肉芽肿性蕈样肉芽肿

报告1例肉芽肿性蕈样肉芽肿。患者女,49岁。因躯干及四肢红斑、斑块1.5年,间断发热半个月就诊。皮损组织病理:表皮萎缩变薄,真皮内片状密集分布淋巴细胞、组织细胞及多核巨细胞,部分淋巴细胞核异形,真皮内可见肉芽肿病变。免疫组化:淋巴细胞CD3、CD4、CD5阳性,CD8散在阳性,组织细胞CD68阳性,T细胞样受体(TCR)基因克隆重排:TCRγ重排+。诊断:肉芽肿性蕈样肉芽肿。     

头皮肉芽肿性蕈样肉芽肿

报告1例头皮肉芽肿性蕈样肉芽肿。患者女,76岁。头皮红斑和溃疡2年,背部红斑3年就诊。皮肤科检查：头皮大片状红色斑块,边缘略隆起,皮损内可见粗大分枝状血管、糜烂、结痂及毛发脱落,红斑约占头皮面积80%。左侧腰、背部一浸润性红色斑块,上覆少许干燥鳞屑。头皮皮损组织病理检查：表皮萎缩变薄,可见淋巴细胞浸润及Pautrier微脓肿形成;真皮内弥漫性淋巴细胞、组织细胞及多核巨细胞浸润,可见肉芽肿形成;部分淋巴细胞有异形性。免疫组化：淋巴细胞CD3、CD4、CD5及CD7均阳性,CD8散在阳性,组织细胞CD68阳性,增殖核抗原（Ki-67）约20%阳性。T细胞受体（TCR）基因重排：TCRβ及TCRγ重排阳性。诊断：肉芽肿性蕈样肉芽肿。     

肉芽肿性蕈样肉芽肿

报告1例肉芽肿性蕈样肉芽肿。患者男,41岁。因全身起红斑、丘疹、结节6年,左侧眉毛脱落2个月就诊。皮损组织病理检查:表皮大致正常,真皮内可见大量淋巴细胞、组织细胞、多核巨细胞浸润,部分淋巴细胞核有异形。免疫组化染色示:淋巴细胞CD3、CD5、CD4阳性,组织细胞及多核巨细胞CD68阳性。结合临床和组织病理表现,诊断为肉芽肿性蕈样肉芽肿。     

间质性蕈样肉芽肿

报告1例间质性蕈样肉芽肿。患者女,60岁。躯干、四肢红斑5年就诊。皮肤科检查:左肩部、左胸部和上背部可见4处环形淡红斑,边缘略隆起,轻度浸润,中央皮肤萎缩,边界欠清。皮损组织病理学检查:真皮内血管周围及胶原纤维束间见弥漫性单一淋巴样细胞浸润,肿瘤细胞核深染,呈异形改变;部分细胞移入表皮。免疫组化检查:肿瘤细胞CD45RO、CD3、CD4阳性,CD20和CD79a阴性。基因重排:T细胞抗原受体(TCR)-γ单克隆性重排。诊断:间质性蕈样肉芽肿。     

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.     

大疱性蕈样肉芽肿

报告1例大疱性蕈样肉芽肿.患者男,40岁.因躯干、四肢红斑伴瘙痒6年,出现斑块半年、水疱1个月就诊.皮肤科检查:面部、躯干及四肢泛发红斑和斑块,部分斑块浸润明显.右下腹斑块上可见0.5 cm×2 ca的水疱.皮损组织病理检查:真皮浅层明显水肿,部分区域可见表皮下疱形成,真皮浅层可见异形明显的单一核细胞呈带状浸润.部分单一核细胞侵入表皮,形成Pautrier微脓肿.免疫组化染色结果示LCA( )、CD4( )、CD45RO( ).结合临床、免疫组化和组织病理检查结果,诊断为大疱性蕈样肉芽肿.     

不伴粘蛋白沉积的嗜毛囊性蕈样肉芽肿1例

报告不伴粘蛋白样沉积的嗜毛囊性蕈样肉芽肿1例。患者男,39岁。全身浅表淋巴结肿大10年,全身红斑、毛发脱落2年。组织病理示:淋巴细胞侵入表皮内,真皮毛囊减少,血管毛囊周围淋巴细胞浸润,部分淋巴细胞侵入毛囊内,部分细胞轻度异型。免疫组化示CD45RO、CD3、CD4和CD5阳性,CD20和CD30阴性。阿新蓝染色:毛囊内无粘蛋白沉积。皮损T细胞受体(TCR)重排阳性。结合临床、组织病理及免疫组化检查诊断为不伴粘蛋白沉积的嗜毛囊性蕈样肉芽肿。     

伴毛囊黏蛋白沉积的向毛囊性蕈样肉芽肿

报告2例伴有毛囊黏蛋白沉积的向毛囊性蕈样肉芽肿.例1.女,39岁.面部、躯干、四肢出现红斑、丘疹,伴瘙痒3年余就诊.例2.男,52岁.躯干、四肢出现红斑、斑块1年就诊;结合组织病理检查、免疫组化染色及淋巴细胞克隆性基因重排检测.2例患者均诊断为伴有毛囊黏蛋白沉积的向毛囊性蕈样肉芽肿.     

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。     

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...     

Granulomatous mycosis fungoides: report of a case with some histopathologic features of granulomatous slack skin

We describe a case of granulomatous mycosis fungoides, tumor stage, mimicking sarcoidosis in an 82-year-old man with a 2-year history of skin disease. The final diagnosis was established after one of seven biopsy specimens showed a nongranulomatous histologic picture of patch-stage mycosis fungoides. Monoclonality was proven for the lymphocytic population by T-cell-receptor rearrangement studies. The unusually extensive granulomatous inflammation with huge giant cells surrounded by CD1a-positive cells in the other six biopsy specimens was suggestive of the histopathology of granulomatous slack skin, another rare granulomatous cutaneous T-cell lymphoma. Because both a clinical and histologic overlap between granulomatous mycosis fungoides and granulomatous slack skin have been reported in the literature, we conclude that they may belong to the spectrum of a single disease.     

伴大细胞转化的蕈样肉芽肿一例

蕈样肉芽肿病情进展缓慢,但发生大细胞转化则提示病情恶化,预后较差。蕈样肉芽肿发生大细胞少见,容易误诊。我们报道一例伴大细胞转化的蕈样肉芽肿。患者男,40岁,因躯干四肢红斑、丘疹伴瘙痒10年,颈后结节5月就诊。患者红斑性皮损显示典型蕈样肉芽肿改变,肿瘤细胞体积较小,表达CD3、CD4,少数细胞表达CD30;而颈后结节性皮损显示有较多的大细胞浸润,可见大细胞亲表皮现象,大细胞阳性表达CD3和CD4,约40%的大细胞表达CD30。结合患者临床病史和组织学改变诊断为蕈样肉芽肿的大细胞转化,给予患者阿维A口服（30 mg/d）, 干扰素皮下注射（1周3次,每次2 × 106 IU）,浅层X线局部照射等治疗,3周后患者皮损好转,目前在随访中。     

CD8‐positive granulomatous mycosis fungoides: a case report with review of the literature

Granulomatous mycosis fungoides (GMF) represents an uncommon variant of mycosis fungoides (MF) characterized by the presence of an associated granulomatous reaction. Most cases of GMF are CD4 positive, and CD8 positive cases are extremely rare. Herein, we report a case of CD8‐positive GMF. A 75‐year‐old Japanese woman presented with brownish maculae on the trunk and upper and lower extremities. She had been diagnosed with MF, and most of the eruption improved by psoralen ultraviolet A therapy. However, the eruption relapsed and gradually expanded 5 months prior to her visit to our hospital. Histopathology showed an atypical lymphocytic infiltrate in the dermis accompanied by granulomatous reaction with multinucleated giant cells. Epidermotropism was evident and elastophagocytosis was also found. Immunohistochemically, the atypical lymphocytes expressed βF1, CD3 and CD8, and some of the atypical lymphocytes were also T cell intracellular antigen‐1 positive. These findings were consistent with CD8‐positive GMF. The dermatopathological diagnosis of GMF is challenging in some cases because of the prominent secondary granulomatous reaction. Therefore, when dermatopathologists diagnose granulomatous skin lesions, GMF should also be considered. In addition, the prognosis of GMF, especially CD8‐positive GMF, is still controversial. Additional studies are required to clarify the clinicopathological features of CD8‐positive GMF. Ishida M, Hotta M, Takikita‐Suzuki M, Kojima F, Okabe H. CD8‐positive granulomatous mycosis fungoides: a case report with review of the literature.     

儿童色素减退型蕈样肉芽肿一例并文献复习

报告儿童色素减退型蕈样肉芽肿一例并对相关文献进行复习。患儿,男,7岁。周身皮肤出现色素减退斑、红斑结痂1年,无明显瘙痒。组织病理检查：角层轻度角化,表皮轻度增生,可见淋巴样细胞亲表皮现象,真皮内可见团块状样细胞浸润。免疫组化示：CD3(+);CD20(个别+);CD21（-）;Ki-67（+10%）;CD2（+）;CD5（+）;CD7（+）;CD8（少许+）;CD4（+）。诊断：色素减退型蕈样肉芽肿。给予患儿NB-UVB治疗3个月皮损基本消退,目前维持治疗中。     

Granulomatöse Mycosis fungoides

A 74-year-old male with granulomatous mycosis fungoides presented with multiple, red-brown macules and plaques up to 8 cm in diameter, just as in classical mycosis fungoides. Dermatohistopathologic findings showed extensive granulomatous infiltrates, in which clonality could be detected in various locations via T cell receptor rearrangement. Granulomatous mycosis fungoides is a very rare form of mycosis fungoides with histological resemblance to granulomatous slack skin. It shows a rather aggressive course and can be challenging to diagnose. In our case, combination treatment with bexarotene and bath PUVA, as recommended in guidelines, resulted in an impressive improvement of the skin lesions within ten weeks.     

Granulomatous mycosis fungoides. Clinicopathologic study of two cases.

Granulomatous mycosis fungoides is a rare form of mycosis fungoides with controversial histogenesis. Early reports seemed to indicate a favorable prognosis for these patients. We report two cases of granulomatous mycosis fungoides, both of which had other unusual clinical features. The cases were studied with routine light microscopy, immunohistochemistry, electron microscopy, and gene probe studies. Despite some clinical and histopathologic similarities, the results of the immunohistochemical and molecular biologic studies were diverse. These results suggest that granulomatous mycosis fungoides does not define a single subset of cases, immunophenotypically or biologically.     

Granulomatous mycosis fungoides with small intestinal involvement and a fatal outcome

We report a case of granulomatous mycosis fungoides that progressed into fatal gastrointestinal involvement 4 years after the onset of skin lesions, despite improvement of the skin lesions in response to a combination of PUVA and systemic interferon-gamma therapy. Histological examination showed Pautrier's microabscesses with granuloma annulare-like features and sarcoidal granuloma formation in the plaque stage, proliferation of blast-transformed atypical lymphocytes with persistent granuloma formation in the tumour stage, and metastatic lesions. A literature review of granulomatous mycosis fungoides revealed that 11 of the 24 reported cases died of the disease, and like our case, seven died within 5 years. We suggest that mycosis fungoides with granulomatous reactions does not indicate a favourable prognosis.     

Mycosis fungoides with focal CD 30 transformation in an adolescent

Mycosis fungoides is rare in children and adolescents. Large cell transformation in mycosis fungoides is typically seen in adult patients with advanced disease. We describe a 16-year-old girl with patch/plaque stage mycosis fungoides who developed a nodule within one of the plaques, which on histopathology showed large cell transformation, with positive labeling with the CD30 immunostain. To the best of our knowledge, this is the second reported case of mycosis fungoides with CD30+ large cell transformation in a child.     

Granulomatous mycosis fungoides responsive to gemcitabine

We report a 61-year-old woman with a 1-year-history of widespread erythematous scaly patches and plaques as well as red/purplish to brownish confluent plaques. Ulcerated lesions with a purulent, hemorrhagic exudate and sharp elevated borders were located on the lower extremities. Diagnosis of granulomatous mycosis fungoides was supported by histopathologic findings showing an inflammatory reaction with epithelioid and large giant cells associated with features characteristic of mycosis fungoides. Immunohistochemical studies showed a T-helper phenotype of neoplastic cells (CD3+, CD4+, CD45RO+) with expression of the cytotoxic protein TIA-1. Molecular analysis of TCRgamma gene demonstrated a monoclonal rearrangement in the lesional skin. After failure of conventional therapies, 6 cycles of gemcitabine treatment produced partial remission of cutaneous lesions and stable disease throughout a 12-month follow-up period, suggesting that gemcitabine is a promising chemotherapeutic agent for refractory mycosis fungoides.