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Cureus May 2024An 84-year-old female experienced progressive erythema on her limbs and chest over the past year. Initially managed with topical steroids, the erythema eventually spread...
An 84-year-old female experienced progressive erythema on her limbs and chest over the past year. Initially managed with topical steroids, the erythema eventually spread throughout her body, forming erosions. A biopsy confirmed the diagnosis of mycosis fungoides (MF) (Stage IIB, T2bN0M0B0). Treatment with oral bexarotene (300 mg/day) and narrow-band UVB therapy showed limited improvement. Electron beam therapy (30 Gy in 10 fractions) applied to facial and plantar tumors resulted in a reduction of the tumors. This case highlights the treatment of tumors of MF on the face showing the effectiveness of combining electron beam therapy with bexarotene.
PubMed: 38933612
DOI: 10.7759/cureus.61164 -
Journal Der Deutschen Dermatologischen... Jun 2024Mogamulizumab, a monoclonal antibody directed against CC chemokine receptor 4, is approved as a second-line treatment of mycosis fungoides and Sézary syndrome. One of... (Review)
Review
Mogamulizumab, a monoclonal antibody directed against CC chemokine receptor 4, is approved as a second-line treatment of mycosis fungoides and Sézary syndrome. One of the most common side effects is mogamulizumab-associated rash (MAR), which can present in a variety of clinical and histological types. Clinically, it can be difficult to differentiate between MAR and progression of the underlying disease, so histological examination is crucial for clinicopathological correlation. Current data analyses suggest that MAR is more common in patients with Sézary syndrome and is associated with a significantly better response to treatment, making the distinction from disease progression particularly important. The management of MAR depends on its severity, and therapy may need to be paused. This article presents three cases from our clinic and reviews the current literature on MAR. It emphasizes the importance of understanding MAR in the management of patients with cutaneous lymphomas.
PubMed: 38924340
DOI: 10.1111/ddg.15432 -
Dermatopathology (Basel, Switzerland) Jun 2024Primary cutaneous lymphomas (PCLs), especially mycosis fungoides (MF), pose significant diagnostic and therapeutic challenges. This study aims to correlate initial...
Primary cutaneous lymphomas (PCLs), especially mycosis fungoides (MF), pose significant diagnostic and therapeutic challenges. This study aims to correlate initial histological features with the disease course and survival in MF patients. A retrospective-prospective cohort study was conducted on 83 patients diagnosed with early-stage MF at the Departments of Dermatovenerology and Pathology, UHC Zagreb, from January 2003 to December 2012. The analyzed histopathological parameters included lichenoid dermal lymphocyte infiltrate, Pautrier microabscesses, and lymphocyte atypia. Patients with more than 30 guardian lymphocytes per 100 keratinocytes exhibited worse overall and progression-free survival. Furthermore, those with over 50% atypical lymphocytes demonstrated a faster progression rate. A dense lichenoid dermal infiltrate and a high count of lymphocyte "keepers" significantly increased the mortality risk within five years of diagnosis. This study did not fully confirm the hypothesis regarding the prognostic value of large Pautrier microabscesses but highlighted the importance of dense lichenoid infiltrates. The study identified new potential histopathological prognostic factors in early-stage MF, suggesting the need for larger studies to confirm these findings. The identification of such predictors could enhance the prognostic stratification and guide more tailored therapeutic approaches for MF patients.
PubMed: 38921054
DOI: 10.3390/dermatopathology11020017 -
Clinics in Dermatology Jun 2024Artificial Intelligence (AI) has evolved to become a significant force in various domains, including medicine. We explore the role of AI in pathology, with a specific...
Artificial Intelligence (AI) has evolved to become a significant force in various domains, including medicine. We explore the role of AI in pathology, with a specific focus on dermatopathology and neoplastic dermatopathology. AI, encompassing Machine Learning (ML) and Deep Learning (DL), has demonstrated its potential in tasks ranging from diagnostic applications on Whole Slide Imaging (WSI) to predictive and prognostic functions in skin pathology. In dermatopathology, studies have assessed AI's ability to identify skin lesions, classify melanomas, and improve diagnostic accuracy. Results indicate that AI, particularly Convolutional Neural Networks (CNNs), can outperform human pathologists in terms of sensitivity and specificity. Moreover, AI aids in predicting disease outcomes, identifying aggressive tumors, and differentiating between various skin conditions. Neoplastic dermatopathology showcases AI's prowess in classifying melanocytic lesions, discriminating between melanomas and nevi, and aiding dermatopathologists in making accurate diagnoses. Studies emphasize the reproducibility and diagnostic aid that AI provides, especially in challenging cases. In inflammatory and lymphoproliferative dermatopathology, limited research exists, but studies show attempts to use AI to differentiate conditions like Mycosis Fungoides and eczema. While some results are promising, further exploration is needed in these areas. We highlight the extraordinary interest AI has garnered in the scientific community and its potential to assist clinicians and pathologists. Despite the advancements, we have stress edthe importance of collaboration between medical professionals, computer scientists, bioinformaticians, and engineers to harness AI's benefits while acknowledging its limitations and risks. The integration of AI into dermatopathology holds great promise, positioning it as a valuable tool rather than as a replacement for human expertise.
PubMed: 38909860
DOI: 10.1016/j.clindermatol.2024.06.010 -
Journal of Evidence-based Medicine Jun 2024Narrowband ultraviolet B (NB-UVB) has been recommended as first-line therapy for early-stage mycosis fungoides (MF) in international guidelines. NB-UVB can be used as...
Effectiveness of narrowband ultraviolet B monotherapy versus combination therapy with systemic agents in patients with early-stage mycosis fungoides and the association with plaque lesions.
OBJECTIVE
Narrowband ultraviolet B (NB-UVB) has been recommended as first-line therapy for early-stage mycosis fungoides (MF) in international guidelines. NB-UVB can be used as monotherapy or part of a multimodality treatment regimen. There is limited evidence on the effectiveness and optimal patients of NB-UVB in combination with systemic therapies in MF. We aimed to assess the effectiveness of the combination versus NB-UVB monotherapy in early-stage MF and if plaque lesion status was related to these effects.
METHODS
This observational cohort study included 247 early-stage MF patients who had received NB-UVB combined with systemic therapies vs. NB-UVB monotherapy from 2009 to 2021. The primary outcome was partial or complete response. Overall response rate and median time to response were calculated. Hazard ratios (HRs) were estimated using the Cox model.
RESULTS
In 139 plaque-stage patients, the response rate for combination therapy group was higher than that of monotherapy group (79.0% vs. 54.3%, p = 0.006). The adjusted HR for combination therapy compared with NB-UVB monotherapy was 3.11 (95% CI 1.72-5.63). The combination therapy group also showed shorter time to response (4 vs. 6 months, p = 0.002). In 108 patch-stage patients, the response rate and time to response in two treatment groups showed no significant difference. There was therefore an observed interaction with patients' plaque lesion status for the effect size of NB-UVB combination therapy. No serious adverse events were observed.
CONCLUSIONS
Adding systemic treatments to NB-UVB did not improve the treatment outcome of patch-stage patients, but it surpassed NB-UVB monotherapy for early-stage patients with plaques.
PubMed: 38898743
DOI: 10.1111/jebm.12623 -
Histopathology Jun 2024There have been exceptional reports of morphoea presenting with epidermal changes overlapping histopathologically with cutaneous T cell lymphoma of the mycosis...
AIMS
There have been exceptional reports of morphoea presenting with epidermal changes overlapping histopathologically with cutaneous T cell lymphoma of the mycosis fungoides type (MF). This phenomenon gives rise to an ambiguous clinicopathological scenario in which distinguishing these conditions may be challenging. The aim of this study is to characterise the clinical, histopathological and molecular findings of this phenomenon through a case series.
METHODS AND RESULTS
Four patients with classical clinical presentation of morphoea but unusual histopathology displaying typical findings of morphoea, together with intra-epidermal CD8 positive lymphocytes indistinguishable from MF, were identified. The clinical phenotypes of morphoea were varied, and they all presented early in the active phase of the disease. They all exhibited intra-epidermal lymphocytes with tagging and cytological atypia. Pautrier-like microabscesses were also seen. Using molecular analysis, two cases showed clonal TCR gene rearrangement. Follow-up of all cases has been consistent with classical morphoea.
CONCLUSION
Early morphoea can seldom present with atypical clonal intra-epidermal lymphocytes indistinguishable from MF. The fact that these changes can occur in several different clinical subtypes of morphoea raises the possibility that this could be a pattern of inflammation in early disease more common than currently appreciated.
PubMed: 38890785
DOI: 10.1111/his.15246 -
The Journal of Dermatology Jun 2024Brentuximab vedotin (BV), a conjugate of anti-CD30 antibody and monomethyl auristatin E, has emerged as a promising treatment option for refractory CD30+ mycosis...
Brentuximab vedotin (BV), a conjugate of anti-CD30 antibody and monomethyl auristatin E, has emerged as a promising treatment option for refractory CD30+ mycosis fungoides (MF) and primary cutaneous anaplastic large-cell lymphoma (pcALCL). BV has been shown to be safe and effective in treating Hodgkin's lymphoma and peripheral T-cell lymphoma. This multicenter, prospective, single-arm phase I/II study evaluated the efficacy of BV in Japanese patients with CD30+ cutaneous lymphomas, namely CD30+ cutaneous T-cell lymphoma. Participants were divided into two groups: those with CD30+ MF or pcALCL (cohort 1, n = 13) and those with CD30+ lymphoproliferative disorders other than those in cohort 1 (cohort 2, n = 3). The studied population included the full analysis set (FAS), modified FAS (mFAS), and safety analysis set (SAF). These sets were identified in cohorts 1 and 1 + 2 and labeled FAS1 and FAS2, mFAS1 and mFAS2, and SAF1 and SAF2, respectively. Each treatment cycle lasted 3 weeks, and BV was continued for up to 16 cycles after the third cycle based on treatment response. The primary endpoint was the 4-month objective response rate (ORR4) determined by the Independent Review Forum (IRF). ORR4 was 69.2% for FAS1 and 62.5% for FAS2 (P < 0.0001). Secondary endpoints of ORR, assessed using the global response score (53.8% in FAS1) and modified severity-weighted assessment tool (62.5% in FAS1), using the IRF, provided results comparable to the primary findings. The incidence of ≥grade 3 adverse events (≥15%) in SAF1 was peripheral neuropathy in three patients (23%) and fever and eosinophilia in two patients (15%). In conclusion, BV showed favorable efficacy, tolerability, and safety profile in Japanese patients with relapsed or refractory CD30+ primary cutaneous T-cell lymphoma. The trial was registered with University Hospital Medical Information Network Clinical Trials Registry, Japan (protocol ID: UMIN000034205).
PubMed: 38874430
DOI: 10.1111/1346-8138.17324 -
The Journal of Dermatological Treatment Dec 2024Response rates of approved systemic therapies for cutaneous T-cell lymphoma (CTCL) hover near 30%, suggesting unmet need. This study describes real-world treatment...
BACKGROUND
Response rates of approved systemic therapies for cutaneous T-cell lymphoma (CTCL) hover near 30%, suggesting unmet need. This study describes real-world treatment patterns and response rates of extracorporeal photopheresis (ECP) in CTCL patients.
METHODS
A chart review was conducted in the United States of adults with CTCL who initiated ECP between January 1, 2017, and February 28, 2019, and received at least three months of ECP treatment as monotherapy or concomitant therapy. Clinical outcomes were collected quarterly for up to 18 months.
RESULTS
The 52 patients were predominantly Caucasian. Half were male; median age was 69 years. Most patients had Sézary syndrome (50%) or mycosis fungoides (36.5%). Nearly 40% of patients had stage IV disease; 33% had lymph node involvement. Nineteen patients (36.5%) achieved response (>50% reduction in BSA affected); median time to response was 6.5 months. The percentage of patients rated as at least minimally improved was 59.5% at 6 months ( = 22), 75.0% at 9 months ( = 24), and 60.0% at 12 months ( = 15) after ECP initiation.
CONCLUSIONS
Despite the ECP treated population in this study being older and having more advanced-stage disease than recent trials, response rates were comparable. These real-world findings support ECP as an effective treatment option for CTCL patients.
Topics: Humans; Photopheresis; Male; Female; Aged; Middle Aged; Lymphoma, T-Cell, Cutaneous; Skin Neoplasms; United States; Treatment Outcome; Retrospective Studies; Adult; Aged, 80 and over; Sezary Syndrome; Mycosis Fungoides; Neoplasm Staging
PubMed: 38852942
DOI: 10.1080/09546634.2024.2360568 -
Archives of Dermatological Research Jun 2024Mycosis fungoides (MF) is the most common primary cutaneous T-cell lymphoma (CTCL) with its etiology not yet fully understood. Interleukin (IL)-35 is an inhibitory...
Mycosis fungoides (MF) is the most common primary cutaneous T-cell lymphoma (CTCL) with its etiology not yet fully understood. Interleukin (IL)-35 is an inhibitory cytokine that belongs to the IL-12 family. Elevated IL-35 in the plasma and the tumor microenvironment increases tumorigenesis and indicates poor prognosis in different types of malignancies. The objective of this study is to estimate the expression levels of IL-35 in tissue and serum of MF patients versus healthy controls. This case-control study included 35 patients with patch, plaque, and tumor MF as well as 30 healthy controls. Patients were fully assessed, and serum samples and lesional skin biopsies were taken prior to starting treatment. The IL-35 levels were measured in both serum and tissue biopsies by ELISA technique. Both tissue and serum IL-35 levels were significantly higher in MF patients than in controls (P < 0.001) and tissue IL-35 was significantly higher than serum IL-35 in MF patients (P < 0.001). Tissue IL-35 was significantly higher in female patients and patients with recurrent MF compared to male patients and those without recurrent disease (P < 0.001). Since both tissue and serum IL-35 levels are increased in MF, IL-35 is suggested to have a possible role in MF pathogenesis. IL-35 can be a useful diagnostic marker for MF. Tissue IL-35 can also be an indicator of disease recurrence.
Topics: Humans; Mycosis Fungoides; Interleukins; Female; Male; Case-Control Studies; Middle Aged; Skin Neoplasms; Adult; Skin; Aged; Biopsy; Biomarkers, Tumor
PubMed: 38850434
DOI: 10.1007/s00403-024-03115-9 -
Skin Health and Disease Jun 2024Brief description of a syringotropic mycosis fungoides.
Brief description of a syringotropic mycosis fungoides.
PubMed: 38846681
DOI: 10.1002/ski2.353