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 farmacológicoRESUMO
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 43â67). 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 UltravioletaRESUMO
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-23RESUMO
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 , SeguimentosRESUMO
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.
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/diagnósticoRESUMO
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
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.