Erythrodermic mycosis fungoides: A case report

BACKGROUND Mycosis fungoides is the most common primary cutaneous T-cell lymphoma,whereas generalized erythroderma is rare.In this report,we describe a case of mycosis fungoides with generalized erythroderma using complete clinical data and[18F]fluoroDglucose positron emission tomography/computed tomography(18F-FDG PET/CT)images.CASE SUMMARY Systemic skin redness with desquamation for three years confirmed mycosis fungoides within one month.The patient underwent left axillary lymphadenectomy biopsy;pathological biopsy suggested abnormal T-cell lesions consistent with mycosis fungoides involving lymph nodes.The patient received methotrexate,5 mg twice weekly,as part of their chemotherapy regimen.Patients January half after discharge,no obvious cause of high fever,left axillary lymph nodes with red heat pain,and rupture entered our hospital for treatment.CONCLUSION The 18F-FDG PET/CT is essential for early diagnosis and timely treatment.

Ultrasound-guided microwave ablation as a palliative treatment for mycosis fungoides eyelid involvement:A case report

BACKGROUND Mycosis fungoides(MF)is a form of lymphoma derived from heterogeneous T cells,and eyelid involvement is extremely rare.The common methods to treat eyelid involvement are radiotherapy and chemotherapy,but their efficacies are limited.Herein,we report a case of advanced-stage MF eyelid involvement,propose ultrasound(US)-guided microwave ablation(MWA)therapy and present a literature review.CASE SUMMARY A male patient was admitted to our hospital in June 2018 and diagnosed with MF via radiological and histopathological examinations.The patient’s condition was not well controlled by various conventional chemotherapies.US-guided MWA was performed to relieve the patient’s symptoms and improve his quality of life,showing satisfactory efficacy.CONCLUSION Eyelid involvement is one of the most troublesome clinical problems for advanced-stage MF patients.This is the first report on the use of US-guided MWA as a palliative therapy for MF eyelid involvement;the treatment successfully relieved the patient’s clinical symptoms and reduced his anxiety behaviours.Our study sheds new light on methods for improving the clinical management of eyelid involvement in MF.

Diagnostic significance of TCR gene clonal rearrangement analysis in early mycosis fungoides

Mycosis fungoides(MF),the most common type of cutaneous T-cell lymphoma,has various unspecific clinical and histological characteristics.Its early diagnosis is challenging.The application of T-cell receptor(TCR) gene clonal rearrangement to the diagnosis of MF has been widely studied.In this study,we used polymerase chain reaction(PCR) to investigate the diagnostic significance of detecting TCR-γ and-β gene clonal rearrangement in the early diagnosis of mycosis fungoides.PCR for TCR-γ and TCR-β gene rearrangement was performed on 19 patients with suspected early MF,6 with typical MF,and 6 with chronic dermatitis.Of the 19 patients with suspected early MF,13 had TCR-γ gene clonal rearrangement,whereas none had TCR-β gene clonal rearrangement.All patients with typical MF had TCR gene clonal rearrangement,in which 4 showed TCR-γ clonal rearrangement,1 showed TCR-β gene clonal rearrangements,and 1 showed both.No patients with chronic dermatitis had TCR gene clonal rearrangement.These results indicate that TCR gene clonal rearrangement analysis is a useful tool in diagnosing early MF.TCR-γ gene is recommended to the routine analysis,whereas TCR-β gene has potential in combination toward intractable cases.

Palliative Local Radiotherapy in the Treatment of Tumor-stage Cutaneous T-cell Lymphoma/ Mycosis Fungoides

Objective To determine the efficacy of palliative radiotherapy in treating tumor-stage cutaneous T-cell lymphoma/mycosis fungoides(MF).Methods From January 2008 to January 2013,a total of 11 patients with tumor-stage MF were treated with local radiation therapy in Peking Union Medical College Hospital.The median age of these patients was 53.36±14.45 years.Female-male ratio was 1:1.2.The average course of disease was 10.82±3.37 years.All the patients were treated with local electronic beam irradiation with a total median dosage of 48.55±9.51(40-74) Gy in an average of 24.55±5.57(20-40) fractions,5 fractions per week.Results The median follow-up time was 55.27±29.3(13-103) months.No severe acute or chronic side effects of irradiation were observed.Complete clinical response(CR) rate of the radiated sites was 54.5%(6/11),partial response(PR) rate was 36.4%(4/11),and the overall response rate(CR+PR) was 90.9%.One patient showed no response.Conclusion Local radiotherapy with psolaren plus ultraviolet A and/or interferon maintaining treatment is an effective palliative therapy in the treatment of tumor-stage MF patients.

Papular mycosis fungoides: Six new cases and association with chronic lymphocytic leukemia

Papular mycosis fungoides(MF) is a rare presentation of MF. Six illustrative cases of papular MF were retrospectively reviewed. Five of the cases studied by immunohistochemistry had variable numbers(range: 1%-20%) of CD30+ cells in the dermal infiltrate, a finding that is characteristic of lymphomatoid papulosis but may occasionally occur in typical early MF. Although none of our papular MF patients had progressive disease, lesions with relatively high numbers of CD30+ cells in 3 patients did not respond well to skin-directed treatments used for MF. Interestingly, these patients had evidence of coexisting clonal B cell populations in the blood(one with clonal B cell lymphocytosis and two with B-cell chronic lymphocytic leukemia). We conclude that:(1) papular MF may contain CD30+ cells, thereby causing confusion with lymphomatoid papulosis; and(2) papular MF, like more typical MF, may be associated with clonal B-cell proliferations including chronic lymphocytic leukemia.

A case of advanced mycosis fungoides with comprehensive skin and visceral organs metastasis:sensitive to chemical and biological therapy

Mycosis fungoides is a common cutaneous T-cell lymphoma,which is usually characterized by chronic,indolence progression,with absence of typical symptoms in early stage,metastasis to lymph nodes,bone marrow and visceral organs in later stage and ultimately progression to systemic lymphoma.It can result in secondary skin infection which is a frequent cause of death. At present,no curative therapy existed.Therapeutic purpose is to induce remission,reduce tumor burden and protect immune function of patients.A case of patient with advanced severe mycosis fungoides receiving CHOP plus interferon a -2a was reported here,with disease-free survival of 7 months and overall survival of over 17.0 months,and current status as well as developments of mycosis fungoides were briefly introduced.

Pancreatic metastasis from mycosis fungoides mimicking primary pancreatic tumor

Mycosis fungoides(MF) is a cutaneous T-cell lymphoma that can undergo local progression with possible systemic dissemination. We report a case of a patient affected by MF with a pancreatic mass that was a diagnostic challenge between primitive tumor and pancreatic metastasis from MF. Clinical setting findings and imaging studies raised the suspicion of a pancreatic primary neoplasm. A diagnostic clue was provided by the combined histomorphologic/immunohistochemical study of pancreatic and cutaneous biopsies, which revealed a pancreatic localization of MF. Considering the rarity of metastatic localization of MF to the pancreas, we next investigated whether chemokinechemokine receptor interactions could be involved in the phenomenon to provide new insight into the possible mechanisms underlying metastatic localization of MF to the pancreas. Histological analyses of archival pancreatic tissue demonstrated that glucagon-secreting cells of the pancreatic islets expressed the CCL27 chemokine, which may have attracted in our case metastatic MF cells expressing the complementary receptor CCR10.

Mycosis Fungoides in Relation to Environmental and Occupational Exposures: A Case-Control Study in the Tunisian Center

Introduction: The mycosis fungoides (MF) is the most common cutaneous T-cell lymphomas representing about 50% of all cutaneous lymphomas. Its pathogenesis remains poorly understood. Thus, its rising incidence rates call for an explanation. Several environmental and occupational factors have been implicated in the pathogenesis of this disease. Objective: To determine the potential occupational factors correlated in the occurrence of MF. Methods: Case-control study was carried out at the teaching Hospital Farhat Hached of Sousse (Tunisia) to investigate associations of MF and occupational risk factors. A questionnaire on lifetime job history was administered to 24 cases and controls. Cases were patients with MF and whose diagnosis was suspected by a dermatologist and confirmed by pathological examination. Controls were patients consulting in the occupational medicine department and matched to cases by age and gender. Bivariate analysis was used to calculate odds ratios (OR) and 95% confidence intervals (CI). Results: The average age of onset of the disease was 55.41 ± 17.4 years with a sex ratio of 2.42. We found an increased risk of MF associated with tobacco consumption higher than 40 Pack-year (OR = 6.6;95% CI = 1.22 to 35.4), exercise in the textile sector (OR = 9.4;CI = 1.06 to 84.3) and exposure to diesel exhaust (OR = 9.47;95% CI = 1.06 to 84.3). Conclusion: Occupational factors could play a role in the pathogenesis of MF. Regarding our study, smoking, working in the textile industry and exposure to diesel exhaust appear to be involved in the pathogenesis of MF. Further investigation is needed to confirm these findings.

High Dose Rate (HDR) Brachytherapy for Mycosis Fungoides of the Wrist

Mycosis fungoides (MF) is the most common cutaneous T-cell lymphoma accounting for approximately half of all cutaneous T-cell lymphomas. Radiation therapy is an effective treatment for early stage MF and has been shown to result in long-term disease-free intervals, with even curative potential. Radiation is also effective as palliative treatment for the localized lesion resistant to the topic or other treatments. In the current study, we report using high dose rate (HDR) radiation treatment for a patient with resistant mycosis fungoides involving the wrist. We report a convenient treatment with an ideal radiation dose distribution, and a excellent clinical outcome.

DETECTION OF TELOMERASE ACTIVITY IN PATIENTS WITH MYCOSIS FUNGOIDES

Objectives. To detect telomerase activity in patients with mycosis fungoides (MF) and to study therole of telomerase in the tumorigenesis of MF.Methods. The technique of PCR-ELISA was employed to detect telomerase activity in 35 patientswith various stages of MF.Results. 92.3% tumor stage of MF, 78.6% plaque stage of MF and 75.0% patch stage of MF hadpositive telomerase activity. The control samples had no telomerase activity. Telomerase activity in tumorstage of MF was significantly higher than that in plaque stage, while the latter was higher than that inpatch stage. Telomerase activity was correlated with the stage of MF.Conclusion. High level of telomerase activity frequently occurred in patients with MF, suggestingthat telomerase might play an important role in the tumorigenesis of MF and is a useful marker for thediagnosis of MF possibly.

Mycosis Fungoides in Iraqi Patients—Clinical, Histopathological and Immunohistochemical Study

Background: Mycosis fungoides (MF) is not an uncommon T-cell lymphoma in Iraqi population which is increasing over years. There are many diagnostic techniques to confirm the diagnosis including histopathological and immunohistochemical tests. Objectives: To evaluate all cases of MF including clinical, histopathological and immunohistochemical tests with CD markers. Patients and Methods: This clinical, histopathological, immunohistochemical outpatient based study took place in the Department of Dermatology, Baghdad Teaching Hospital, Medical City, Baghdad, Iraq during the period from May 2012-September 2013. Twenty five patients with MF were included in the present work. History was obtained from each patient regarding all socio-demographic aspects related to the disease. Also, clinical evaluation was carried out for all patients. Incisional biopsies for ordinary histopathology and immunohistochemical tests were done. Then staging was carried out depending on TNMB classification for all patients. Results: All cases of MF were confirmed after clinical evaluation, histopathology and immunohistochemical examination. Male to female ratio was 1.5:1. The mean age of onset at presentation was 47.45 ± 16.9 years. Itching was found in 84% of cases. The patch stage was seen in 36% patients. The hypopigmentaed MF was presented in 12% cases. The lower extremities were seen in 80%. Lymph node involvement as a manifestation of the disease was seen in 44% cases. The histopathological features of MF went parallel with the clinical stage of disease. Immunohistochemical study aids in the diagnosis of patch and erythrodermic stages. The most patients presented with stage IB (36%). Conclusion: MF seems to be not uncommon problem in Iraqi population and is increasing over years. The disease was diagnosed early commonly with patch stage (stage IB) while hypopigmented MF was not a common variant when compared with neighboring countries.

Mycosis fungoides: A mimicker of benign dermatoses

Mycosis fungoides, the most common primary cutaneous lymphoma, may present with a broad spectrum of clinical features. As both clinical and dermatopathological findings in mycosis fungoides occasionally closely imitateother dermatoses, correct diagnosis may be a challenge both for clinicians as well as dermatopathologists. As a consequence, diagnosis of cutaneous lymphoma may be initially missed and, therefore, prompt and adequate therapeutic measures delayed. Hence, the purpose of our article was to give an overview of hitherto published "mimickers" of mycosis fungoides with a review of its diverse clinical features to alert the clinicians about the wide spectrum of this dissimulating disease. By integrating our own encountered atypical cases of mycosis fungoides we provide a comprehensive illustrated histological and moleculargenetic workup thereof and thereby critically revise the different available diagnostic tools of daily routine. Finally, we derive a practical algorithm to obtain the correct diagnosis even in such ambiguous cases of mycosis fungoides.

Mycosis fungoides and Sézary syndrome: Role of chemokines and chemokine receptors

Mycosis fungoides is the most common form of cutaneous T-cell lymphoma(CTCL), and is characterized by a clonal expansion of malignant CD4+ T lymphocytes with skinhoming properties. Clinically and pathologically, mycosis fungoides can be categorized into patch, plaque and tumor stages. The clinical course of mycosis fungoides is usually chronic and indolent, but a proportion of patients may develop progressive disease with peripheral blood, lymph node and visceral organ involvement. Sézarysyndrome is an aggressive leukemic form of CTCL characterized by a clonal population of malignant T cells in the peripheral blood. Various forms of skin-directed and systemic treatments are available for mycosis fungoides and Sézary syndrome. However, current treatments are generally not curative, and can only control the disease. Currently, the etiology and pathogenesis of mycosis fungoides and Sézary syndrome are not well defined. Proposed mechanisms include chronic antigenic stimulation by infectious agents, expression of specific adhesion molecules, altered cytokine production, mutations of oncogenes and tumor suppressor genes, and avoidance of apoptosis. In recent years, a number of chemokine receptors and their corresponding chemokine ligands have been found to contribute to the migration and survival of lymphoma cells in mycosis fungoides and Sézary syndrome, including CC chemokine receptor 4(CCR4), CCR10, C-X-C chemokine receptor type 4(CXCR4), CCR7, CCR3 and CXCR3. Since chemokines and chemokine receptors have been found to play important roles in the pathophysiology of mycosis fungoides and Sézary syndrome, they may be potentially useful targets for the development of new treatments for these diseases in the future.

Treatment of mycosis fungoides, in the era of stem cell transplantation

Mycosis fungoides and Sèzary syndrome are the most common subtypes of cutaneous T-cell lymphomas. Even though, in early-stage disease, Mycosis fungoides commonly has a more indolent course, disease will progress in about 20% of such patients. About 30% of patients have been reported to develop advancedstage disease and, at present, there is no cure for the disease. A number of systemic approaches have been used for advanced-stage mycosis fungoides(IIB-IV) and transformed disease. Aggressive approaches seem to be warranted in such patients. The scope of this review is the stem cell transplantation in mycosis fungoides and its leukemic variant, Sèzary syndrome.

Mycosis Fungoides: Epidemiology in Isfahan, Iran

Background: Mycosis Fungoides (MF) is the most common and indolent form of Cutaneuse T-cell Lymphomas (CTCL), that usually occurs in old adults. Objectives: To determine epidemiologic features and patients characteristics of MF in Isfahan (Iran). Methods: We performed a retrospective study in MF clinic of alzahra hospital that is the main center for treatment of MF patients in Isfahan (Iran) and evaluated clinicopathologic features. Results: In 3 years 25 patients were referred to Alzahra MF clinic. 18 patients diagnosed as MF. Seven (38.9%) patients were male and 11 (61.1%) were female with male to female ratio of 1:1.57. The mean age of patients was 41.06 years. 88.9% of our patients were in stages ΙA and ΙB. Conclusion: Most of our patients presented in early stages that were similar to other studies, while male: female ratio is different from other studies.

Hypopigmented Mycosis Fungoides in a 7-Year-Old Boy

Hypopigmented mycosis fungoides (HMF) is an uncommon variant of cutaneous T-cell lymphoma. It is more frequent in dark-skinned people, particularly children. The HMF diagnose is difficult, especially in early stages because this condition resembles benign skin diseases. Thus is histopathological analysis very important for the diagnosis. We report a case of a 7-year-old child with widespread HMF confirmed by histopathology that showed cells tagging along the dermal/epidermal junction and extending into the epidermis in a pattern of epidermotropism and focal cell aggregates in the epidermis (Pautrier’s microabscess). We demonstrate the importance of clinical suspicion for this cutaneous neoplasia in patients with hypopigmentated lesions.

Interstitial Mycosis Fungoides with Systemic Sclerosis-Like Features: A Case Report

Introduction Mycosis fungoides (MF) is the most common form of cutaneous lymphoma,accounting for almost 50% of all types.1 MF occasionally manifests as the interstitial lymphocytic infiltrate that mimics inflammatory morphea,interstitial granulomatous dermatitis,and interstitial granuloma annulare,and this rare histopathological variant has been termed interstitial mycosis fungoides (IMF).IMF can be considered as a transient histopathological pattern in conventional MF,with common clinical presentation as patches and plaques.

14例儿童色素减退型蕈样肉芽肿诊疗分析

回顾性分析2015年7月至2020年7月在武汉市第一医院皮肤科经临床和病理确诊的14例儿童色素减退型蕈样肉芽肿(HMF)临床、影像、病理、治疗及随访资料。14例患儿中男5例,女9例。诊断年龄4~13岁,发病年龄3~12岁,病程9个月~7年。皮损表现为大小不等、不规则形或类圆形,边界欠清晰的色素减退斑,局部融合。皮肤镜下见色素减退呈波点状、斑马样和网格状模式,血管呈点状、精子样、短细线状和星状血管结构模式。反射式共聚焦显微镜(RCM)下色素减退斑均表现为基底层色素轻度减退,未见明显色素缺失,真表皮交界处及真皮浅层可见较多高折光异形细胞。皮损组织病理示棘层下部和基底层可见异形淋巴细胞浸润,核深染,部分扭曲呈脑回状,部分聚集呈Pautrier微脓疡。免疫组化:CD3均阳性,CD20均阴性,4例仅CD8阳性,10例CD8、CD4均阳性。12例患儿采用NB-UVB照射治疗,8例完全消退,4例部分消退,3例复发再次照射后完全消退。2例分别给予糠酸莫米松和他克莫司治疗,6个月和8个月后消退面积>80%。本研究显示皮肤影像有一定辅助诊断价值,NB-UVB治疗HMF安全有效,HMF预后一般良好,但仍需警惕治愈后有疾病复发及进展可能,需进行长期随访。