern was observed in 23 of 245 (9.4%) MM and 44 of 855 (5.1%) non-MM neoplasms. MMs presenting this feature were generally thicker: 82.6% > 1 mm and 43.0% > 2 mm. Compared with non-MMs, rainbow pattern in MMs was more commonly focal (82.7% vs. 36.4% nonfocal, P = 0.001) and associated with > 2 dermoscopic structures associated with MM (100% vs. 9% with fewer, P = 0.001). CONCLUSION: The rainbow pattern is a dermoscopic sign that can occasionally be observed in invasive MMs. In MMs, this feature is usually associated with other dermoscopic criteria of MM and located in a focal and eccentric area, as opposed to a diffuse and isolated presentation in non-MM neoplasms.
Subject(s)
Dermoscopy , Melanoma/pathology , Skin Neoplasms/pathology , Diagnosis, Differential , Humans , Melanoma/diagnosis , Retrospective Studies , Skin Neoplasms/diagnosisSubject(s)
Lymphoma, Large-Cell, Anaplastic , Lymphoma, Primary Cutaneous Anaplastic Large Cell , Skin Neoplasms , Dual-Specificity Phosphatases/genetics , Gene Rearrangement , Humans , Lymphoma, Large-Cell, Anaplastic/genetics , Lymphoma, Primary Cutaneous Anaplastic Large Cell/genetics , Mitogen-Activated Protein Kinase Phosphatases/genetics , Skin Neoplasms/geneticsABSTRACT
INTRODUCTION: Bednar tumor is a rare low-grade sarcoma considered the pigmented variant of dermatofibrosarco ma protuberans (DFSP). OBJECTIVE: To describe the clinical and histopathological characteristics, treatment and evolution of this rare neoplasm. CLINICAL CASE: A 9-year old female presented with a 2-year history of an indurated, asymptomatic papule on the back of her fourth left toe. The incisio nal biopsy was compatible with pigmented DFSP. The immunohistochemical study showed intense positivity for CD34 throughout the lesion, with negative factor XIIIa. We complemented the study with molecular cytogenetics (FISH) for PDGFB gene (22q13.1) which showed an abnormal pattern in tumor cells, but not in the melanocytes or the peritumoral skin. Delayed Mohs surgery and skin substitute dressing were performed without neoplastic recurrence at 5 years of follow up. Conclu sion: Pigmented DFSP is a low-grade sarcoma that is very rare in pediatric patients. The classical and pigmented variants should be suspected in the presence of a single papulonodular lesion of slow and progressive growth, with presence of spindle cells with storiform pattern in the biopsy and positive immunohistochemical study for CD34. It is an entity with good prognosis, with little risk of recurren ce and metastasis, if complete excision is achieved.
