Frequency of skin cancer among psoriasis, vitiligo, and mycosis fungoides patients treated with narrowband ultraviolet B phototherapy.

BACKGROUND: Narrowband ultraviolet B (NB-UVB) phototherapy is a popular and relatively contemporary treatment option. However, only a few studies to date have explored the potential risk of skin cancer following NB-UVB treatment. OBJECTIVE: This study aimed to investigate the potential long-term risk of skin cancer in patients treated with NB-UVB. METHODS: This cohort study included patients with psoriasis, vitiligo, and mycosis fungoides treated with NB-UVB at two university hospitals in Israel in 2000-2005. Patients were followed up for skin cancer for at least 10 years. Data were extracted from the hospital and community medical records. RESULTS: A total of 767 patients were included in this study: 509 with psoriasis, 122 with vitiligo, and 136 with mycosis fungoides. The mean follow-up duration was 13 years. Among these patients, 4.43% developed skin cancer during the follow-up (3.93% had psoriasis, 2.46% had vitiligo, and 8.09% had mycosis fungoides). Old age and fair skin type were the only significant independent risk factors for skin cancer. There was no significant difference in the mean number of NB-UVB treatments among patients who developed skin cancer and those who did not (99.09 vs. 94.79, respectively). CONCLUSION: No association was observed between the number of NB-UVB treatments and carcinogenesis in any study group. Age is a significant risk factor, and older patients treated with NB-UVB should be followed up carefully.

Paediatric Mycosis Fungoides: Clinical Variants, Treatment Modalities and Response to Therapy.

Mycosis fungoides is a rare cutaneous lymphoma in the paediatric population. The aim of this study was to examine the epidemiological, clinical, and histological characteristics, as well as the treatment modalities and response to therapy of paediatric patients with mycosis fungoides. This retrospective cohort study reviewed the records of 37 paediatric patients treated at Rambam Medical Center, Israel, between 2013 and 2021. Extracted data included epidemiology, clinical presentation, histological reports, infiltrate clonality status, treatment modalities and response to therapy. The mean follow-up period was 60 months. All patients were diagnosed with stage IA or IB disease. Folliculotropic mycosis fungoides was the most prevalent variant (49%). Most patients were treated with phototherapy (90%), with a response rate of 85%, and a complete response rate of 55% after the first course. There were no significant differences in response to phototherapy between the folliculotropic or other variants (p = 0.072). Similarly, delayed diagnosis, atopic diathesis, clonality, phototherapy type or number of treatments, were not associated with response to therapy, while protracted phototherapy was associated with prolonged remission. In conclusion, mycosis fungoides in the paediatric population is an indolent disease with a favourable prognosis and potentially prolonged response to phototherapy.

Clinical Outcomes of COVID-19 in Mycosis Fungoides and Sezary Syndrome.

INTRODUCTION: Haemato-oncologic patients are more susceptible to severe infections with SARS-CoV-2. We aimed to assess the clinical outcomes of SARS-CoV-2 infection among patients with Mycosis Fungoides and Sezary Syndrome (MF/SS). METHODS: The data were retrieved from anonymized electronic medical records of Maccabi Healthcare Services (MHS), the second-largest healthcare organization in Israel. Patients diagnosed with MF/SS were included in the study. COVID-19 PCR test results together with sociodemographic and clinical data were extracted and analyzed to evaluate the association of COVID-19 with clinical outcomes. RESULTS: In the period of 2020-2022, 1,472 MF/SS patients were included in the study. Among them, 768 (52%) had SARS-CoV-2 infection. The hospitalization rate was 2.9% and infection by the Delta variant was associated with the highest hospitalization rate (7.7%). The hospitalization rate was lower among fully vaccinated patients (p = 0.032) but higher for patients older than 65 (p < 0.001) and patients with SS (vs. MF) (p < 0.001) or COPD (p = 0.024) diagnosis. There was a tendency for decreased hospitalization among patients treated with nirmatrelvir + ritonavir within 5 days of infection, with a 79% risk reduction, although it was not statistically significant (p = 0.164). CONCLUSION: Patients with MF/SS do not necessarily have worse COVID-19 outcomes compared to the general population.

Skin Cancer in Skin of Color.

Skin cancer is often associated with greater morbidity and mortality in skin of color patients because most medical literature and research on skin cancer to date has been predominantly focused on lighter skin types. It is crucial that dermatologic providers be able to recognize different presentations of skin cancer in skin of color patients to optimize the early detection of these tumors and ensure equitable outcomes. This article details the epidemiology, risk factors, clinical features, and disparities in the treatment of melanoma, squamous cell carcinoma, basal cell carcinoma, and mycosis fungoides subtype of cutaneous T-cell lymphoma in skin of color patients.

Pediatric Primary Cutaneous Lymphoma in China: A Retrospective Study.

BACKGROUND: The incidence and clinical features of primary cutaneous lymphoma (PCL) tend to differ by age, gender, geographical, and racial variation. All-aged and adult groups of PCL in various regions have been well demonstrated and compared, while the research concentrating on pediatric PCL is rare, especially in Asian countries. OBJECTIVE: The aim of this study was to investigate the clinical characteristics of PCL in pediatric population at a single center in China. METHODS: We conducted a retrospective study of 101 pediatric cases with PCL, diagnosed at the Institute of Dermatology, Chinese Academy of Medical Sciences, from January 2010 to December 2021. RESULTS: Mycosis fungoides (MF), accounting for 41.6% of the total cases, was the most common subtype in pediatric PCL, and the hypopigmented MF accounted for 47.6% of all the MF cases. Lymphomatoid papulosis and chronic active Epstein-Barr virus infection tied for second place with a proportion of 22.8%. Primary cutaneous anaplastic large cell lymphoma, subcutaneous panniculitis-like T-cell lymphoma, primary cutaneous peripheral T-cell lymphoma, rare subtypes and primary cutaneous B-cell lymphoma, respectively, accounted for 2.0%, 4.0%, 4.0%, and 3.0%. Most patients had favorable prognosis during the follow-up. CONCLUSION: The study suggested that MF was the most common subtype in pediatric PCL in China, and most types of pediatric PCL had favorable prognosis.

Epidemiology and prognostic factors of 114 patients with mycosis fungoides in a Moroccan cohort: a 29-year review.

Limited data regarding survival of Moroccan patients with mycosis fungoides (MF). To evaluate the clinical profile and long-term outcomes of these patients. A retrospective review of 114 MF cases diagnosed from 1993 to 2022 who were followed up for more than 6 months of diagnosis was performed. Of 114 patients, 71.9% were male and the median age at diagnosis was 56 years. Approximately 64 and 36% of the patients had an early stage and advanced stage, respectively. Median follow-up duration was 56 months, and median duration of symptoms before diagnosis was 31 months. Various subtypes were observed, including mycosis fungoides folliculotropic (12.3%), poikilodermatous (11.4%), and palmaris et plantaris MF (5.3%). The 10-year overall survival was 89% in early-stage patients and 48.8% in advanced-stage patients. Complete response to treatment occurred in 45.6%, stable disease in 16.7% and disease progression in 7.9% of patients. Older age of > 60 years, higher T-stage (T3/T4) and advanced-stage MF were statistically significant in predicting poorer outcomes in MF. Despite delay in diagnosis, most cases of MF in Morocco were diagnosed in early stages. We observed a high proportion of classic MF and favorable prognosis.

Clinicopathologic characteristics and outcomes of patients with mycosis fungoides: A single tertiary center retrospective analysis in Saudi Arabia.

OBJECTIVES: To determine the distinctive clinical and pathological characteristics and outcomes of MF in a Saudi population. METHODS: We conducted a retrospective analysis of all MF cases diagnosed clinically with pathological confirmation at King Abdulaziz Medical City in Riyadh from January 2016 to July 2022. Variables include demographical, clinical, and pathological MF traits and disease outcomes. RESULTS: A sum of 73 patients were enrolled. The mean age was 44 years. The female-to-male ratio was 1.3:1.The mean duration between cutaneous manifestations and MF diagnosis was 33 months (2.7 years). Classic MF was the most common variant (60.3%), followed by hypopigmented MF (20.5%). Most patients (82.2%) had early-stage MF (IA, IB, and IIA). Patients who had CD4+/CD8+ with CD8 predominance had a favorable disease course (p=0.029). Topical corticosteroids were the most frequently prescribed treatment (79.5%). Three patients (4.1%) died from MF. The disease-specific survival rate for advanced-stage MF was 84.6%, which was significantly lower compared to early-stage MF (p=0.032). CONCLUSION: Among the Saudi population, MF has an earlier onset and slightly higher prevalence in females. Hypopigmented MF is more prevalent in this ethnic group. Immunohistochemical staining of CD4+/CD8+ with CD8 predominance may elucidate a favorable disease course.

Risk of skin cancers in mycosis fungoides patients receiving PUVA therapy: A real-life experience from a single tertiary center.

BACKGROUND: Mycosis fungoides (MF) is the most common cutaneous T-cell lymphoma. Skin-directed therapies, including phototherapy, are the first-line treatment modalities. Psoralen plus ultraviolet A light photochemotherapy (PUVA) is quite effective in controlling the disease; however, long-term adverse effects, particularly carcinogenesis, are the cons of this treatment. OBJECTIVE: There are various studies on the negative impact of PUVA on skin cancer in patients with autoimmune skin diseases. The data on the long-term effects of phototherapy on MF patients are scarce. METHODS: All MF cases that received PUVA alone or combined with other treatments at a single tertiary center were analyzed. This study compared the development of non-melanoma skin cancers, melanoma, and solid organ tumors in MF patients with at least 5-year follow-up data with age- and sex-matched controls. RESULTS: A total of 104 patients were included in the study. Ninety-two malignancies were detected in 16 (15.4%) patients, and six developed multiple malignancies. Skin cancers consisted of 56 basal cell carcinomas, 16 Bowen's disease, four squamous cell carcinomas, three melanomas, two basosquamous cell carcinomas, one Kaposi sarcoma, and one keratoacanthoma were found in nine (8.7%) patients. Eight patients developed three solid cancers and six lymphomas. The risk of developing skin cancer was associated with the total number of PUVA sessions (<250 vs ≥250 sessions; hazard ratio (HR) 4.44, 95% confidence interval (CI) 1.033-19.068; p = .045). 9 (13.2%) of 68 patients who had follow-ups for at least 5 years developed skin cancer. Compared to an age- and sex-matched cohort, the prevalence of new skin cancer was considerably greater (p = .009). CONCLUSIONS: Patients with MF are predisposed to develop secondary malignancies, and continual exposure to PUVA may potentiate this risk. Annual digital dermoscopic follow-up in MF patients treated with UVA is advised for early diagnosis and treatment of secondary cutaneous malignancies.

Care structure of patients with mycosis fungoides and Sézary syndrome in Germany - Care research based on SHI claims data.

BACKGROUND: Cutaneous T-cell lymphomas (CTCLs) are rare forms of non-Hodgkin's lymphoma of T-cell origin that occur mainly in the skin. The most common form is mycosis fungoides (MF), but Sézary syndrome (SS), a more aggressive form of CTCL, is another relevant subgroup. Due to the rare nature of the disease, population-based studies of the epidemiology and disease burden and insights into care delivery are limited. PATIENTS AND METHODS: Based on an anonymized, age and sex-adjusted routine dataset comprising approximately five million people with statutory health insurance, a retrospective, longitudinal healthcare research study was conducted over a six-year period (2012-2017). RESULTS: In 55 % of patients with MF and SS, the initial diagnosis was documented in an outpatient setting; in 59 % of cases by a dermatologist. Immunophenotyping by flow cytometry is considered an important investigative tool for the detection and follow-up surveillance of blood involvement of cutaneous lymphomas, as the disease stage is the most important prognostic factor in MF and SS; this was performed in only 10 % of patients. The first-line treatment was topical (76 %), in particular with corticosteroids (66 %). CONCLUSIONS: The findings from this healthcare research point to the need for increased guideline-based care.

African American and Caucasian patients with Sézary syndrome have no differences in outcomes at an ethnically diverse urban medical center.

Sézary syndrome (SS) is an aggressive cutaneous T-cell lymphoma with poor survival. We performed a retrospective review of SS patients at Emory University from 1990 to 2020. We collected data on race, clinical characteristics, therapy, and social determinants of health. Clinical endpoints were overall survival (OS) and time to next treatment (TTNT). Univariate association and multivariable analyses were assessed by Cox proportional hazards models. Among 62 patients, 45.2% were AA. The median OS and TTNT were 3.1 years and 6.3 months, respectively, with no difference by race. AA patients had a higher median baseline LDH (360 vs. 232, p = 0.002) and a longer delay in initiation of systemic therapy compared to CC patients (3.17 vs. 2.14 months, p = 0.039), but a shorter commute (<10 miles) and no difference in insurance coverage (p = 0.260). AA patients at an academic center had unique clinical features and treatment patterns, but similar survival to CC SS patients.

Mycosis fungoides in pediatric population: comprehensive review on epidemiology, clinical presentation, and management.

Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma. However, it is rare in pediatric population. Most of the cases of pediatric MF present with hypopigmented patches and/or various other forms, which may often mimic common childhood dermatoses, thereby causing a delay in the diagnosis. There are no established treatment guidelines for pediatric MF. As the progression of childhood MF is extremely rare and it has an indolent course, it is usually diagnosed at an early stage (IA, IB, IIA), and hence phototherapy with a response rate of >80% is a well-established effective treatment in children. However, as recurrences are frequently seen on stopping the therapies, a maintenance regimen and long-term follow-up is equally important. This article reviews the epidemiological factors, clinical presentations, diagnosis, and various treatment modalities used in pediatric MF. We analyzed and compared the data of almost 616 childhood MF cases from various studies undertaken from 1988 to 2021.

Characteristics and outcomes of 727 patients with mycosis fungoides and Sézary syndrome from a Brazilian cohort.

BACKGROUND: Mycosis fungoides (MF) and Sézary syndrome (SS) are the most prevalent cutaneous lymphomas. They were not described in a large Brazilian cohort yet. We aimed, with this single-center, retrospective cohort analysis, to describe the characteristics and outcomes of MF/SS in a tertiary public health service in Brazil. METHODS: MF/SS patients evaluated at the University of São Paulo Medical School between 1989 and 2018 were included. Data were collected at diagnosis. Demographic, clinical, histopathological, immunopathological, molecular, laboratory, and follow-up data were analyzed. RESULTS: Among 727 patients, 92.6% (673) were diagnosed with MF, 7.4% (54) with SS. There were 51.2% (372) of males, 48.8% (355) of females. The median age was 51.8 years; it was higher in erythrodermic MF (60.2) and SS (60.9). Among MF, 41.8% (281) had classic MF, 4.9% (33) folliculotropic MF, 1.8% (12) granulomatous slack skin, and 0.3% (2) pagetoid reticulosis. Common subtypes included erythrodermic (14.1%, 95), hypopigmented (10.8%, 73), and poikilodermatous MF (10.8%, 73). Extracutaneous involvement was rare. Five, 10, 20, and 30-year overall survival rates were 97.3%, 92.4%, 82.6%, and 82.6% for early-stage, and 58.6%, 42.7%, 20.8%, and 15.4% for advanced-stage disease, respectively. After multivariate analysis, SS diagnosis, folliculotropic MF, erythrodermic MF, clinical stage, age (≥60 years), increased lactate dehydrogenase, and large cell transformation conferred poorer prognosis. CONCLUSIONS: We observed a higher percentage of hypopigmented MF compared to the literature, and demographic (older age) and prognostic (poorer prognosis) similarities between erythrodermic MF and SS, suggesting a possible relationship between these erythrodermic lymphomas. Factors associated with a poorer prognosis were compatible with the literature.

Primary Cutaneous Lymphomas in Thailand: A 10-Year Retrospective Study.

BACKGROUND: Primary cutaneous lymphomas (PCLs) refer to cutaneous lymphomas that primarily develop in the skin with no evidence of extracutaneous disease at the time of diagnosis. The epidemiological and clinical data of PCLs in Thailand are lacking. OBJECTIVES: To evaluate the frequency, demographic data, and clinical characteristics of different subtypes of PCLs in a tertiary care university hospital. METHODS: In total, 137 patients with PCLs diagnosed in our hospital in 2008-2017 were retrospectively reviewed. RESULTS: Of the 137 patients, 57 (41.6%) were male and 80 (58.4%) were female (M : F = 1 : 1.4). The median age at diagnosis was 40 years. Most patients (134, 97.8%) had cutaneous T-cell lymphomas (CTCLs). Three patients (2.2%) had cutaneous B-cell lymphomas (CBCLs). The most common subtype was mycosis fungoides (MF) (67.9%), followed by subcutaneous panniculitis-like T-cell lymphoma (SPTCL) (21.2%), primary cutaneous anaplastic large cell lymphoma (pcALCL) (3.6%), lymphomatoid papulosis (LyP) (1.5%), primary cutaneous gamma/delta T-cell lymphoma (pcGDTCL) (1.5%), Sézary syndrome (SS) (0.7%), extranodal NK/T-cell lymphoma, nasal type (ENKTCL-NT) (0.7%), primary cutaneous peripheral T-cell lymphoma, not otherwise specified (pcPTCL-NOS) (0.7%), primary cutaneous diffuse large B-cell lymphoma, leg type (pcDLBCL-LT) (1.5%), and primary cutaneous follicle center lymphoma (pcFCL) (0.7%). Most patients with MF presented with early-stage disease (84.0%), with hypopigmented MF the most common variant (42.6%). CONCLUSIONS: Compared to earlier Caucasian and Asian studies, the present study revealed a higher proportion of CTCL patients with a younger age at onset and a female predominance. MF was the most common CTCL subtype, followed by SPTCL. More than 80% of MF patients were diagnosed at an early stage.

Screening for second malignancies in mycosis fungoides: non-Hodgkin lymphoma, Hodgkin lymphoma, lung cancer, bladder cancer and melanoma.

BACKGROUND: Patients with mycosis fungoides (MF) are at increased risk of developing non-Hodgkin lymphoma (NHL), Hodgkin lymphoma (HL), lung cancer, bladder cancer and melanoma. The characteristics of patients developing these malignancies have not been specifically delineated. In addition, there are no established guidelines for screening MF patients for second malignancies. MATERIALS/METHODS: We identified 742 patients with MF who developed second malignancies in the Surveillance Epidemiology and End Result-18 database. RESULTS: The majority of second malignancy patients were white and male, mean age 55-67 years at diagnosis of MF, and mean age 61-72 years at diagnosis of second malignancy. The majority of patients diagnosed with second malignancies had early stage MF. MF patients with NHL, lung cancer, and bladder cancer tended to be diagnosed at earlier stages of the second malignancy than patients without MF and demonstrated better 5-year overall survival. There was no improvement in stage at diagnosis or survival for MF patients who were diagnosed with melanoma compared to patients without MF. CONCLUSIONS: Improvements in survival in MF/NHL, MF/lung cancer and MF/bladder cancer patients may reflect differences in disease biology secondary to having MF or the importance of increased contact with the healthcare system. MF/melanoma data suggest that patients require regular pigmented-lesion-focused skin examinations. Tools for screening include regular lymph node examinations, pigmented-lesion-focused examinations and detailed review of systems questions. Smoking cessation counseling is key intervention in this population, as is ensuring that all age- and sex-specific cancer screenings are up-to-date (e.g. lung cancer screening, mammography, and colonoscopy). The utility of regular imaging for second malignancy screening and lab testing such as routine urinalysis requires additional study and expert consensus.