Unilesional follicular mycosis fungoides

Follicular mycosis fungoides (FMF) is an unusual clinical and histological variant of MF, characterized by selective involvement of hair follicles by atypical lymphocytes. We describe a female patient who had follicular papules located only on the medial aspect of her right thigh. To the best of our knowledge, this patient represents the first reported case of unilesional FMF.     

Folliculotropic mycosis fungoides with follicular mucinosis--case report

Reports on clinical and histologic follicular alterations in patients previously diagnosed with mycosis fungoides (MF) or at the time of MF diagnosis are rare. The clinical and histologic criteria to distinguish MF associated with follicular mucinosis from follicular MF are a matter of debate. A patient is described with advanced clinical and histologic alterations predominated by follicular lesions and presence of mucin. In the early stage of the disease, folliculotropism was clinically and histologically present but less pronounced than epidermotropism and classic plaque-like lesions. The patient died four years after the diagnosis. As the term 'folliculotropic' describes a particular histopathologic finding, we consider it correct to use the term "folliculotropic MF" to denote atypical lymphocyte folliculotropism in the absence or presence of mild epidermotropism, presence of mucin, or no evidence for intrafollicular mucin. Folliculotropic MF seems to represent a specific clinicopathologic entity which may have a poorer prognosis than classic MF.     

Rapidly progressing mycosis fungoides presenting as follicular mucinosis

Follicular mucinosis can occur as a primary idiopathic disorder or can arise in association with benign or malignant disease, most notably mycosis fungoides. We describe a patient with an aggressive folliculotropic variant of mycosis fungoides that initially presented as follicular mucinosis with alopecia. One month after the diagnosis of follicular mucinosis, a diagnosis of mycosis fungoides was made, and 3 months later inguinal lymph node involvement with mycosis fungoides developed. A skin biopsy specimen demonstrated prominent follicular mucinosis with folliculotropism of atypical cells and intrafollicular Pautrier's microabscesses. As demonstrated in this case, follicular mucinosis can be a presenting sign of rapidly progressive mycosis fungoides. In our review of follicular mucinosis and its association with mycosis fungoides, we found that the folliculotropic variant of mycosis fungoides appears more commonly to have an aggressive course than classic mycosis fungoides.     

嗜毛囊性蕈样肉芽肿的最新分类与治疗进展

嗜毛囊性蕈样肉芽肿(folliculotropic mycosis fungoides, FMF)是蕈样肉芽肿(mycosis fungoides, MF)的一种独特变异型,临床少见,组织学改变以毛囊为中心的非典型淋巴细胞浸润为特征。过去认为FMF预后普遍较经典型MF差,但近年研究发现FMF是一组异质性疾病,根据预后不同至少可以分为两类,一类为早期病变,以斑片、毛囊性丘疹、痤疮样皮疹为主要表现,预后良好;另一类为晚期病变,以结节、肿瘤为主要表现,或者出现皮肤外其他器官受累,预后差。故FMF治疗方式的选择应同经典型MF一样根据分类分期而定。本综述重点阐述了FMF的最新分类,预后因素及其治疗选择。     

伴蕈样肉芽肿的毛囊黏蛋白病一例

患者男,36岁,躯干四肢泛发毛囊性丘疹、斑片及浸润性斑块伴瘙痒15年余,头面部结节性浸润性肿块伴脱发3个月.发病后第13年无明确的与恶性淋巴瘤相关的临床及组织学证据.发病后第15年组织活检见真皮内及毛囊周围单个核细胞团块状浸润或在胶原束间穿插,部分细胞核大深染,有异形性,并见单个核细胞移入毛囊,部分毛囊上皮水肿破坏、甚至形成囊腔结构,其中充满黏蛋白.阿新蓝染色阳性.免疫组化示:CD45RO++,CD20-,CD4++++,CD8-.该患者在诊断为毛囊黏蛋白病15年后发现合并蕈样肉芽肿.     

A case of follicular mycosis fungoides with follicular mucinosis: a rare association

Follicular mycosis fungoides (FMF) is a rare cutaneous T cell lymphoma characterized by an atypical lymphoid infiltrate spreading within and around hair follicles without epidermotropism or follicular mucin deposits. Its occasional presentation with minimal epidermal involvement and/or follicular mucinosis suggests the need for uniform histologic criteria. We describe a new case of FMF associated with follicular mucinosis and discuss its morphologic spectrum of presentation.     

伴毛囊黏蛋白沉积的亲毛囊性蕈样肉芽肿一例

患者,男,37岁。全身丘疹、斑块、剧痒3年。3年前无明显诱因下于躯干、四肢等处出现丘疹、斑块,剧烈瘙痒,挤压后部分皮疹可见脓性分泌物。曾被诊断为＂毛囊炎、皮炎＂,先后口服异维A酸、糖皮质激素、雷公藤等治疗未好转。3年来皮疹逐渐增多。无发热、关节痛等不适,一般情况好。既往有痛风病史。查体：全身浅表淋巴结未触及肿大。系统检查未见明显异常。皮肤科情况：额面部潮红、浸润,以眉毛部位为显著并形成浸润性肿块。     

不伴有毛囊黏蛋白沉积的向毛囊性蕈样肉芽肿1例国内首报

报告1例不伴有毛囊黏蛋白沉积的向毛囊性蕈样肉芽肿.患者女,53岁.全身泛发红色毛囊性丘疹伴瘙痒1年.皮肤科检查见躯干、四肢泛发针头大红色毛囊性丘疹,密集分布,互不融合,外观酷似突起的鸡皮疙瘩,触之粗糙.皮损不累及头面部、手掌和足部.皮损组织病理检查:毛囊周围可见以淋巴细胞为主的细胞浸润,部分细胞侵入毛囊上皮和表皮,细胞深染.核周有空晕,异形不明显.免疫组化染色结果证实浸润的淋巴细胞主要是T淋巴细胞.皮损T细胞受体(TcR)基因重排阳性.诊断:不伴有毛囊黏蛋白沉积的向毛囊性蕈样肉芽肿.     

Basaloid folliculolymphoid hyperplasia: a distinctive finding in follicular mycosis fungoides

Follicular mycosis fungoides is an increasingly recognized subtype of mycosis fungoides. There is great variability in the clinical and histologic presentation of this variant of mycosis fungoides. Epithelial hyperplasia may occur in cutaneous lymphomas, and basaloid induction of the epidermis is a recognized phenomenon in cutaneous neoplasms. We have identified three patients with follicular mycosis fungoides with basaloid folliculolymphoid hyperplasia, a finding that has received little attention in the literature. The patients were all men with prominent follicular papules on the face. We believe that these changes are a distinctive histologic pattern of follicular mycosis fungoides. Hence, we describe the clinical-pathologic findings of basaloid folliculolymphoid hyperplasia in these three patients with follicular mycosis fungoides.     

Hypopigmented mycosis fungoides

INTRODUCTION: Hypopigmented mycosis fungoides is a rare clinical form of the disease, first described in 1978. Since then, only a hundred odd cases have been documented. CASE REPORT: A young 19 year-old African woman had presented with hypochromatic macules since the age of 9 and for which the diagnostic enquiry had finally concluded in hypopigmented mycosis fungoides. DISCUSSION: The particularities of this form of mycosis fungoides, grade I according to the TNM classification, are principally its onset in black-skinned persons or of Asian origin, and the age of early onset with a predominance of pediatric cases. Its course is indolent for several years and thus source of delayed diagnosis. The differential diagnoses to be evoked are basically vitiligo, achromate eczematides and parapsoriasisis. The post-inflammatory depigmentation, frequent in black-skinned subjects, is only an eliminating diagnosis. Its treatment is that of classical Grade I mycosis fungoides: topical mechlorethamine, phototherapy and topical corticosteroids.     

向附属器性蕈样肉芽肿

阐述向附属器性蕈样肉芽肿的临床表现、组织病理、免疫组化染色、诊断、鉴别诊断、治疗和预后。     

Follicular mycosis fungoides

We describe a patient with follicular mycosis fungoides (MF), a rare folliculotropic variant of cutaneous T-cell lymphoma (CTCL). Follicular involvement in CTCL usually presents clinically as alopecia mucinosa associated histologically with follicular mucinosis. Follicular MF differs from alopecia mucinosa/follicular mucinosis associated with MF with regard to its clinical presentation, histology and, presumably, prognosis. Our patient presented with the characteristic findings of follicular MF, i.e. infiltrated plaques showing numerous enlarged, comedo-like follicular infundibula; histology was dominated by exclusive folliculotropism of atypical lymphocytes sometimes forming follicular Pautrier's microabscesses, and by lack of epidermotropism and follicular mucinosis. Despite photochemotherapy and treatment with oral retinoids and interferon alpha, the patient's follicular MF rapidly developed into a progressive CTCL with large tumorous lesions, but responded to electron beam therapy. The course of our patient's disease confirms the notion that follicular MF may be associated with a worse prognosis than classical MF. However, electron beam irradiation induced remission of follicular MF that was maintained by a combination therapy consisting of extracorporeal photopheresis and interferon alfa.     

Folliculotropic mycosis fungoides

Accepted by the WHO and EORTC as a variant of classic mycosis fungoides, folliculotropic (syn.: follicular or pilotropic) mycosis fungoides (FMF) is characterized by a broad clinical and histological spectrum with numerous differential diagnoses. Recent studies have shown that FMF can be divided into two prognostically different subgroups, both marked by histological as well as clinical differences. Treatment should therefore be tailored to the various subtypes and clinical courses. The present review highlights the clinical and histological manifestations of FMF as well as the new subclassification. Moreover, important differential diagnoses and therapeutic options are discussed.     

Subcutaneous mycosis fungoides

In five cases of mycosis fungoides, previously treated with electron-beam therapy, subcutaneous nodules developed. Clinically, these lesions were thought to be epidermoid cysts or lipomas, but on biopsy were discovered to be subcutaneous infiltrates, three of which were diagnosed as mycosis fungoides. The other two specimens showed only a nonspecific subcutaneous infiltrate. There is no ready explanation for the appearance of these lesions, but it is speculated that they may be the result of inadequate penetration of the electron beam to the depth at which some atypical cells may originally have been located. Patients with mycosis fungoides who develop unusual subcutaneous nodules should be fully investigated so that appropriate and adequate therapy may be initiated.     

Ichthyosiform mycosis fungoides

Malignancy-associated acquired ichthyosis is well known, but the ichthyosiform subset of mycosis fungoides (MF) is rarely reported. We report on two patients with a clinical presentation for whom diagnosis of mycosis fungoides was established on histological grounds. In both cases, long term remission was obtained with non aggressive therapies. This rare condition must be added to newly described forms of MF with epidermal hyperplasia such as keratosis lichenoides chronica like MF and pilotropic MF.     

Ichthyosiform mycosis fungoides

BACKGROUND: Mycosis fungoides (MF) is a skin malignancy of T helper lymphocytes with a wide clinical spectrum. Among the atypical variants of MF, there is an ichthyosis-like presentation. However, to date, only 1 case of ichthyosiform MF has been reported. OBJECTIVE: Our goal was to summarize the clinical characteristics and course, and the pathological, immunohistochemical and molecular genetic findings on 4 patients with ichthyosiform MF. METHODS: A retrospective study was conducted. RESULTS: The 4 patients represented 1.8% of the 221 patients with MF seen by us since 1975. None progressed to systemic disease in up to 12 years (median, 10 years) after the onset of the cutaneous manifestations. Interestingly, skin lesions typical of so-called follicular MF (FMF) were associated in 3 of 4 cases, whereas cutaneous manifestations of classic MF were absent in all 4 patients. CONCLUSION: Ichthyosiform MF represents a rare variant within the clinicopathologic spectrum of MF usually featuring a benign course and a tendency to be associated with lesions of FMF but not with lesions of classic MF.     

Hypopigmented mycosis fungoides

We report the case of a 25-year-old Jamaican woman with hypopigmented mycosis fungoides. She first developed a hypopigmented patch on her arm at the age of 11 years. Further lesions developed on the trunk and limbs over a period of 10 years. The lesions were completely impalpable. Skin biopsy showed an infiltrate of atypical lymphocytes, some with cerebriform nuclei, suggesting a diagnosis of mycosis fungoides. The lesions cleared with PUVA therapy.