Mycosis fungoides bullosa

A case of a 52-year-old man with mycosis fungoides bullosa. plaque-stage IIA, follicular mucinosis and milia is described. The disease started about 15 years ago and evolved with periods of remission induced by photochemotherapy (PUVA) and, later, by topical nitrogen mustard and etretinate. Vesiculobullous lesions, alopecia and milia sequentially appeared in the course of its evolution. Besides characteristic features of mycosis fungoides, histopathology revealed subepidermal vesicles and follicular mucinosis associated with lymphoid cells (CD3⁺). The pathophysiology of these particular aspects of the mycosis fungoides is discussed.     

Mycosis fungoides with involvement of the oral cavity

Mycosis fungoides rarely involves the oral cavity. To our knowledge only 29 cases of oral cutaneous T-cell lymphoma have been described up to 1994. This report presents a case of mycosis fungoides with involvement of the oral cavity in a 57-year-old man who died from septicemia 7 months after the onset of oral involvement.     

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.     

Pathogenesis of Mycosis fungoides

Mycosis fungoides is the most common type of primary cutaneous lymphomas. The phenotype of the tumor cell corresponds to an effector/memory‐type of helper T cell which, given its repertoire of homing receptors, is specialized for recirculation through the skin. In recent years genetic analyses have uncovered various chromosomal aberrations in the tumour cells of mycosis fungoides. Their relevance to the pathogenesis and clinical appearance are discussed in the following.     

Mycosis fungoides responding to systemic itraconazole

Mycosis fungoides is a form of cutaneous lymphoma with a typically indolent course. We describe an unexpected, repeated response to a systemic antifungal agent, itraconazole. The mechanism of action is speculative but itraconazole has marked immunosuppressive activity on T-lymphocyte proliferation in vitro.     

Cutaneous immunoblastic T-cell lymphoma

Summary The case of a 69-year-old male patient with an unusual type of malignant lymphoma is presented. Clinically, it was at first characterized by follicular papules and erythematous patches, later, by the development of cutaneous tumors and enlarged lymph nodes, and by a severe, finally excruciating pruritus. Treatment with PUVA (psoralen-ultraviolet-A) combined with 40–80 mg prednisolone and then with chemotherapy [COPP regimen (cyclophosphamide, vincristine, procarbacine, prednisone), high-dosage methotrexate followed by citrovorum factor rescue] was not successful. The patient died of pneumonia 2.5 years after the onset of the first clinical symptoms. An immunoblastic infiltrate was observed histologically and electromicroscopically in the initial lesions of the skin. Therefore, the diagnosis of a cutaneous immunoblastic T-cell lymphoma was tentatively made at the beginning, which was later confirmed in numerous biopsies and laboratory investigations. Immunocytologically and enzymecytochemically, the infiltrating cells were shown to be immature T cells; in the lymph nodes, numerous immunoblasts and large Sézary cells were noted beginning in the paracortical areas and leading to the destruction of the normal lymph node structure. A maximum of 18% Sézary cells was observed in the peripheral blood, though there were no very large Sézary cells or blast cells. In the autopsy, a systemic involvement with an atypical lymphoid infiltration was found in numerous internal organs.The special nature of this case justifies its classification as high-grade malignant lymphoma and its differentiation from normal cases of mycosis fungoides. In contrast, mycosis fungoides generally fulfils criteria typical of low-grade malignant lymphomas.Supported by the Deutsche Forschungsgemeinschaft     

Illness perception,coping, and quality of life in early-stage Mycosis fungoides

BackgroundMycosis fungoides is the most common type of cutaneous T-cell lymphoma. Most early-stage mycosis fungoides cases follow an indolent course, hence considered by doctors a relatively easy condition. However, since mycosis fungoides bears the title of cancer, patients might perceive it differently.ObjectiveTo investigate patients’ illness perception, and its relationships to quality of life, depression, anxiety, and coping among early-stage mycosis fungoides patients.MethodsA cross-sectional questionnaire-based study was conducted. Patients from a single tertiary medical center completed the Revised Illness Perception Questionnaire, the MF/SS-CTCL Quality of Life scale, the Hospital Anxiety and Depression Scale, and The Mental Adjustment to Cancer Scale.ResultsThirty patients (25 males, five females, mean age 51.60) with stage I mycosis fungoides were enrolled. Mycosis fungoides had a little impact on patients’ daily life, quality of life, and levels of depression and anxiety, and they generally coped well. Disease understanding was low and was negatively correlated with impairment to quality of life and depression. Patients felt that stress and worry were features of the disease’s etiology.Study limitationsA small sample of patients was included.ConclusionPatients with early-stage mycosis fungoides adapt well to their disease. Psychological interventions should be aimed at improving patients coping style and enhancing illness understanding, in order to maintain high quality of life.     

掌跖蕈样肉芽肿一例

患者男,41岁。手足反复脓疱和脱屑1年,左足趾肿物4个月。体检：双手拇指、食指为主及掌侧大鱼际肌部位、双足趾及双足跖前1/3处可见不规则淡红色斑片,表面有小片状白色鳞屑,局部红斑基础上见深在性小水疱、脓疱,破溃后形成点状或融合成片状浅糜烂面,表面结黄褐色痂,部分趾指甲增厚、变形明显。左足第4趾掌跖关节前端一约4 cm × 3.5 cm × 3.5 cm肿物,质稍硬,表面糜烂,伴较多脓性分泌物渗出及新生肉芽组织生长,混合后形成较厚黑色血痂,触痛明显。甲床和足趾肿物组织病理检查：表皮、真皮全层和皮下脂肪弥漫性致密小到中等大淋巴细胞浸润,细胞有异形,其间混杂少量炎性细胞,亲表皮现象明显,见Pautier微脓疡。免疫组化检查：异形淋巴样细胞LCA、波形蛋白、CD3、CD45RO、CD4、Bcl-2均阳性,CD8、CD5、CD10均为散在阳性,Ki-67 ＞ 80%阳性,CD79a、CD20、CD30、细胞角蛋白、S-100、Bcl-6、间变性淋巴瘤激酶、HMB45、CD1a、P63均为阴性。诊断：掌跖蕈样肉芽肿。     

Zur Frage der filamentösen Einlagerung in einer Zelle des korialen Infiltrates bei Mycosis fungoides

Ohne ZusammenfassungMit Unterstützung der Deutschen Forschungsgemeinschaft     

蕈样肉芽肿的端粒酶研究

目的 检测各期蕈样肉芽肿(MF)的端粒酶活性,探讨端粒酶在MF肿瘤发生机制中的作用.方法 采用端粒酶聚合酶链反应-酶联免疫吸附测定法(PCR-ELISA),对35例MF患者进行端粒酶的定性和定量分析.结果 92.3%的肿瘤期MF、78.6%的斑块期MF和75.0%的斑片期MF显示端粒酶阳性;肿瘤期MF的端粒酶活性水平高于斑块期和斑片期,且差异有显着性;而斑块期与斑片期之间差异无显着性.对照组的端粒酶均为阴性.讨论 MF患者存在高水平的端粒酶活性,提示端粒酶可能在MF的肿瘤发生中起重要作用,对MF的诊断有一定的临床意义.     

Mycosis fungoides in pediatric patients: Clinical features,diagnostic challenges,and advances in therapeutic management

Mycosis fungoides (MF) is the most common primary cutaneous lymphoma in pediatric patients. Given the indolent nature of MF, symptoms often present in childhood but may not be diagnosed as MF until adulthood. Delayed diagnosis is associated with poor long-term prognosis. Thus, increased clinician recognition and accurate diagnosis of early-stage MF in pediatric patients is critically important. In this review, we summarize the clinical features of the most common pediatric MF subtypes and highlight important differences between pediatric and adult MF. Moreover, we reviewed all pediatric MF case series published between 2008 and 2018 to analyze treatment modalities and identify emerging therapies. As treatment of pediatric MF is complex, selection of therapy varies significantly depending upon the specific clinical characteristics, disease severity, and patients' preferences.     

临床表现为环状红斑的蕈样肉芽肿1例

患者女，６８岁，病期１０年，腹部，腰背部大小不等的环状，半环状红斑，暗紫红色，环的边缘较宽，浸润明显，上附鳞屑，伴有明显瘙痒，组织病理诊断蕈样肉芽肿，用雷公藤合剂口服及外用氮芥生理盐水治疗有效。     

异维A酸联合放疗治疗9例蕈样肉芽肿

应用异维A酸 0 75mg/(kg·d) ,结合浅层放射治疗 9例蕈样肉芽肿患者 ,其中 6例为斑块期 ,3例为肿瘤期。每例患者照射总剂量为 16GY。治疗 2个月后 8例患者 90 %的皮损痊愈 ,结节损害由平均 11个减为 1个。此联合疗法与单独口服异维A酸或放射治疗相比 ,可缩短疗程、减少剂量 ,且副作用小。     

具有多种表现的蕈样肉芽肿一例

患者,男,80岁。手足反复出现多种皮损伴瘙痒、疼痛4年,加重1年。皮肤活检和组织病理示：表皮内及真皮浅层血管周围灶状淋巴样细胞浸润。免疫组化示：CD3（+）、CD4（+）、CD5（-）、CD7（+）、CD8（+）、CD20（+）,Ki67（10％）。诊断：蕈样肉芽肿。予以抗组胺药物、糖皮质激素药膏、窄谱中波紫外线照射治疗,随访。     

Mycosis fungoides mimicking erysipelas

Mycosis fungoides may rarely simulate facial erysipelas. In a patient with that clinical presentation, a biopsy specimen revealed a diffuse dermal infiltrate with numerous neutrophils that also mimicked erysipelas histopathologically. The diagnosis of mycosis fungoides was made on the basis of atypical lymphocytes with focal epidermo‐ and folliculotropism. It was confirmed by typical findings of mycosis fungoides in a second biopsy from a clinically inconspicuous patch. An identical T‐cell receptor γ‐gene rearrangement was detected in lymphocytes of both biopsy specimens.