ABSTRACT
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.
Subject(s)
Dermatology , Lymphoma, T-Cell, Cutaneous , Mycosis Fungoides , Skin Neoplasms , Venereology , Male , Humans , Middle Aged , Female , Lymphoma, T-Cell, Cutaneous/diagnosis , Lymphoma, T-Cell, Cutaneous/epidemiology , Lymphoma, T-Cell, Cutaneous/therapy , Skin Neoplasms/diagnosis , Skin Neoplasms/epidemiology , Skin Neoplasms/therapy , Registries , Mycosis Fungoides/pathologySubject(s)
Papilloma , Skin Neoplasms , Humans , Papilloma/diagnosis , Papilloma/therapy , Skin Neoplasms/diagnosis , Skin Neoplasms/therapyABSTRACT
No disponible
Subject(s)
Humans , Female , Pregnancy , Young Adult , Poxviridae Infections/diagnosis , Pregnancy Complications, Infectious/diagnosis , Pregnancy Complications, Infectious/virology , Skin Diseases, Viral/diagnosis , Skin Diseases, Viral/virologyABSTRACT
Las enfermedades monogénicas autoinflamatorias son un grupo de enfermedades emergentes y heterogéneas en continuo estudio y desarrollo en la actualidad. Nuestro objetivo es revisar estas enfermedades desde el punto de vista de su etiopatogenia y principales manifestaciones, con el fin de proponer una clasificación, basada en las características clinicopatológicas de las lesiones cutáneas típicas, que resulte de utilidad en la práctica clínica habitual de los dermatólogos. El texto está enfocado en el diagnóstico de estos síndromes durante la edad pediátrica, ya que es el periodo habitual de aparición de los primeros síntomas y signos. La primera parte de la revisión se centrará en el desarrollo de los síndromes urticariformes, que incluyen a su vez las criopirinopatías y los síndromes hereditarios asociados a fiebres periódicas, y de los síndromes pustulosos, resumiendo al final del texto las alternativas terapéuticas de estos síndromes autoinflamatorios y sus mutaciones genéticas (AU)
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 (AU)
Subject(s)
Humans , Child , Hereditary Autoinflammatory Diseases/genetics , Urticaria/diagnosis , Skin Diseases, Vesiculobullous/genetics , Mutation/genetics , Cryopyrin-Associated Periodic Syndromes/genetics , Skin Ulcer/genetics , Hereditary Autoinflammatory Diseases/classificationABSTRACT
El descubrimiento de nuevos síndromes autoinflamatorios y nuevas mutaciones está avanzando a una velocidad vertiginosa en los últimos años. La segunda parte de la revisión está centrada en el estudio de los síndromes histiocítico-macrofágicos y de los síndromes vasculopáticos, incluyendo al final del texto una tabla con las alternativas terapéuticas de estos síndromes autoinflamatorios y sus mutaciones genéticas (AU)
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 (AU)
Subject(s)
Humans , Histiocytic Disorders, Malignant/genetics , Hereditary Autoinflammatory Diseases/genetics , Peripheral Vascular Diseases/genetics , Mutation/genetics , Hereditary Autoinflammatory Diseases/classification , Vasculitis, Leukocytoclastic, Cutaneous/genetics , Telangiectasis/genetics , Complement C1q/deficiency , Adenosine Deaminase/deficiencySubject(s)
Collagen Diseases/diagnosis , Hodgkin Disease/complications , Skin Diseases/diagnosis , Adjustment Disorders/etiology , Adult , Antineoplastic Agents/administration & dosage , Antineoplastic Agents/therapeutic use , Collagen Diseases/etiology , Collagen Diseases/pathology , Female , Hodgkin Disease/drug therapy , Hodgkin Disease/pathology , Humans , Neoplasm Staging , Pruritus/diagnosis , Pruritus/etiology , Skin Diseases/pathology , Treatment OutcomeABSTRACT
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.
Subject(s)
Hereditary Autoinflammatory Diseases , Skin Diseases, Genetic , Autoantibodies/immunology , Autoantigens/immunology , Child , Enzymes/genetics , Enzymes/immunology , Hereditary Autoinflammatory Diseases/classification , Hereditary Autoinflammatory Diseases/immunology , Humans , Receptors, Cytokine/immunology , Skin Diseases, Genetic/classification , Skin Diseases, Genetic/immunology , Skin Ulcer/genetics , Skin Ulcer/immunology , Urticaria/classification , Urticaria/genetics , Urticaria/immunologyABSTRACT
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.
Subject(s)
Hereditary Autoinflammatory Diseases , Skin Diseases, Genetic , Autoimmune Diseases of the Nervous System/genetics , Autoimmune Diseases of the Nervous System/immunology , Child , Disease Management , Hereditary Autoinflammatory Diseases/classification , Hereditary Autoinflammatory Diseases/immunology , Histiocytosis/genetics , Histiocytosis/immunology , Humans , Interferon-alpha/physiology , Macrophage Activation , Skin Diseases, Genetic/classification , Skin Diseases, Genetic/immunology , Vasculitis/genetics , Vasculitis/immunologyABSTRACT
No disponible
Subject(s)
Humans , Male , Middle Aged , Skin Diseases, Vesiculobullous/diagnosis , Ecthyma, Contagious/diagnosis , Poxviridae/isolation & purification , Diagnosis, DifferentialABSTRACT
No disponible
Subject(s)
Humans , Male , Female , Middle Aged , Aged , Aged, 80 and over , Skin Diseases/chemically induced , Skin Diseases/complications , Enoxaparin/adverse effects , Enoxaparin/therapeutic use , Heparin, Low-Molecular-Weight/therapeutic use , Anticoagulants/therapeutic use , Pulmonary Disease, Chronic Obstructive/complications , Pulmonary Disease, Chronic Obstructive/diagnosis , Retinoblastoma/complicationsABSTRACT
No disponible
Subject(s)
Humans , Male , Female , Stem Cells/pathology , Stem Cells/physiology , Carcinoma, Basal Cell/diagnosis , Carcinoma, Basal Cell/genetics , Carcinoma, Basal Cell/pathology , Hair Follicle , Epidermis/anatomy & histology , Epidermis/pathology , Internship and Residency , Discussion ForumsSubject(s)
Anticoagulants/adverse effects , Enoxaparin/adverse effects , Skin Diseases, Vesiculobullous/chemically induced , Aged , Anticoagulants/therapeutic use , Antineoplastic Agents/adverse effects , Enoxaparin/therapeutic use , Female , Humans , Skin Diseases, Vesiculobullous/diagnosis , Thrombosis/chemically induced , Thrombosis/prevention & controlABSTRACT
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.
