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1.
Int J Mol Sci ; 25(4)2024 Feb 12.
Artigo em Inglês | MEDLINE | ID: mdl-38396877

RESUMO

Mogamulizumab (MOG) is an antibody targeting the CCR4 receptor, authorized for relapsed or refractory peripheral T-cell (PTCL) and cutaneous T-cell lymphomas (CTCL). Its adoption in guidelines and endorsement by FDA and EMA established it as a systemic treatment, especially for advanced disease stages due to its comparatively lower toxicity. Clinical trials and real-world evidence have underscored its efficacy in advanced CTCLs, including mycosis fungoides and Sézary syndrome; PTCLs; and adult T-cell leukemia/lymphoma (ATLL), showcasing positive outcomes. Notably, the drug has demonstrated significant response rates, disease stability, and extended periods of progression-free survival, suggesting its applicability in cases with multiple treatment lines. Its safety profile is generally manageable, with adverse events (AEs) primarily related to the skin, infusion-related reactions, drug eruptions, autoimmune diseases, and skin disorders. The latter seem to appear as CCR4 can promote the skin-specific homing of lymphocytes, and MOG is directed against this receptor. While combination with immunostimulatory agents like interferon alpha and interleukin 12 has shown promising results, caution is urged when combining with PD1 inhibitors due to the heightened risk of immune-mediated AEs. The introduction of MOG as a systemic treatment implies a significant advancement in managing these diseases, supported by its favorable safety profile and complementary mechanisms.


Assuntos
Anticorpos Monoclonais Humanizados , Leucemia-Linfoma de Células T do Adulto , Linfoma Cutâneo de Células T , Micose Fungoide , Síndrome de Sézary , Neoplasias Cutâneas , Adulto , Humanos , Linfoma Cutâneo de Células T/tratamento farmacológico , Micose Fungoide/patologia , Síndrome de Sézary/patologia , Leucemia-Linfoma de Células T do Adulto/tratamento farmacológico , Leucemia-Linfoma de Células T do Adulto/patologia , Neoplasias Cutâneas/patologia
2.
Am J Dermatopathol ; 45(2): 81-85, 2023 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-36669069

RESUMO

ABSTRACT: An 84-year-old woman presented with a 3-month history of a papular rash on the trunk, abdomen, and back. Histopathological examination revealed atypical lymphoid deep and band-like dermal infiltrates with marked epidermotropism. Neoplastic cells expressed B-cell markers (CD20), and clonal immunoglobulin gene rearrangement was observed. A complete peripheral blood study revealed aberrant circulating villous lymphocytes with the expression of B-cell markers (CD20, CD22, and CD79a) and aberrant expression of CD5. A staging workup revealed discrete splenic enlargement and bone marrow and gastrointestinal tract involvement. Skin lesions regressed spontaneously several weeks after diagnosis. Throughout evolution, the patient developed scattered cutaneous nodules and generalized papulo-nodules showing either epidermotropic or nonepidermotropic atypical dermal lymphoid infiltrates. This case illustrates the observation of autoinvolutive and recurrent epidermotropic B-cell atypical cutaneous infiltrates as a characteristic feature of secondary cutaneous involvement in splenic marginal B-cell lymphoma. Previously reported cases of epidermotropic B-cell lymphoma have been reviewed. Concurrent and simultaneous observation of epidermotropic and nonepidermotropic lesions seems to indicate that epidermotropism is an important but nonconstant diagnostic feature of splenic marginal B-cell lymphoma.


Assuntos
Leucemia Linfocítica Crônica de Células B , Linfoma de Zona Marginal Tipo Células B , Dermatopatias , Neoplasias Cutâneas , Feminino , Humanos , Idoso de 80 Anos ou mais , Linfoma de Zona Marginal Tipo Células B/patologia , Pele/patologia , Neoplasias Cutâneas/patologia , Leucemia Linfocítica Crônica de Células B/patologia , Dermatopatias/patologia
3.
J Clin Lab Anal ; 37(23-24): e24982, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-38115685

RESUMO

BACKGROUND: Previous investigations pointed out a role for antigen stimulation in Sezary syndrome (SS). High-throughput sequencing of the T cell receptor (TR) offers several applications beyond diagnostic purposes, including the study of T cell pathogenesis. METHODS: We performed high-throughput RNA sequencing of the TR alpha (TRA) and beta (TRB) genes focusing on the complementarity-determining region 3 (CDR3) in 11 SS and one erythrodermic mycosis fungoides (MF) patients. Five psoriasis patients were employed as controls. Peripheral blood CD4+ cells were isolated and RNA sequenced (HiSeq2500). High-resolution HLA typing was performed in neoplastic patients. RESULTS: Highly expanded predominant TRA and TRB CDR3 were only found in SS patients (median frequency: 94.4% and 93.7%). No remarkable CDR3 expansions were observed in psoriasis patients (median frequency of predominant TRA and TRB CDR3: 0.87% and 0.69%, p < 0.001 compared to SS). CDR3 almost identical to the predominant were identified within each SS patient and were exponentially correlated with frequencies of the predominant CDR3 (R2 = 0.918, p < 0.001). Forty-six different CDR3 were shared between SS patients displaying HLA similarities, including predominant TRA and TRB CDR3 in one patient that were found in other three patients. Additionally, 351 antigen matches were detected (Cytomegalovirus, Epstein-Barr, Influenza virus, and self-antigens), and the predominant CDR3 of two different SS patients matched CDR3 with specificity for Influenza and Epstein-Barr viruses. CONCLUSIONS: Besides detecting clonality, these findings shed light on the nature of SS-related antigens, pointing to RNA sequencing as a useful tool for simultaneous clonality and biological analysis in SS.


Assuntos
Psoríase , Síndrome de Sézary , Neoplasias Cutâneas , Humanos , Síndrome de Sézary/genética , Síndrome de Sézary/patologia , Receptores de Antígenos de Linfócitos T alfa-beta/genética , Receptores de Antígenos de Linfócitos T/genética , Regiões Determinantes de Complementaridade/genética , Sequenciamento de Nucleotídeos em Larga Escala , Neoplasias Cutâneas/genética
4.
J Eur Acad Dermatol Venereol ; 37(1): 57-64, 2023 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-36017748

RESUMO

BACKGROUND: Brentuximab vedotin (BV) has been approved for CD30-expressing cutaneous T-cell lymphoma (CTCL) after at least one previous systemic treatment. However, real clinical practice is still limited. OBJECTIVES: To evaluate the response and tolerance of BV in a cohort of patients with CTCL. METHODS: We analysed CTCL patients treated with BV from the Spanish Primary Cutaneous Lymphoma Registry (RELCP). RESULTS: Sixty-seven patients were included. There were 26 females and the mean age at diagnosis was 59 years. Forty-eight were mycosis fungoides (MF), 7 Sézary syndrome (SS) and 12 CD30+ lymphoproliferative disorders (CD30 LPD). Mean follow-up was 18 months. Thirty patients (45%) showed at least 10% of CD30+ cells among the total lymphocytic infiltrate. The median number of BV infusions received was 7. The overall response rate (ORR) was 67% (63% in MF, 71% in SS and 84% in CD30 LPD). Ten of 14 patients with folliculotropic MF (FMF) achieved complete or partial response (ORR 71%). The median time to response was 2.8 months. During follow-up, 36 cases (54%) experienced cutaneous relapse or progression. The median progression free survival (PFS) was 10.3 months. The most frequent adverse event was peripheral neuropathy (PN) (57%), in most patients (85%), grades 1 or 2. CONCLUSIONS: These results confirm the efficacy and safety of BV in patients with advanced-stage MF, and CD30 LPD. In addition, patients with FMF and SS also showed a favourable response. Our data suggest that BV retreatment is effective in a proportion of cases.


Assuntos
Imunoconjugados , Linfoma Cutâneo de Células T , Transtornos Linfoproliferativos , Micose Fungoide , Síndrome de Sézary , Neoplasias Cutâneas , Feminino , Humanos , Pessoa de Meia-Idade , Brentuximab Vedotin/uso terapêutico , Imunoconjugados/efeitos adversos , Neoplasias Cutâneas/patologia , Micose Fungoide/patologia , Síndrome de Sézary/patologia , Sistema de Registros , Antígeno Ki-1
5.
Vasc Med ; 27(1): 63-72, 2022 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-34392750

RESUMO

INTRODUCTION: Venous leg ulcers (VLU) embody the most severe stage of the broad spectrum of chronic venous disease. Approximately 40% of patients with VLU present with the underlying deep venous disease (DVD). Although the data are scarce, these deep venous disease-related VLU (DRV) are thought to have higher recurrence rates and a substantial economic burden. The objective of this study was to assess the economic burden of DRV across Australia, France, Germany, Italy, Spain, the UK, and the USA. METHODS: A comprehensive literature review was undertaken to identify publications documenting the incidence and prevalence of VLU and DRV, medical resource utilization, and associated costs of DRV. Findings from this literature review were used to estimate the economic burden of illness, including direct medical costs over a 12-month interval following initial presentation of a newly formed DRV. RESULTS: Total annual incidence of new or recurrent DRV in Australia, France, Germany, Italy, Spain, UK, and the US are estimated at 122,000, 263,000, 345,000, 253,000, 85,000, 230,000, and 643,000 events, respectively, in 2019. Incidence ranges from 0.73 to 3.12 per 1000 persons per year. The estimated annual direct medical costs for patients managed conservatively in these geographies total ~ $10.73 billion (USD) or $5527 per person per year. CONCLUSION: The availability of published data on the costs of VLU care varies widely across countries considered in this analysis. Although country-specific VLU practice patterns vary, there is a uniform pattern of high-cost care.


Assuntos
Estresse Financeiro , Úlcera Varicosa , França , Humanos , Incidência , Prevalência , Úlcera Varicosa/diagnóstico , Úlcera Varicosa/epidemiologia , Úlcera Varicosa/terapia
6.
Dermatol Ther ; 35(11): e15865, 2022 11.
Artigo em Inglês | MEDLINE | ID: mdl-36175141

RESUMO

Guselkumab is a monoclonal antibody that selectively blocks the p19 subunit of interleukin 23 and has been approved for the treatment of moderate to severe psoriasis and active psoriatic arthritis in adult patients due to its efficacy in different clinical trials. Therefore, itis important to know the performance of guselkumab in this setting of patients in clinical practice given that a high percentage of them are not represented in these clinical trials. Our objective was to evaluate the effectiveness and tolerability of guselkumab in clinical practice in the first patients with psoriasis and psoriatic arthritis treated since the date of its approval for psoriasis in Spain, in joint dermatology-rheumatology clinics. A multicenter retrospective data collection was carried out, in which 14 hospitals participated, including a total of 90 patients with psoriatic arthritis confirmed by a rheumatologist. Data collection was recorded at baseline and at weeks 12, 24, and 52 for both the articular and cutaneous domains. Ninety PsA patients started treatment with guselkumab and therefore were included in this study. The vast majority had already failed to at least to one biologic therapyprior guselkumab prescription. The median age was 55 years, 61% were female and 46% had a BMI ≥ 30 kg/m2 . Sixty-nine percent suffered from peripheral arthritis, and in 34% an axial involvement was also detected; dactylitis or enthesitis was present in 24% and 29% of patients, respectively. Guselkumab was effective in controlling both articular and skin manifestations of PsA patients. Absolute PASI significantly decreased from 10.5 to 4.8, 1.9 and 1.3 at weeks 12, 24, and 52, respectively. In 29 out of 61 (48%) of cases, DAPSA was moderate or high, and patients showed a significant reduction in DAPSA at 12, 24, and 52 weeks of treatment (mean DAPSA values at baseline and follow up were 29, 20, 16, and 14, respectively). Patients with DAPSA in low activity or in remission at the time of initiation of guselkumab maintained response at the end of the study period. No new safety concerns were detected. Seventy-eight out of 90 patients (84.4%) persisted on treatment after 2 years follow-up. Our experience suggests that guselkumab isan effective drug for PsA and PsO patients in clinical practice with good tolerability and no additional safety signals, making it a new therapeutic alternative for the treatment of PsA and PsO patients.


Assuntos
Artrite Psoriásica , Psoríase , Adulto , Humanos , Feminino , Pessoa de Meia-Idade , Masculino , Artrite Psoriásica/tratamento farmacológico , Estudos Retrospectivos , Resultado do Tratamento , Psoríase/diagnóstico , Psoríase/tratamento farmacológico , Índice de Gravidade de Doença
7.
Am J Dermatopathol ; 44(4): e41-e45, 2022 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-34966050

RESUMO

ABSTRACT: A 59-year-old woman presented with a persistent eruption manifested as multiple agminated miliary facial papules. Histopathological examination showed prominent nodular dermal lymphoid infiltrates with hyperplastic follicles that were initially interpreted as B-cell reactive lymphoid hyperplasia. Several years later, an additional biopsy showed a dense perifollicular infiltrate with reactive primary and secondary follicles. Accompanying T cells corresponded to CD3/CD4/PD1/CXCL13-positive cells and scattered Epstein-Barr virus-positive B cells were identified by in situ hybridization. A monoclonal T-cell population was demonstrated by TCRγ and TCRß Polymerase Chain Reaction amplification, as well as a minor abnormal circulating T-cell population by flow cytometry (0.62% of the white blood cells, CD4+CD3s-CD7-). A biopsy specimen from an enlarged right supraclavicular lymph node disclosed nodal involvement by angioimmunoblastic T-cell lymphoma. The observation of B-cell dermal nodular infiltrates with well-demarcated lymphoid aggregates forming primary lymphoid follicles may lead to overlook the T-cell component in some cases of angioimmunoblastic T-cell lymphoma. In such cases, a careful assessment of the apparently minor T-cell component is important to establish a correct diagnosis.


Assuntos
Linfócitos B/patologia , Infecções por Vírus Epstein-Barr/diagnóstico , Linfadenopatia Imunoblástica/diagnóstico , Linfoma de Células T/diagnóstico , Diagnóstico Diferencial , Infecções por Vírus Epstein-Barr/complicações , Infecções por Vírus Epstein-Barr/patologia , Feminino , Humanos , Linfadenopatia Imunoblástica/complicações , Linfadenopatia Imunoblástica/patologia , Linfoma de Células T/complicações , Linfoma de Células T/patologia , Pessoa de Meia-Idade , Pseudolinfoma/diagnóstico
8.
Acta Derm Venereol ; 101(5): adv00460, 2021 May 19.
Artigo em Inglês | MEDLINE | ID: mdl-33954802

RESUMO

BIOMED-2 Concerted Action BMH4-CT98-3936 (BIOMED-2) PCR protocols are an important diagnostic tool in the evaluation of cutaneous lymphomas. The aim of this study was to assess the diagnostic value of the genotyping results obtained by these techniques in daily clinical practice. A total of 360 paraffin-embedded skin samples were retrospectively reviewed from 114 cutaneous T-cell lymphomas and 35 cutaneous B-cell lym-phomas. A total of 249 biopsies from 180 patients with benign lymphoid infiltrates served as controls. T-cell receptor and immunoglobulin gene rearrangements were assessed using the BIOMED-2 method. A combined T-cell receptor gamma and beta assay approach reliably distinguished cutaneous T-cell lymphomas from benign skin T-cell infiltrates (sensitivity 89.4%; specificity 81.5%). Analysis of complete immunoglobulin heavy chain rearrangements also differentiated cutaneous B-cell lymphomas from benign B-cell infiltrates (sensitivity 85.7%; specificity 82.4%). In conclusion, the full BIOMED-2 protocol is a useful aid combined with clinical, histological and immunophenotypical findings for assessment of lymphoid clonality in skin lymphoid proliferations.


Assuntos
Linfoma de Células B , Linfoma Cutâneo de Células T , Neoplasias Cutâneas , Humanos , Linfoma de Células B/diagnóstico , Linfoma de Células B/genética , Linfoma Cutâneo de Células T/diagnóstico , Linfoma Cutâneo de Células T/genética , Reação em Cadeia da Polimerase , Estudos Retrospectivos , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/genética
9.
Am J Dermatopathol ; 43(4): 300-304, 2021 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-33264131

RESUMO

ABSTRACT: A 45-year-old woman presented with a solitary breast nodule that histologically corresponded to a dense dermal/subcutaneous infiltration of atypical cytotoxic T-lymphocytes (CD3+, CD8+, CD56+, TIA-1+, CD5-, CD4-, CD30-, EBV-), resembling subcutaneous panniculitic T-cell lymphoma. The presence of TCRδ gene rearrangement and the absence of ßF1 expression let to suspect the diagnosis of primary cutaneous γδT-cell lymphoma. As a consequence of jejunum perforation following chemotherapy treatment, a mucosal atypical lymphoid infiltration with marked epitheliotropism was observed in the resected intestinal sample, and the diagnosis of monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) was finally established. Disease progression appeared with multiple erythematous plaques showing a dense lichenoid atypical cytotoxic T-cell infiltrate with intense epidermotropism, mimicking primary cutaneous epidermotropic aggressive CD8+ T-cell lymphoma. MEITL is an uncommon and aggressive peripheral T-cell lymphoma that often presents in adults with gastrointestinal symptoms. Secondary cutaneous involvement is a rare phenomenon that may show clinicopathologic and immunohistochemical features that overlap with different subtypes of primary cutaneous cytotoxic T-cell lymphomas. In the absence of gastrointestinal symptoms, the diagnosis may be challenging, and only the evidence of underlying MEITL may allow to establish the definite diagnosis.


Assuntos
Linfoma de Células T Associado a Enteropatia/patologia , Linfoma Cutâneo de Células T/patologia , Neoplasias Cutâneas/patologia , Progressão da Doença , Linfoma de Células T Associado a Enteropatia/imunologia , Linfoma de Células T Associado a Enteropatia/terapia , Evolução Fatal , Feminino , Humanos , Linfócitos do Interstício Tumoral/imunologia , Linfoma Cutâneo de Células T/imunologia , Linfoma Cutâneo de Células T/terapia , Pessoa de Meia-Idade , Neoplasias Cutâneas/imunologia , Neoplasias Cutâneas/terapia , Linfócitos T/imunologia
10.
Am J Dermatopathol ; 42(2): e22-e25, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31313693

RESUMO

Herein, we report a case of an adult male patient with a chronic and recurrent papulopustular eruption mainly involving the trunk and lower extremities. A dense superficial perifollicular inflammatory infiltrate with palisading necrobiotic granuloma formation and infundibular perforation was observed at the histological examination, with no granulomatous inflammatory infiltrates in deeper areas. The possibility that this peculiar clinicopathological presentation constitutes a case of generalized perforating granuloma annulare (PGA) or an individualized skin condition is discussed. The observation of a pustular follicular generalized PGA represents an exceedingly rare phenomenon and constitutes an infrequent subtype of PGA that can mimic pustular eruptions secondary to many different etiologies. The clinicopathological features of this rare variant may represent a diagnostic challenge, often requiring multiple biopsies to establish a definite diagnosis.


Assuntos
Exantema/patologia , Granuloma Anular/patologia , Acitretina/uso terapêutico , Idoso , Exantema/tratamento farmacológico , Granuloma Anular/tratamento farmacológico , Humanos , Ceratolíticos/uso terapêutico , Masculino
11.
Am J Dermatopathol ; 42(10): 717-730, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32956080

RESUMO

Neutrophilic infiltrates in panniculitis can be seen in different clinical-pathological entities. There are a "mostly neutrophilic inflammatory infiltrate" in some entities classically defined as neutrophilic panniculitis and already included in algorithms, such as enzymatic panniculitis, infective and factitial ones, erythema induratum, or subcutaneous Sweet syndrome, but there are also other panniculitis where neutrophils are frequently observed such as panniculitis associated with inflammatory bowel disease or rheumatoid arthritis, or drug-induced panniculitis associated with BRAF inhibitors, and finally, some panniculitis are better classified in other panniculitides groups but may present with neutrophil-rich variants, such as the neutrophil-rich subcutaneous fat necrosis of the newborn. We review the main clinical and histopathological features of most of these panniculitides and construct a diagnostic algorithm including these diseases.


Assuntos
Algoritmos , Eritema Nodoso/patologia , Neutrófilos/patologia , Paniculite/etiologia , Paniculite/patologia , Dermatopatias Infecciosas/complicações , Doenças Autoimunes/complicações , Síndrome de Behçet/patologia , Corpos Estranhos/complicações , Humanos , Pancreatopatias/complicações , Paniculite/diagnóstico , Inibidores de Proteínas Quinases/efeitos adversos , Síndrome de Sweet/complicações , Deficiência de alfa 1-Antitripsina/complicações
12.
J Cutan Pathol ; 46(3): 182-189, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30511443

RESUMO

BACKGROUND: Primary cutaneous follicular center-cell lymphoma (PCFCL) is one of the most common types of cutaneous B-cell lymphoma. Differences in immunohistochemical expression of BCL2 and CD10 antigens along with the presence of t(14:18) translocation in neoplastic cells have been postulated as relevant clues in differentiating PCFCL from cutaneous lesions secondary to a systemic follicular lymphoma (SCFL). The aim of this study is to evaluate the significance and usefulness of these parameters in a large series of patients. METHODS: Patients with PCFCL and SCFL diagnosed at three university hospitals in Barcelona, from 2000 to 2015 were reviewed. Clinical, histopathological, immunophenotypical, genetic, and outcome parameters were analyzed. RESULTS: Eighty-one cases (59 PCFCL and 22 SCFL) were included. There were no significant differences between PCFCL and SCFL cases regarding clinical presentation, site of involvement, or predominant type of skin lesions. Most patients in both groups showed positivity for BCL2 and CD10, but strong expression of BCL2 and CD10 was associated with SCFL cases. Although more frequent in SCFL, a small proportion of PCFCL cases also showed the t(14:18) on FISH analysis. CONCLUSION: The intensity of BCL2 expression was found to be the single most valuable clue in differentiating PCFCL from SCFL cases on histopathological grounds.


Assuntos
Biomarcadores Tumorais/análise , Linfoma Folicular/diagnóstico , Neoplasias Cutâneas/diagnóstico , Adulto , Idoso , Idoso de 80 Anos ou mais , Cromossomos Humanos Par 14/genética , Cromossomos Humanos Par 18/genética , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neprilisina/análise , Neprilisina/biossíntese , Proteínas Proto-Oncogênicas c-bcl-2/análise , Proteínas Proto-Oncogênicas c-bcl-2/biossíntese , Translocação Genética/genética , Adulto Jovem
13.
Acta Derm Venereol ; 99(2): 164-169, 2019 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-30281142

RESUMO

This cross-sectional study evaluated the usefulness of an ultrasound technique in assessment of nail changes in 35 patients with psoriatic onychopathy and 25 with nail dystrophy secondary to onychomycosis. All patients underwent 3 examinations: a complete clinical assessment; a nail ultrasound study; and fungal culture. Nails of patients with psoriatic onychopathy presented a thinner nail plate and nail bed, measured by ultrasound, than did those with onychomycosis. The percentage of patients with a power Doppler signal ?2 at nail bed was significantly higher in psoriatic onychopathy than in onychomycosis, and structural bone lesions were more frequent in psoriatic onychopathy than in onychomycosis. These results suggest that the presence of structural damage and high-power Doppler signal are the main ultrasound findings supporting a diagnosis of psoriatic onychopathy.


Assuntos
Doenças da Unha/diagnóstico por imagem , Unhas/diagnóstico por imagem , Onicomicose/diagnóstico por imagem , Psoríase/diagnóstico por imagem , Ultrassonografia Doppler , Adulto , Estudos Transversais , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes
14.
Acta Derm Venereol ; 99(4): 417-422, 2019 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-30628634

RESUMO

Psoriasis is a chronic skin disease associated with considerable physical and psychological comorbidities. Stress and emotional disturbances have been implicated in both triggering the onset and exacerbation of psoriasis. In order to determine the level of perceived stress and mood alterations in patients with psoriasis and their association with disease severity, 300 individuals completed diverse validated questionnaires assessing stress and psychological mood. Evaluation of perception of disease was also measured. A significant association between psoriasis severity and mood, emotional disturbances and an impact on assessments of the quality of life were observed. Particularly, Montgomery-Asberg Depression Rating Scale, Hamilton Rating Scale and Hospital Anxiety and Depression Scale for Depression detected a significant risk for depression in relation to the disease severity. The association between depression features, anxiety and perceived stress with psoriasis severity is important and can influence the appropriate management of psoriasis.


Assuntos
Ansiedade/psicologia , Depressão/psicologia , Emoções , Psoríase/psicologia , Estresse Psicológico/psicologia , Adolescente , Adulto , Afeto , Idoso , Idoso de 80 Anos ou mais , Ansiedade/diagnóstico , Ansiedade/epidemiologia , Comorbidade , Efeitos Psicossociais da Doença , Depressão/diagnóstico , Depressão/epidemiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Psoríase/diagnóstico , Psoríase/epidemiologia , Qualidade de Vida , Fatores de Risco , Índice de Gravidade de Doença , Espanha/epidemiologia , Estresse Psicológico/diagnóstico , Estresse Psicológico/epidemiologia , Inquéritos e Questionários , Adulto Jovem
15.
Am J Dermatopathol ; 41(1): 60-64, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-29877895

RESUMO

Self-healing cutaneous mucinosis (SHCM) is an idiopathic localized cutaneous mucinosis mainly described in children and characterized clinically by an acute onset of papules and nodules that exhibit a spontaneous resolution in a period ranging from weeks to few months. Histologically, a diffuse mucin deposition in the dermis and/or hypodermis associated with a proliferation of spindle-shaped cells and some large epithelioid gangliocyte-like mononuclear cells is usually observed. An uncommon adult variant of SHCM has also been reported; however, the clinicopathological features described in these patients are extremely heterogeneous and differ significantly from the juvenile variant of the disease, often showing exclusively dermal involvement. We report a case of a 37-year-old female patient with multiple asymptomatic nodules located on the legs and arms that resolved spontaneously in a period of 2 years, showing the typical subcutaneous features of the juvenile variant of SHCM at the histological examination (ie, mucinous areas associated with dense bands of fibrosis containing arborizing thin-walled vessels, spindle-shaped fibroblasts, and some gangliocyte-like cells). To the best of our knowledge, this is the first report of SHCM showing the classic pattern of deep-seated subcutaneous involvement of the disease in an adult patient. We also review the cases of adult-onset SHCM reported in the literature.


Assuntos
Mucinoses/patologia , Pele/patologia , Cicatrização , Adulto , Fatores Etários , Biópsia , Feminino , Humanos , Fenótipo , Remissão Espontânea
17.
J Am Acad Dermatol ; 79(5): 892-898, 2018 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-30126736

RESUMO

BACKGROUND: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare primary cutaneous lymphoma of mature cytotoxic T cells. Initially, patients with SPTCL were treated with doxorubicin-based polychemotherapy. OBJECTIVE: To analyze clinical, biologic, immunophenotypical, molecular, imaging, treatment, and outcome data reflecting the current state of knowledge. METHODS: A retrospective multicenter study of 16 patients with SPTCL that was diagnosed between 1996 and 2016. RESULTS: The female-to-male ratio was 1.7. The median age at diagnosis was 46.5 years. Patients presented with multiple nodular or plaque-like lesions preferentially affecting the legs and/or trunk. Histopathology typically showed a lobular panniculitis with individual adipocytes surrounded by atypical lymphocytes, usually with a CD3+, CD4-, CD8+, CD56-, TIA1 cytotoxic granule associated RNA binding protein 1-positive phenotype and high proliferation rate. SPTCL was associated with autoimmune diseases in 25% of patients, and with the development of hemophagocytic syndrome in 18% of patients. Oral steroids alone or in combination with low-dose methotrexate or cyclosporine A were the most common initial treatment, achieving a complete response in 85% of the treated patients. The median follow-up time was 14 months. The 5-year disease-specific survival rate was 85.7%. LIMITATIONS: This was a retrospective study. CONCLUSIONS: SPTCL has an excellent prognosis. Immunosuppressive agents can be considered for first-line treatment.


Assuntos
Linfoma Cutâneo de Células T/patologia , Linfoma Cutâneo de Células T/terapia , Linfoma de Células T/patologia , Linfoma de Células T/terapia , Paniculite/patologia , Paniculite/terapia , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/terapia , Adulto , Idoso , Quimiorradioterapia/métodos , Estudos de Coortes , Intervalo Livre de Doença , Feminino , Humanos , Linfoma de Células T/diagnóstico por imagem , Linfoma de Células T/mortalidade , Linfoma Cutâneo de Células T/diagnóstico por imagem , Linfoma Cutâneo de Células T/mortalidade , Masculino , Pessoa de Meia-Idade , Paniculite/diagnóstico por imagem , Paniculite/mortalidade , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada/métodos , Estudos Retrospectivos , Medição de Risco , Estudos de Amostragem , Neoplasias Cutâneas/diagnóstico por imagem , Neoplasias Cutâneas/mortalidade , Espanha , Análise de Sobrevida , Adulto Jovem
18.
Rheumatol Int ; 38(11): 2037-2043, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-30143818

RESUMO

Although several randomized clinical trials and observational studies have evaluated the effectiveness, safety and drug survival of etanercept (ETN) in the treatment of psoriatic arthritis (PsA), long-term data regarding these aspects are currently scarce. For this reason, we sought to investigate the long-term survival and safety of ETN in PsA patients in 4 tertiary care Spanish hospitals over a 13-year observation period (from 2004 to 2017). The records of 85 PsA patients were reviewed. ETN showed an excellent survival profile, with rates of treatment discontinuation at 1, 3, 5 and 10 years of 15, 37, 46 and 59%, respectively. In our cohort, a trend toward longer drug survival in patients with shorter disease duration and those who were treated with ETN as their first biologic agent was observed. On the other hand, combination therapy with conventional disease-modifying antirheumatic drugs did not provide greater improvement on the long-term drug survival. Only 12% of the patients reported adverse events (AEs) during therapy, being most of them of mild to moderate intensity, and in only 7% AEs led to drug discontinuation. To the best of our knowledge, the present study shows the largest follow-up period of ETN-treated population analyzed in a real-life setting, and these results demonstrate the positive safety profile and long-term effectiveness of this biologic agent in the management of PsA patients.


Assuntos
Antirreumáticos/administração & dosagem , Artrite Psoriásica/tratamento farmacológico , Etanercepte/administração & dosagem , Padrões de Prática Médica , Adulto , Idoso , Idoso de 80 Anos ou mais , Antirreumáticos/efeitos adversos , Artrite Psoriásica/diagnóstico , Artrite Psoriásica/imunologia , Esquema de Medicação , Etanercepte/efeitos adversos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Espanha , Centros de Atenção Terciária , Fatores de Tempo , Resultado do Tratamento
19.
Am J Dermatopathol ; 40(9): 694-698, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-29649008

RESUMO

The presence of acquired generalized keratotic follicular papules and comedones developing in adulthood constitute an uncommon clinical situation. Although this clinical presentation has been described in several noninflammatory, inflammatory, and neoplastic skin disorders, its association with an exclusive perifollicular epitheliod or granulomatous inflammatory reaction represents an exceedingly rare phenomenon. We report a case of a 57-year-old male patient presenting clinically numerous acquired disseminated follicular papules and comedones, showing isolated perifollicular sarcoid-like granulomatous inflammatory infiltrates at the histological examination. These lesions experienced a complete spontaneous resolution after several months. The possibility that this disseminated, epithelioid, granulomatous, perifollicular dermatosis may represent an isolated follicular variant of lichen nitidus or a previously nonreported skin disorder is discussed.


Assuntos
Granuloma/patologia , Líquen Nítido/patologia , Dermatopatias/patologia , Pele/patologia , Biópsia , Diagnóstico Diferencial , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Remissão Espontânea , Fatores de Tempo
20.
Pediatr Dermatol ; 35(5): e291-e293, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-29974513

RESUMO

Inflammatory linear verrucous epidermal nevus and linear psoriasis are different entities with clinical and histopathologic similarities. Isolated reports of inflammatory linear verrucous epidermal nevus with concomitant psoriasis or a positive family history of psoriasis have been described, and the possibility that inflammatory linear verrucous epidermal nevus may be a mosaic form of cutaneous psoriasis has been postulated. We report a 17-year-old boy with a congenital, linear, erythematous, keratotic plaque on the dorsum of the fifth finger of the left hand with ipsilateral nail dystrophy. Histopathologic examination showed epidermal hyperplasia with alternating orthokeratosis and parakeratosis. During follow-up, he developed erosive monoarthritis of the distal interphalangeal joint. This case seems to confirm the association between inflammatory linear verrucous epidermal nevus and arthritis and supports a possible relationship between inflammatory linear verrucous epidermal nevus and psoriasis.


Assuntos
Artrite/complicações , Nevo Sebáceo de Jadassohn/diagnóstico , Adolescente , Artrite/tratamento farmacológico , Diagnóstico Diferencial , Glucocorticoides/uso terapêutico , Humanos , Masculino , Nevo Sebáceo de Jadassohn/complicações , Nevo Sebáceo de Jadassohn/tratamento farmacológico , Pele/patologia
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