具有多种表现的蕈样肉芽肿1例

报告1例具有多种表现的蕈样肉芽肿。患者男,48岁。全身皮肤出现红斑、脱屑,并伴进行性皮肤松弛,四肢有斑块、破溃8年,秃发6个月。体格检查发现全身皮肤红斑,上覆大片状鳞屑和结痂;颈部两侧可见表皮松弛;四肢皮肤呈暗红色浸润的松弛性斑块和深在的溃疡;枕部头皮呈条片状胶质样秃发斑;颈项部、双上肢及胸部群集表面光亮的肤色丘疹和结节：左腹股沟可触及数个增大的淋巴结。诊断为蕈样肉芽肿,同时具有蕈样肉芽肿的多种表现：鱼鳞病样蕈样肉芽肿、肉芽肿性皮肤松弛症或肉芽肿性蕈样肉芽肿、毛囊性蕈样肉芽肿。     

儿童肉芽肿性蕈样肉芽肿

报告1例儿童肉芽肿性蕈样肉芽肿.患儿男,14岁.自6岁起头皮开始出现脓疱,并逐渐脱发,形成浸润性红斑,近年来耳前及左胸部亦出现类似红斑.皮损组织病理检查见典型的表皮内Pautrier微脓肿、异形淋巴细胞浸润及真皮内肉芽肿改变.高倍镜下可见异形淋巴细胞.免疫组化染色显示CD3、CD45RO阳性.诊断:肉芽肿性蕈样肉芽肿.推荐外用糖皮质激素,补骨脂素长波紫外线(PUVA),维A酸及α-干扰素治疗.     

肉芽肿性蕈样肉芽肿

患者女,57岁.皮肤出现红斑4年,结节半年.皮损组织病理检查示:表皮轻度萎缩,Pautrier微脓肿形成,真皮大片状单一核细胞和组织细胞为主浸润,呈肉芽肿样改变.免疫组织化学染色显示LCA(+),CD2(+),CD3(+),CD4(+),CD8部分阳性,CD45Ro(+),CD68部分阳性,CD20(-).诊断为肉芽肿性蕈样肉芽肿.     

肉芽肿性蕈样肉芽肿一例

患者男,42岁.因全身皮肤红斑鳞屑10年,结节、肿瘤8年于2004年8月就诊.10年前患者无明显诱因背部出现一鱼鳞病样斑片,无自觉症状.1个月后,胸背部皮肤出现片状淡红斑,微痒,并逐渐扩展至头面部、颈、四肢,呈弥漫性,红斑上有细小糠状鳞屑.在当地医院多次按湿疹治疗无明显好转.     

Solitary plaque on the leg of a child: A report of two cases and a brief review of acral pseudolymphomatous angiokeratoma of children and unilesional mycosis fungoides

Acral pseudolymphomatous angiokeratoma of children (APACHE) and unilesional mycosis fungoides (MF) are two rare dermatoses in the pediatric population which may have overlapping clinical and histopathologic features, making differentiation between these two diagnoses difficult. We present two similar cases of a solitary plaque on the thigh of a child, one representing APACHE and the other representing unilesional MF with granulomatous features, and we provide a brief overview of the clinical and histopathologic features of APACHE and unilesional MF.     

Granulomatous syringotropic mycosis fungoides with two lesions having reactive B‐cell proliferation

Mycosis fungoides (MF) represents the most common type of cutaneous lymphoma. The diagnosis of MF may occasionally be challenging, particularly for variants of MF such as folliculotropic MF, syringotropic MF and granulomatous MF. Herein, we describe a case of MF in which syringotropism and a prominent granulomatous reaction were noted around the affected eccrine gland. This case represents a rare example of MF with multiple unusual histopathological features, including syringotropism, a granulomatous reaction and a reactive B‐cell proliferation.     

肉芽肿性蕈样肉芽肿二例并文献复习

报道2例以局部浸润性斑块为主要表现的肉芽肿性蕈样肉芽肿并复习相关文献.2例患者均表现为红斑、斑块,皮损组织病理检查见真皮内淋巴样细胞及巨细胞浸润,患者1免疫组化示CD2、CD3、CD5、CD7阳性,患者2 CD2、CD3、CD4阳性及CD68组织细胞阳性.诊断:肉芽肿性蕈样肉芽肿.患者1口服阿维A及肿块局部浅层X线照射...     

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。     

结肠癌与蕈样肉芽肿多原发性恶性肿瘤一例

患者男,83岁.1996年6月开始出现间断性稀便、黑便,伴阵发性腹痛,口服镇痛剂、抗生素后仅疼痛稍缓解,遂于1996年6月19日就诊.各系统检查无异常,血、尿、粪常规,肝、肾功能正常.     

伴有狮面的蕈样肉芽肿累及毛囊一例

患者，女，67岁。躯干、四肢斑丘疹4年，面部肥厚红斑2年，伴剧烈瘙痒。皮肤组织病理示棘层及毛囊上皮pautrier微脓肿和真皮单一核细胞带状浸润，异形性明显。免疫组化示CD3、CD4强阳性，Ki-67 20%。TCR基因重排示TCRγ、TCRβ、TCRδ均阳性。诊断：蕈样肉芽肿肿瘤期累及毛囊。给予口服美卓乐24 mg/d，2周后加量至32 mg/d，隔日肌肉注射一次300万IU重组人干扰素α-2a，2周后患者面部肿胀及瘙痒明显消退。     

Follicular mycosis fungoides: presentation of a case and review of the literature

Follicular mycosis fungoides is a rare variant of mycosis fungoides (MF). Structural-wise there are several acneiform lesions made up of comedones, cysts and hyperkeratosis. The main histological finding is atypical lymphocytic infiltration around follicular structures, without epidermotropism. The association with follicular mucinosis is widely discussed in the literature. We report a case of follicular (MF) and review the cases published to date.     

Narrowband UVB phototherapy for early-stage mycosis fungoides: evaluation of clinical and histopathological changes

Background Early‐stage (IA, IB, IIA) mycosis fungoides (MF) has long been treated with various agents including topical potent steroids, nitrogen mustard, carmustine, oral psoralen plus UVA (PUVA), broadband UVB, electron‐beam radiotherapy, interferon‐α and retinoids. However, each of these modalities is associated with various side‐effects. Narrowband UVB (NB‐UVB) therapy has the same effect but is safer to use than the other methods. Objective Our purpose in this prospective study was to determine the effects of NB‐UVB in early‐stage MF both clinically and histopathologically. Materials and methods Twenty‐three patients (20 men, three women, aged 27–78 years) with clinically and histologically confirmed MF were enrolled. Patients received NB‐UVB therapy three times a week. Clinical and histological responses, cumulative doses, total number of treatments, side‐effects and duration of remission period were noted. Results Six patients had stage IA MF, 15 patients stage IB and two patients stage IIA. Eighteen patients had patch stage and five patients had plaque stage histopathologically. All of the patients in the patch group had a complete response (CR). In the plaque group, three patients (60%) had a CR and two (40%) had partial (PR) or no clinical response (NR). The clinical response between patch and plaque groups was statistically significant. Regarding the histopathological findings, 17 (94.4%) had complete clearing and only one (5.6%) patient had a partial improvement in the patch group. In the plaque group, one (20%) patient had complete clearing and four (80%) patients had partial or no improvement. The difference between the two groups was statistically significant. In the patch group, the mean cumulative dose was 90.15 J/cm² and the mean number of treatments was 35.33. In the plaque group, the mean cumulative dose was 90.67 J/cm² and the mean total number of treatments was 39.40. The differences were not statistically significant, either between the mean cumulative dose or the mean number of treatments. The mean duration of follow‐up was 10.87 months (range 1–25 months). Only one of the patients had a relapse. Conclusions NB‐UVB therapy for patients with early‐stage MF is an effective and safe treatment with the effect lasting for months. We suggest that clinical clearance correlates with histological improvement except for patients in the plaque stage.     

A simple clinical scoring system to improve the sensitivity and standardization of the diagnosis of mycosis fungoides type cutaneous T-cell lymphoma: logistic regression of clinical and laboratory data

BACKGROUND: The diagnosis of mycosis fungoides (MF) is notoriously difficult to establish because in the early stages, histological features may be nonspecific or merely suggestive. OBJECTIVES: To standardize the diagnosis of MF. METHODS: We studied 138 patients with suspected MF referred over a 7-year period to a university department of a dermatology-based cutaneous lymphoma clinic. Six diagnostic criteria were evaluated: clinical morphology, clinical distribution, skin biopsy T-cell receptor gene rearrangement (TCR-GR), skin biopsy pan T-cell marker loss > or = 2, skin biopsy CD4/CD8 ratio > or = 6, and skin biopsy diffuse epidermal HLA-DR expression. These six clinical and laboratory criteria were compared by logistic regression analysis in patients with histologically diagnosed MF and those with benign disease. RESULTS: Of the 138 patients, 74 had histology of MF, 47 of benign dermatoses and 17 were indeterminate. Close associations were found between a histological diagnosis of MF and TCR-GR (odds ratio 14.4), classical morphology (7.5), classical distribution (2.5) and diffuse epidermal HLA-DR expression (2.8). Logistic regression models were developed depending on the availability of data (either TCR-GR or HLA-DR). Probabilities for correctly diagnosing MF compared with histology as the 'gold standard' were derived from these logistic regression models. A scoring system assigning point values based on these probabilities was then created in order to assist the clinician in making the diagnosis. If using TCR-GR data, a positive TCR-GR = 2.5 points, the presence of classical morphology = 2.0 points, and the presence of classical distribution = 1.5 points. A total score of > or = 3.5 points assigns a high probability (> 85%) of having MF. If using HLA-DR expression, then the presence of classical morphology = 2.5 points, a positive diffuse epidermal HLA-DR expression = 2.0 points, and the presence of classical distribution = 1.5 points. In this case, a total score of > or = 4.0 points assigns a high probability (> 85%) of MF. CONCLUSIONS: The logistic regression models and scoring systems integrate clinical and laboratory assessments, allow rapid probability estimation, and provide a threshold for the diagnosis of MF in an objective, standardized manner.     

以四肢色素沉着为主要表现的蕈样肉芽肿1例

报告1例以四肢色素沉着为主要表现的蕈样肉芽肿,患者男,56岁,手足及四肢伸侧黑褐色斑4年,境界不清楚,进行性加重,伴轻度瘙痒,组织病理检查见多个深梁的单一核细胞侵入表皮,并形成Pautriet微腺肿,真皮内单一核细胞带状浸润,浸润细胞轻度异形,免疫组化提示为CD4( )T细胞来源的肿瘤,给予糖皮质激素及氮芥外用,皮损略缓解,临床随访6年,病情稳定.     

以泛发性色素沉着为主要临床表现的蕈样肉芽肿1例

报告1例以泛发性色素沉着为主要临床表现的蕈样肉芽肿。患者男,41岁。颈部、躯干、四肢色素沉着12年,进行性加重,无自觉症状。皮肤科检查:颈部、躯干、四肢见浅褐色及深褐色斑片,部分皮损呈条索状,轻度苔藓样变。皮损组织病理学检查:真皮内有以淋巴细胞为主的浸润,部分淋巴细胞异形,少量浸润细胞侵入表皮,形成Pautrier微脓肿。免疫组化染色:LCA及CD45RO均( )。诊断:蕈样肉芽肿。给予阿维A治疗,皮损略缓解。随访期间病情稳定。     

色素减退型蕈样肉芽肿

报告1例以色素减退为主要表现的蕈样肉芽肿.患者男,51岁.四肢起淡白斑5年.皮肤科检查见四肢散在圆形色素减退斑,大小不等,边界清楚,部分色素减退斑中央可见色素沉着.皮损组织病理检查:表皮内有散在淋巴细胞,可见Pautrier微脓肿,真皮乳头层有淋巴细胞浸润.免疫组化染色结果示:CD45RO( )、CD8( )、CD117( )、CD45RA(-)、CD20(-).诊断:蕈样肉芽肿.     

以鱼鳞病样皮损为首发表现的蕈样肉芽肿

报告1例以鱼鳞病样皮损为首发表现的蕈样肉芽肿。患者女,28岁。全身泛发灰褐色色素沉着,四肢伸侧可见大小不等的深褐色菱形或多角形鳞状斑片,表现轻度萎缩,伴糖皮样脱屑。皮损组织病理学改变符合蕈样肉芽肿。