RESUMO
BACKGROUND AND OBJECTIVE: Primary cutaneous lymphomas (PCL) are uncommon. Observations based on the first year of data from the Spanish Registry of Primary Cutaneous Lymphomas (RELCP, in its Spanish abbreviation) of the Spanish Academy of Dermatology and Venereology (AEDV) were published in February 2018. This report covers RELCP data for the first 5 years. PATIENTS AND METHODS: RELCP data were collected prospectively and included diagnosis, treatments, tests, and the current status of patients. We compiled descriptive statistics of the data registered during the first 5 years. RESULTS: Information on 2020 patients treated at 33 Spanish hospitals had been included in the RELCP by December 2021. Fifty-nine percent of the patients were men; the mean age was 62.2 years. The lymphomas were grouped into 4 large diagnostic categories: mycosis fungoides/Sézary syndrome, 1112 patients (55%); primary B-cell cutaneous lymphoma, 547 patients (27.1%); primary CD30+lymphoproliferative disorders, 222 patients (11%), and other T-cell lymphomas, 116 patients (5.8%). Nearly 75% of the tumors were registered in stage I. After treatment, 43.5% achieved complete remission and 27% were stable at the time of writing. Treatments prescribed were topical corticosteroids (1369 [67.8%]), phototherapy (890 patients [44.1%]), surgery (412 patients [20.4%]), and radiotherapy (384 patients [19%]). CONCLUSION: The characteristics of cutaneous lymphomas in Spain are similar to those reported for other series. The large size of the RELCP registry at 5 years has allowed us to give more precise descriptive statistics than in the first year. This registry facilitates the clinical research of the AEDV's lymphoma interest group, which has already published articles based on the RELCP data.
Assuntos
Dermatologia , Linfoma Cutâneo de Células T , Micose Fungoide , Neoplasias Cutâneas , Venereologia , Masculino , Humanos , Pessoa de Meia-Idade , Feminino , Linfoma Cutâneo de Células T/diagnóstico , Linfoma Cutâneo de Células T/epidemiologia , Linfoma Cutâneo de Células T/terapia , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/terapia , Sistema de Registros , Micose Fungoide/patologiaRESUMO
The discovery of new autoinflammatory syndromes and novel mutations has advanced at breakneck speed in recent years. Part 2 of this review focuses on vasculitis syndromes and the group of histiocytic and macrophage activation syndromes. We also include a table showing the mutations associated with these autoinflammatory syndromes and treatment alternatives.
Assuntos
Doenças Hereditárias Autoinflamatórias , Dermatopatias Genéticas , Doenças Autoimunes do Sistema Nervoso/genética , Doenças Autoimunes do Sistema Nervoso/imunologia , Criança , Gerenciamento Clínico , Doenças Hereditárias Autoinflamatórias/classificação , Doenças Hereditárias Autoinflamatórias/imunologia , Histiocitose/genética , Histiocitose/imunologia , Humanos , Interferon-alfa/fisiologia , Ativação de Macrófagos , Dermatopatias Genéticas/classificação , Dermatopatias Genéticas/imunologia , Vasculite/genética , Vasculite/imunologiaRESUMO
Monogenic autoinflammatory diseases are a heterogeneous emergent group of conditions that are currently under intensive study. We review the etiopathogenesis of these syndromes and their principal manifestations. Our aim is to propose a classification system based on the clinicopathologic features of typical skin lesions for routine clinical use in dermatology. Our focus is on diagnosis in pediatric practice given that this is the period when the signs and symptoms of these syndromes first appear. In Part 1 we discuss the course of urticaria-like syndromes, which include cryopyrin-associated periodic conditions and hereditary periodic fever syndromes. Pustular syndromes are also covered in this part. Finally, we review the range of therapies available as well as the genetic mutations associated with these autoinflammatory diseases.
Assuntos
Doenças Hereditárias Autoinflamatórias , Dermatopatias Genéticas , Autoanticorpos/imunologia , Autoantígenos/imunologia , Criança , Enzimas/genética , Enzimas/imunologia , Doenças Hereditárias Autoinflamatórias/classificação , Doenças Hereditárias Autoinflamatórias/imunologia , Humanos , Receptores de Citocinas/imunologia , Dermatopatias Genéticas/classificação , Dermatopatias Genéticas/imunologia , Úlcera Cutânea/genética , Úlcera Cutânea/imunologia , Urticária/classificação , Urticária/genética , Urticária/imunologiaRESUMO
Patients with autoimmune disorders are predisposed to develop a second immunologic disease, frequently with systemic involvement. We present a patient who developed lesions of discoid lupus erythematosus (DLE) limited to the face, and, concurrently, a linear morphoea involving her right axilla. No criteria for systemic lupus erythematosus or systemic scleroderma were present in the patient. To our knowledge, no patients with concomitant DLE and linear morphoea, without systemic involvement, have been previously reported in the literature.
Assuntos
Lúpus Eritematoso Discoide/patologia , Esclerodermia Localizada/patologia , Dermatopatias/patologia , Braço/patologia , Face/patologia , Feminino , Humanos , Pessoa de Meia-Idade , Doença Mista do Tecido Conjuntivo/patologiaRESUMO
BACKGROUND: Paclitaxel and docetaxel are antineoplastic drugs that bind the microtubules, producing the arrest of mitoses, which may be seen histopathologically. These histopathologic changes may simulate an intraepidermal keratinocytic malignant neoplasm, and an accurate diagnosis may be only established by clinicopathological correlation. OBJECTIVES: We report six cases of cutaneous eruptions by taxanes in which a striking cytotoxic effect was evident histopathologically. METHODS: Cutaneous biopsies were obtained in each patient. RESULTS: Atypical starburst-like or ring-like mitoses and dyskeratosis on basal and suprabasal layers of the epidermis. Areas of squamous syringometaplasia were also seen in one case. DISCUSSION: These findings were interpreted as expression of mitotic arrest due to taxanes. Similar changes have been described in association with other chemotherapeutic drugs such as vincristine, podophyllin and its derivative etoposide; colchicine, busulfan and maytansine, but cases like ours due to taxanes are exceptional or under-reported. CONCLUSION: Dermatopathologists should be aware of these effects in order to interpret carefully cutaneous biopsy specimens of patients receiving taxanes.
Assuntos
Hidrocarbonetos Aromáticos com Pontes/efeitos adversos , Exantema/induzido quimicamente , Neoplasias Cutâneas/diagnóstico , Pele/efeitos dos fármacos , Taxoides/efeitos adversos , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Pele/patologiaRESUMO
Stem cells are characterized by their ability to self-renew and differentiate into the different cell lineages of their tissue of origin. The discovery of stem cells in adult tissues, together with the description of specific markers for their isolation, has opened up new lines of investigation, expanding the horizons of biomedical research and raising new hope in the treatment of many diseases. In this article, we review in detail the main characteristics of the stem cells that produce the specialized cells of the skin (epidermal, mesenchymal, and melanocyte stem cells) and their potential implications and applications in diseases affecting the skin. Part I deals with the principal characteristics and potential applications of epidermal stem cells in dermatology.