RESUMO

We report a case of difficult-to-control mycosis fungoides (MF), where the role of the dental surgeon was crucial for the control and prognosis of the disease. A 62-year-old female patient diagnosed with MF had a previous record of red patches and small raised bumps on the face, along with a cancerous growth in the cervical and vulvar region. The patient was initially treated with methotrexate and local radiotherapy without resolution. Chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisone was then started (CHOP protocol). The dental team of a reference hospital was consulted to evaluate swelling in the anterior region of the palate, which had been developing for two months, reporting discomfort when eating. The role of the dentistry team was fundamental in the differential diagnosis of oral lesions with dental infections, second neoplasia, or even a new site of disease manifestation, in addition to controlling mucosal changes resulting from chemotherapy. After ruling out dental infection, the dentistry team performed a lesion biopsy to confirm the diagnosis. The histopathological and immunohistochemical analysis showed atypical lymphoid infiltration of T cells (CD3+/CD4+/CD7-/CD8-), coexpression of CD25, and presence of CD30 cells, corresponding to the finding for MF. Identifying CD30 + allowed for a new chemotherapy protocol with brentuximab vedotin (BV) combined with gemcitabine. This protocol effectively controlled MF, which previous protocols had failed to do. The diagnosis by the dental team was essential for therapeutic change and improvement of the patient's clinical condition without the need for invasive medical procedures.

Assuntos

Protocolos de Quimioterapia Combinada Antineoplásica , Micose Fungoide , Humanos , Feminino , Pessoa de Meia-Idade , Micose Fungoide/patologia , Micose Fungoide/tratamento farmacológico , Micose Fungoide/terapia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/tratamento farmacológico , Doxorrubicina/uso terapêutico , Brentuximab Vedotin/uso terapêutico , Vincristina/uso terapêutico , Prednisona/uso terapêutico , Ciclofosfamida/uso terapêutico , Equipe de Assistência ao Paciente , Diagnóstico Diferencial , Neoplasias Palatinas/patologia , Neoplasias Palatinas/tratamento farmacológico

RESUMO

BACKGROUND: Mycosis fungoides is the most frequent form of cutaneous T-cell lymphoma. It is characterized by a chronic, slow, and progressive course, and is associated with mortality rates that depend on several factors, such as clinical staging. A median survival time of up to 13 months is found in patients with advanced stages that require more aggressive treatments, with greater toxicity and higher costs. In Latin America, few prognostic studies of the disease are available. OBJECTIVE: To determine the rate of progression from early stages (IA, IB, IIA) to more advanced stages (> IIB) in patients older than 18 years with mycosis fungoides treated at two medical centers in Colombia between January 1, 2010, and December 31, 2019. METHODS: Retrospective cohort study with a longitudinal design. RESULTS: 112 patients diagnosed with early mycosis fungoides were included. 56.2% were male (n = 63), with a median age of 53 years (IQR 43‒67). The most frequent clinical variant was classic (67.9%; n = 76), followed by folliculotropic (16%; n = 18), and hypopigmented (10.7%; n = 12). The most common first-line treatment was NB-UVB phototherapy (27.7%; n = 31), followed by PUVA phototherapy (25.8%; n = 29%), and topical corticosteroids (25%; n = 28). The global rate of disease progression was 8% (n = 9), with an overall mortality of 12.5% (n = 14). STUDY LIMITATIONS: Its retrospective design and the lack of molecular studies for case characterization. CONCLUSIONS: Early mycosis fungoides is a disease with a good prognosis in most patients, with a progression rate of 8% (n = 9).

Assuntos

Progressão da Doença , Micose Fungoide , Estadiamento de Neoplasias , Neoplasias Cutâneas , Humanos , Micose Fungoide/patologia , Micose Fungoide/terapia , Micose Fungoide/mortalidade , Masculino , Feminino , Estudos Retrospectivos , Pessoa de Meia-Idade , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/mortalidade , Neoplasias Cutâneas/terapia , Adulto , Idoso , Colômbia/epidemiologia , Estudos Longitudinais , Fatores de Risco , Prognóstico , Terapia PUVA , Fatores de Tempo , Terapia Ultravioleta

RESUMO

Introduction: Pruritus is a common symptom in dermatological practice. Affecting patients with a wide range of cutaneous and systemic diseases. It can be caused by cutaneous disorders, systemic diseases, neurological disorders, psychological disorders, medications, among others. When assessing individuals with pruritus and cutaneous lesions, it is essential to consider mycosis fungoides and granuloma annulare as noteworthy differential diagnoses. Case presentation: A 51-year-old female patient exhibited symptoms of pruritus and two occurrences of pruritic skin lesions. Accompanied by a low-grade fever measuring 37.7 ºC, as well as asthenia and myalgia. Physical examination revealed two rounded plaques with erythematous borders and multiple non-confluent papular lesions. Discussion: Differentiating between mycosis fungoides and granuloma annulare can be challenging due to the similarities in their clinical presentations. However, performing a biopsy is essential to reach a definitive diagnosis.Conclusions: A biopsy is being suggested for the front part of the left lower limb. The application of mometasone furoate twice a day for two weeks was prescribed. Subsequently, a meeting has been arranged to conduct a review and to carefully analyze the biopsy findings within thirty days.

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Introducción: El prurito es un síntoma frecuente en la práctica dermatológica, que afecta a pacientes con una amplia gama de enfermedades cutáneas y sistémicas. Puede estar causado por trastornos cutáneos, enfermedades sistémicas, trastornos neurológicos, trastornos psicológicos y medicamentos, entre otros. En la evaluación de personas con prurito y lesiones cutáneas, es fundamental tener en cuenta la micosis fungoide y el granuloma anular como diagnósticos diferenciales destacables. Presentación del caso clínico: Una paciente de 51 años de edad presentaba síntomas de prurito y dos apariciones de lesiones cutáneas pruriginosas, acompañadas de fiebre baja de 37.7 ºC, así como astenia y mialgias. El examen físico reveló dos placas redondeadas con bordes eritematosos y múltiples lesiones papulares no confluentes. Discusión: Diferenciar entre micosis fungoide y granuloma anular puede ser un reto debido a las similitudes en sus presentaciones clínicas. Sin embargo, la realización de una biopsia es esencial para llegar a un diagnóstico definitivo. Conclusiones:Se sugiere la realización de una biopsia en la parte anterior del miembro inferior izquierdo. Se prescribe la aplicación de furoato de mometasona dos veces al día durante dos semanas. Posteriormente, se ha concertado una reunión para realizar una revisión y deliberar sobre los resultados de la biopsia en un plazo de treinta días

Assuntos

Humanos , Feminino , Pessoa de Meia-Idade , Pele/lesões , Relatos de Casos , Micose Fungoide/diagnóstico

RESUMO

Immunobiologicals represent an innovative therapeutic option in dermatology. They are indicated in severe and refractory cases of different diseases when there is contraindication, intolerance, or failure of conventional systemic therapy and in cases with significant impairment of patient quality of life. The main immunobiologicals used in dermatology basically include inhibitors of tumor necrosis factor-alpha (anti-TNF), inhibitors of interleukin-12 and -23 (anti-IL12/23), inhibitors of interleukin-17 and its receptor (anti-IL17), inhibitors of interleukin-23 (anti-IL23), rituximab (anti-CD20 antibody), dupilumab (anti-IL4/IL13) and intravenous immunoglobulin. Their immunomodulatory action may be associated with an increase in the risk of infections in the short and long term, and each case must be assessed individually, according to the risk inherent to the drug, the patient general condition, and the need for precautions. This article will discuss the main risks of infection associated with the use of immunobiologicals, addressing the risk in immunocompetent and immunosuppressed patients, vaccination, fungal infections, tuberculosis, leprosy, and viral hepatitis, and how to manage the patient in the most diverse scenarios.

Assuntos

Anticorpos Monoclonais , Psoríase , Humanos , Anticorpos Monoclonais/uso terapêutico , Psoríase/tratamento farmacológico , Qualidade de Vida , Inibidores do Fator de Necrose Tumoral , Fator de Necrose Tumoral alfa , Interleucina-12 , Interleucina-23

RESUMO

BACKGROUND: An increased risk of Secondary Malignancies (SMs) in Mycosis Fungoides (MF) has been suggested previously. However, the relationship between this risk and the features of MF is not well-known. OBJECTIVE: To investigate the rate and types of SMs in a large cohort of MF patients focusing on the associated features of these patients. METHODS: The demographic features, subtype, and stage of MF, as well as the temporal relationship between the diagnosis of MF and the development of SMs were determined. Major clinical features of MF in this group were compared with MF patients without association of SMs. RESULTS: Among 730 MF patients with a mean follow-up period of 67.9 ± 52.4 months, 56 SMs were identified in a total of 52 (7.1%) patients. While 28.8% of patients were previously diagnosed with other malignancies, then subsequently had a diagnosis of MF, it was vice versa in 53.8% of patients. Most of the SM-associated MF patients had early-stage (80.7%) and classical type of MF (86.5%) without a significant difference from MF patients without association of SMs; 85.5% and 72.5%, respectively. The most commonly identified SMs were hematologic malignancies (64.3%) including lymphomatoid papulosis (n = 22), Hodgkin's lymphoma (n = 4), non-Hodgkin's lymphoma (n = 5), polycythemia vera (n = 2). Other most commonly associated malignancies were breast cancer (n = 4), prostate cancer (n = 3), renal cell carcinoma (n = 2), melanoma (n = 2), and Kaposi's sarcoma (n = 2). STUDY LIMITATIONS: A single tertiary dermatology center study with a retrospective design. CONCLUSION: Apart from the well-known lymphomatoid papulosis association, systemic hematological malignancies were also quite common in the large cohort of MF patients.

Assuntos

Micose Fungoide , Segunda Neoplasia Primária , Neoplasias Cutâneas , Humanos , Micose Fungoide/patologia , Micose Fungoide/epidemiologia , Masculino , Feminino , Pessoa de Meia-Idade , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/epidemiologia , Adulto , Segunda Neoplasia Primária/patologia , Segunda Neoplasia Primária/epidemiologia , Idoso , Estudos Retrospectivos , Fatores de Risco , Adulto Jovem , Estadiamento de Neoplasias , Adolescente , Idoso de 80 Anos ou mais , Fatores de Tempo , Seguimentos

RESUMO

Abstract Immunobiologicals represent an innovative therapeutic option in dermatology. They are indicated in severe and refractory cases of different diseases when there is contraindication, intolerance, or failure of conventional systemic therapy and in cases with significant impairment of patient quality of life. The main immunobiologicals used in dermatology basically include inhibitors of tumor necrosis factor-alpha (anti-TNF), inhibitors of interleukin-12 and -23 (anti-IL12/23), inhibitors of interleukin-17 and its receptor (anti-IL17), inhibitors of interleukin-23 (anti-IL23), rituximab (anti-CD20 antibody), dupilumab (anti-IL4/IL13) and intravenous immunoglobulin. Their immunomodulatory action may be associated with an increase in the risk of infections in the short and long term, and each case must be assessed individually, according to the risk inherent to the drug, the patient general condition, and the need for precautions. This article will discuss the main risks of infection associated with the use of immunobiologicals, addressing the risk in immunocompetent and immunosuppressed patients, vaccination, fungal infections, tuberculosis, leprosy, and viral hepatitis, and how to manage the patient in the most diverse scenarios.

RESUMO

Abstract Background An increased risk of Secondary Malignancies (SMs) in Mycosis Fungoides (MF) has been suggested previously. However, the relationship between this risk and the features of MF is not well-known. Objective To investigate the rate and types of SMs in a large cohort of MF patients focusing on the associated features of these patients. Methods The demographic features, subtype, and stage of MF, as well as the temporal relationship between the diagnosis of MF and the development of SMs were determined. Major clinical features of MF in this group were compared with MF patients without association of SMs. Results Among 730 MF patients with a mean follow-up period of 67.9 ± 52.4 months, 56 SMs were identified in a total of 52 (7.1%) patients. While 28.8% of patients were previously diagnosed with other malignancies, then subsequently had a diagnosis of MF, it was vice versa in 53.8% of patients. Most of the SM-associated MF patients had early-stage (80.7%) and classical type of MF (86.5%) without a significant difference from MF patients without association of SMs; 85.5% and 72.5%, respectively. The most commonly identified SMs were hematologic malignancies (64.3%) including lymphomatoid papulosis (n = 22), Hodgkin's lymphoma (n = 4), non-Hodgkin's lymphoma (n = 5), polycythemia vera (n = 2). Other most commonly associated malignancies were breast cancer (n = 4), prostate cancer (n = 3), renal cell carcinoma (n = 2), melanoma (n = 2), and Kaposi's sarcoma (n = 2). Study limitations A single tertiary dermatology center study with a retrospective design. Conclusion Apart from the well-known lymphomatoid papulosis association, systemic hematological malignancies were also quite common in the large cohort of MF patients.

RESUMO

Abstract Background Mycosis fungoides is the most frequent form of cutaneous T-cell lymphoma. It is characterized by a chronic, slow, and progressive course, and is associated with mortality rates that depend on several factors, such as clinical staging. A median survival time of up to 13 months is found in patients with advanced stages that require more aggressive treatments, with greater toxicity and higher costs. In Latin America, few prognostic studies of the disease are available. Objective To determine the rate of progression from early stages (IA, IB, IIA) to more advanced stages (> IIB) in patients older than 18 years with mycosis fungoides treated at two medical centers in Colombia between January 1, 2010, and December 31, 2019. Methods Retrospective cohort study with a longitudinal design. Results 112 patients diagnosed with early mycosis fungoides were included. 56.2% were male (n = 63), with a median age of 53 years (IQR 43‒67). The most frequent clinical variant was classic (67.9%; n = 76), followed by folliculotropic (16%; n = 18), and hypopigmented (10.7%; n = 12). The most common first-line treatment was NB-UVB phototherapy (27.7%; n = 31), followed by PUVA phototherapy (25.8%; n = 29%), and topical corticosteroids (25%; n = 28). The global rate of disease progression was 8% (n = 9), with an overall mortality of 12.5% (n = 14). Study limitations Its retrospective design and the lack of molecular studies for case characterization. Conclusions Early mycosis fungoides is a disease with a good prognosis in most patients, with a progression rate of 8% (n = 9).

RESUMO

Background: Common variable immunodeficiency disorders (CVIDs), which are primary immunodeficiencies characterized by the failure of primary antibody production, typically present with recurrent bacterial infections, decreased antibody levels, autoimmune features, and rare atypical manifestations that can complicate diagnosis and management. Although most cases are sporadic, approximately 10% of the patients may have a family history of immunodeficiency. Genetic causes involving genes related to B-cell development and survival have been identified in only a small percentage of cases. Case presentation: We present the case of a family with two brothers who presented with mycosis fungoides as an exclusive symptom of a common variable immunodeficiency disorder (CVID). Whole-exome sequencing of the index patient revealed a pathogenic variant of the NFKB2 gene. Based on this diagnosis and re-evaluation of other family members, the father and brother were diagnosed with this rare immune and preneoplastic syndrome. All CVID-affected family members presented with mycosis fungoides as their only symptom, which is, to the best of our knowledge, the first case to be reported. Conclusion: This case highlights the importance of high-throughput sequencing techniques for the proper diagnosis and treatment of hereditary hematological disorders.

RESUMO

Paciente de 44 años de sexo femenino, sin ninguna enfermedad de base preexistente, con una historia de aproximadamente diez meses de presentar lesiones eritemato-descamativas pruriginosas inicialmente localizadas en extremidades inferiores y que luego se generalizaron en todo el cuerpo, asociándose a la pérdida de peso de aproximadamente 15 kg. El manejo inicial consistió en corticoides tópicos y antihistamínicos orales con poca respuesta clínica. Se inició el estudio por dermatología y se confirmó el diagnóstico inicial de neoplasia cutánea maligna de células T. Luego se realizó el frotis de médula ósea, en el que se identificaron células «cerebriformes¼ que confirmaron el diagnóstico de síndrome de Sézary. La paciente recibió esquema de quimioterapia ciclofosfamida, doxorrubicina, vincristina, etopósido y prednisona. La respuesta inicial fue favorable, con alta hospitalaria y seguimiento en la consulta externa. Transcurridos tres meses de tratamiento, la paciente consultó por episodio febril, tos productiva más distrés respiratorio asociado a estertores basales bilaterales, presentó insuficiencia respiratoria y durante la inducción a la ventilación mecánica sufrió un paro cardiorrespiratorio y falleció

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44-year-old female patient, with no preexisting underlying disease, with a history of approximately ten months of presenting pruritic erythematous-desquamative lesions initially localized in the lower extremities and later generalized throughout the body, associated with weight loss of 15 kg. Treatment. Initial management consisted of topical corticosteroids and oral antihistamines with little clinical response. A dermatology wok-up was initiated, and the initial diagnosis of malignant T-cell neoplasm was confirmed. A bone marrow smear was performed, in which "cerebriform" cells were identified, confirming the diagnosis of Sézary syndrome. The patient received cyclophosphamide, doxorubicin, vincristine, etoposide, and prednisone chemotherapy. Outcome. The initial response was favorable, with hospital discharge and outpatient follow-up. After three months of treatment, the patient consulted for a febrile episode, productive cough plus respiratory distress associated with bilateral basal rales, presented respiratory failure, and during induction of mechanical ventilation suffered cardiorespiratory arrest and died.

Assuntos

Humanos , El Salvador

RESUMO

Background: Lymphoma neoplasms originate from the lymphocytes. Anatomically, these tumors can be classified into multicentric, digestive, mediastinal, and cutaneous forms. The etiology of cutaneous lymphoma remains unclear; however, it has been associated with chronic skin inflammation. The definitive diagnosis is based on histological analysis and immunohistochemistry, although fine-needle aspiration cytology has shown good results. The aim of this paper is to describe the clinicopathological aspects of a case of cutaneous epitheliotropic T cell lymphoma, classified as mycosis fungoides, in a Lhasa Apso dog. Case: A 8-year-old bitch Lhasa Apso with multiple non-pruritic skin nodules and history of 10-day evolution was referred to the Veterinary Hospital of the Centro Universitário do Espírito Santo (UNESC), Colatina, ES, Brazil. The nodules were erythematous, exophytic, firm, circumscribed, and measured 0.2-4 cm in diameter in locations throughout the animal's body. An incisional biopsy was performed with an 8-mm punch and sent for histopathological examination. An infiltrative, poorly demarcated, non-encapsulated, densely cellular neoplasm, which was replacing the dermal collagen and displacing the adnexa, was observed in the dermis. The tumor was composed of a population of round cells, with generally distinct cell borders and a small-to-moderate amount of eosinophilic cytoplasm. The nuclei were irregularly rounded and occasionally edentulous, with vesicular chromatin, a visible nucleus, and 11 mitotic figures in an area of 2.37 mm2 . The immunohistochemical test, which was positive for the CD3 marker, confirmed the diagnosis of T cell lymphoma. On an ultrasound to identify metastasis, the liver showed heterogeneous parenchyma, heterogeneous expansive formation, areas of cavitary appearance, and cytology compatible with lymphoma. Antineoplastic chemotherapy was administered using the CHOP regimen (cyclophosphamide, doxorubicin, vincristine, and prednisone). However, the animal died after 45 days. Discussion: A diagnosis of the mycosis fungoides type of cutaneous epitheliotropic T cell lymphoma was established based on clinical, laboratory, anatomopathological, and immunohistochemical findings. Pruritus is a common clinical condition in animals with mycosis fungoides, particularly in those with the erythrodermic form of the disease. Epitheliotropic lymphomas have no sexual or racial predilections and usually affect dogs over 9 years of age. The Cocker Spaniel, English Bulldog, Boxer, Golden Retriever, Scottish Terrier, Briard, English Springer Spaniel, Beagle, German Shepherd, and English Cocker Spaniel breeds are frequently affected by these lymphomas. These neoplasms can have a primary skin origin, or they can be secondary and associated with lymphoma found elsewhere in the body. Chemotherapy is the treatment of choice, especially in cases with multifocal distribution. Protocol preference varies with disease stage, patient clinical and laboratory conditions, and the degree of toxicity. Commonly used chemotherapy regimens include L-CHOP (vincristine, cyclophosphamide, doxorubicin, L-asparaginase, and prednisolone), CHOP, COP (cyclophosphamide, vincristine, and prednisone), LAP (lomustine, L-asparaginase, and prednisolone), LOPP (lomustine, vincristine, procarbazine, prednisolone), chlorambucil, and prednisolone. The prognosis of canine epitheliotropic cutaneous lymphoma is unfavorable, with a survival time ranging from a few months to 2 years. The animal in this study survived for 105 days. In addition, epitheliotropic cutaneous T cell lymphoma is aggressive, which may result in a shorter survival time in animals affected by this type of tumor.

Assuntos

Animais , Feminino , Cães , Neoplasias Cutâneas/veterinária , Linfoma Cutâneo de Células T/veterinária , Micose Fungoide/veterinária , Epitélio/patologia , Imuno-Histoquímica/veterinária

RESUMO

BACKGROUND: Mycosis fungoides (MF), the most common subtype of Cutaneous T-cell lymphomas, is caused by malignant T-cell proliferations in the skin that can invade blood, lymph nodes, or viscera. Currently, data on efficacy of maintenance therapies in MF are lacking. We developed a unique protocol to use chlormethine/mechlorethamine 0.016% gel formulation as maintenance regimen for MF patients in remission. PURPOSE: To determine progression-free survival and efficacy of chlormethine/mechlorethamine as maintenance and active treatment regimens for MF. MATERIALS AND METHODS: A retrospective review of MF patients seen at Thomas Jefferson University from 2012 to 2020 was conducted. Patients of all stages treated with chlormethine/mechlorethamine as maintenance or active treatment with 2 consecutive mSWATs (modified Severity Weighted Assessment Tool) documented were included. Treatment outcomes were assessed by change in mSWAT and progression-free survival. Dermatology Life Quality Index surveys before and after treatment were analyzed. RESULTS: Of 186 MF patients, 44 met inclusion criteria. Patients on maintenance therapy had a 65.22% progression-free survival rate with median time to progression of 29.45 months. By-time analysis for responders on active and maintenance treatment showed an increased response over time. Peak responses were seen at last mSWAT recorded. Both cohorts experienced improved quality-of-life scores from initiation to discontinuation of chlormethine/mechlorethamine. CONCLUSION: Patients on maintenance and active chlormethine/mechlorethamine treatment regimens demonstrated improvement in mSWAT and quality-of-life. Chlormethine/mechlorethamine treatment showed progression-free survival for a median of 29.45 months, indicating this therapy may be an effective maintenance regimen.

Assuntos

Linfoma Cutâneo de Células T , Micose Fungoide , Neoplasias Cutâneas , Humanos , Mecloretamina/efeitos adversos , Mecloretamina/uso terapêutico , Micose Fungoide/tratamento farmacológico , Micose Fungoide/patologia , Estudos Retrospectivos , Neoplasias Cutâneas/tratamento farmacológico , Neoplasias Cutâneas/patologia

RESUMO

Abstract Lobomycosis is a chronic granulomatous infection caused by the yeast Lacazia loboi, typically found in tropical and subtropical geographical areas. Transmission occurs through traumatic inoculation into the skin, especially in exposed areas, of men who work in contact with the soil. Lesions are restricted to the skin and subcutaneous tissue, with a keloid-like appearance in most cases. The occurrence of squamous cell carcinoma on skin lesions with a long evolution is well known; however, there are scarce reports of lobomycosis that developed into squamous cell carcinoma. The authors report a patient from the Brazilian Amazon region, with lobomycosis and carcinomatous degeneration, with an unfavorable outcome, due to late diagnosis.

Assuntos

Humanos , Masculino , Lacazia , Lobomicose/patologia , Queloide/patologia , Pele/patologia , Brasil

RESUMO

Lobomycosis is a chronic granulomatous infection caused by the yeast Lacazia loboi, typically found in tropical and subtropical geographical areas. Transmission occurs through traumatic inoculation into the skin, especially in exposed areas, of men who work in contact with the soil. Lesions are restricted to the skin and subcutaneous tissue, with a keloid-like appearance in most cases. The occurrence of squamous cell carcinoma on skin lesions with a long evolution is well known; however, there are scarce reports of lobomycosis that developed into squamous cell carcinoma. The authors report a patient from the Brazilian Amazon region, with lobomycosis and carcinomatous degeneration, with an unfavorable outcome, due to late diagnosis.

Assuntos

Queloide , Lacazia , Lobomicose , Brasil , Humanos , Queloide/patologia , Lobomicose/patologia , Masculino , Pele/patologia

RESUMO

Abstract Cutaneous T-cell lymphomas are a heterogeneous group of lymphoproliferative disorders, characterized by infiltration of the skin by mature malignant T cells. Mycosis fungoides is the most common form of cutaneous T-cell lymphoma, accounting for more than 60% of cases. Mycosis fungoides in the early-stage is generally an indolent disease, progressing slowly from some patches or plaques to more widespread skin involvement. However, 20% to 25% of patients progress to advanced stages, with the development of skin tumors, extracutaneous spread and poor prognosis. Treatment modalities can be divided into two groups: skin-directed therapies and systemic therapies. Therapies targeting the skin include topical agents, phototherapy and radiotherapy. Systemic therapies include biological response modifiers, immunotherapies and chemotherapeutic agents. For early-stage mycosis fungoides, skin-directed therapies are preferred, to control the disease, improve symptoms and quality of life. When refractory or in advanced-stage disease, systemic treatment is necessary. In this article, the authors present a compilation of current treatment options for mycosis fungoides and Sézary syndrome.

Assuntos

Humanos , Neoplasias Cutâneas/terapia , Linfoma Cutâneo de Células T , Micose Fungoide/terapia , Síndrome de Sézary/terapia , Qualidade de Vida

RESUMO

The delta-amino acid 5-aminolevulinic acid (ALA), is the precursor of the endogenous photosensitiser Protoporphyrin IX (PpIX), and is currently approved for Photodynamic Therapy (PDT) of certain superficial cancers. However, ALA-PDT is not very effective in diseases in which T-cells play a significant role. Cutaneous T-cell lymphomas (CTCL) is a group of non-Hodgkin malignant diseases, which includes mycosis fungoides (MF) and Sézary syndrome (SS). In previous work, we have designed new ALA esters synthesised by three-component Passerini reactions, and some of them showed higher performance as compared to ALA. This work aimed to determine the efficacy as pro-photosensitisers of five new ALA esters of 2-hydroxy-N-arylacetamides (1f, 1 g, 1 h, 1i and 1 k) of higher lipophilicity than ALA in Myla cells of MF and HuT-78 cells of SS. We have also tested its effectiveness against ALA and the already marketed ALA methyl ester (Me-ALA) and ALA hexyl ester (He-ALA). Both cell Myla and SS cells were effectively and equally photoinactivated by ALA-PDT. Besides, the concentration of ALA required to induce half the maximal porphyrin synthesis was 209 µM for Myla and 169 µM for HuT-78 cells. As a criterion of efficacy, we calculated the concentration of the ALA derivatives necessary to induce half the plateau porphyrin values obtained from ALA. These values were achieved at concentrations 4 and 12 times lower compared to ALA, according to the derivative used. For He-ALA, concentrations were 24 to 25 times lower than required for ALA for inducing comparable porphyrin synthesis in both CTCL cells. The light doses for inducing 50% of cell death (LD50) for He-ALA, 1f, 1 g, 1 h and 1i were around 18 and 25 J/cm2 for Myla and HuT-78 cells respectively, after exposure to 0.05 mM concentrations of the compounds. On the other hand, the LD50s for the compound 1 k were 40 and 57 J/cm2 for Myla and HuT-78, respectively. In contrast, 0.05 mM of ALA and Me-ALA did not provoke photokilling since the concentration employed was far below the porphyrin saturation point for these compounds. Our results suggest the potential use of ALA derivatives for topical application in PDT treatment of MF and extracorporeal PDT for the depletion of activated T-cells in SS.

Assuntos

Ácido Aminolevulínico/análogos & derivados , Fármacos Fotossensibilizantes/farmacologia , Ácido Aminolevulínico/farmacologia , Ácido Aminolevulínico/uso terapêutico , Linhagem Celular Tumoral , Sobrevivência Celular/efeitos dos fármacos , Sobrevivência Celular/efeitos da radiação , Humanos , Luz , Linfócitos/efeitos dos fármacos , Linfócitos/metabolismo , Linfócitos/fisiologia , Fotoquimioterapia , Fármacos Fotossensibilizantes/química , Fármacos Fotossensibilizantes/uso terapêutico

RESUMO

INTRODUCTION: Mycosis fungoides (MF) is the most common primary skin T-cell lymphoma, which is characterized for a heterogeneous clinical expressivity. OBJECTIVE: To report clinical variants and sociodemographic characteristics in patients with MF under the care of a dermatological hospital. METHODS: 290 patients with MF clinical and histopathological diagnosis attended to over the course of 11 years were included. Sociodemographic description of patients was made, who were classified according to clinical and histopathological variants. RESULTS: MF was recorded in 57.9 % of women and 42 % of men. The most common clinical variant was the classic type in 46.2 %; dyschromic variants accounted for 35.2 %, out of which hypopigmented MF was the most representative (17.6 %); poikilodermatous MF accounted for 4.1 %, and folliculotropic, for 3.1%. The papular variant occurred in six patients (2.1 %), the single-plaque variety in three (1%), and the ichthyosiform, syringotropic and granulomatous slack skin varieties occurred in one patient each. The granulomatous variant was found in 0.7 %, and 1.4 % had erythroderma. CONCLUSIONS: The most common MF clinical variant was classic plaque stage, followed by dyschromic variants. Other clinical variants accounted for 18.6 %.

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INTRODUCCIÓN: La micosis fungoide es el linfoma primario de células T en piel más frecuente, con expresividad clínica heterogénea. OBJETIVO: Reportar las variedades clínicas y las características sociodemográficas en pacientes con micosis fungoide tratados en un hospital dermatológico. MÉTODOS: Se incluyeron 290 pacientes con diagnóstico clínico e histopatológico de micosis fungoide atendidos en el transcurso de 11 años. Se realizó descripción sociodemográfica de los pacientes, quienes se clasificaron conforme las variantes clínicas e histopatológicas. RESULTADOS: La micosis fungoide se presentó en 57.9 % mujeres y 42 % hombres. La variedad clínica más común fue la clásica en 46.2 %; la discrómica representó 35.2 %, del cual la hipopigmentada fue la más representativa (7.6 %); la poiquilodérmica constituyó 4.1 % y la foliculotrópica, 3.1 %. La variedad papular se presentó en seis pacientes (2.1 %), la de placa única en tres (1 %) y la ictiosiforme, siringotrópica y la piel laxa granulomatosa, en un paciente cada una. La variedad granulomatosa se encontró en 0.7 % y 1.4 % presentó eritrodermia. CONCLUSIONES: La variedad clínica más frecuente de micosis fungoide fue la clásica en fase de placa, seguida de las variedades discrómicas. Otras variedades clínicas representaron 18.6 %.

Assuntos

Micose Fungoide/patologia , Neoplasias Cutâneas/patologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Micose Fungoide/classificação , Micose Fungoide/terapia , Estudos Retrospectivos , Neoplasias Cutâneas/classificação , Neoplasias Cutâneas/terapia , Resultado do Tratamento , Adulto Jovem

RESUMO

Cutaneous T-cell lymphomas are a heterogeneous group of lymphoproliferative disorders, characterized by infiltration of the skin by mature malignant T cells. Mycosis fungoides is the most common form of cutaneous T-cell lymphoma, accounting for more than 60% of cases. Mycosis fungoides in the early-stage is generally an indolent disease, progressing slowly from some patches or plaques to more widespread skin involvement. However, 20% to 25% of patients progress to advanced stages, with the development of skin tumors, extracutaneous spread and poor prognosis. Treatment modalities can be divided into two groups: skin-directed therapies and systemic therapies. Therapies targeting the skin include topical agents, phototherapy and radiotherapy. Systemic therapies include biological response modifiers, immunotherapies and chemotherapeutic agents. For early-stage mycosis fungoides, skin-directed therapies are preferred, to control the disease, improve symptoms and quality of life. When refractory or in advanced-stage disease, systemic treatment is necessary. In this article, the authors present a compilation of current treatment options for mycosis fungoides and Sézary syndrome.

Assuntos

Linfoma Cutâneo de Células T , Micose Fungoide , Síndrome de Sézary , Neoplasias Cutâneas , Humanos , Micose Fungoide/terapia , Qualidade de Vida , Síndrome de Sézary/terapia , Neoplasias Cutâneas/terapia

Assuntos

Mucinose Folicular , Micose Fungoide , Anormalidades da Pele , Neoplasias Cutâneas , Queixo , Humanos

RESUMO

Abstract Background: Mycosis fungoides is the most common type of cutaneous T-cell lymphoma. Most early-stage mycosis fungoides cases follow an indolent course, hence considered by doctors a relatively easy condition. However, since mycosis fungoides bears the title of cancer, patients might perceive it differently. Objective: To investigate patients' illness perception, and its relationships to quality of life, depression, anxiety, and coping among early-stage mycosis fungoides patients. Methods: A cross-sectional questionnaire-based study was conducted. Patients from a single tertiary medical center completed the Revised Illness Perception Questionnaire, the MF/SS-CTCL Quality of Life scale, the Hospital Anxiety and Depression Scale, and The Mental Adjustment to Cancer Scale. Results: Thirty patients (25 males, five females, mean age 51.60) with stage I mycosis fungoides were enrolled. Mycosis fungoides had a little impact on patients' daily life, quality of life, and levels of depression and anxiety, and they generally coped well. Disease understanding was low and was negatively correlated with impairment to quality of life and depression. Patients felt that stress and worry were features of the disease's etiology. Study limitations: A small sample of patients was included. Conclusion: Patients with early-stage mycosis fungoides adapt well to their disease. Psychological interventions should be aimed at improving patients coping style and enhancing illness understanding, in order to maintain high quality of life.

Assuntos

Humanos , Masculino , Feminino , Neoplasias Cutâneas , Micose Fungoide , Percepção , Qualidade de Vida , Adaptação Psicológica , Estudos Transversais , Pessoa de Meia-Idade