大疱性蕈样肉芽肿和色素异常性蕈样肉芽肿

阐述大疱性蕈样肉芽肿和色素异常性蕈样肉芽肿的临床表现、组织病理变化和鉴别诊断。     

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一例

肉芽肿性蕈样肉芽肿(granulomatous mycosis fungoides,GMF)是蕈样肉芽肿(MF)的一种特殊的病理类型,其肉芽肿形成的病因学、发病机制及临床意义尚不清楚．预后也有争议。该病临床罕见．现遇一例报道如下。     

肉芽肿性蕈样肉芽肿

1病例资料 例1,男,72岁,因躯干和四肢出现淡红色或淡灰白色斑片3年,于1994年8月10日就诊就于我科。1991年初患者躯干和四肢陆续出现淡红色或淡灰白色斑片,表面有少量鳞屑,不痒。既往体健,无外伤和传染病史。     

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

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。     

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。     

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1例

1 病历摘要 患者男,69岁.因全身皮肤出现红斑、斑块、破溃及愈合后色素沉着16年,于2011年6月来我所就诊.16年前无明显原因患者双下肢出现暗红斑,同时膝关节内侧出现暗红色结节、溃疡,约3个月余自行愈合,留有色素沉着,后皮损缓慢蔓延至全身,无自觉症状.曾诊断"肉样瘤",未进行系统治疗.近1年来左额部及右肩背部出现暗红斑块,并破溃结痂,未愈合,自觉疼痛.     

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 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.     

Granulomatous mycosis fungoides presenting as poikiloderma

Granulomatous mycosis fungoides (MF) is a rare subtype of MF, characterized by the histological presence of a granulomatous reaction, but distinct clinical characteristics are not present. A 41-year-old healthy man presented with poikiloderma, ichthyosis and erythematous scaly plaque. Histological examination of a biopsy taken from poikilodermic skin showed a granulomatous reaction to epidermotropic atypical lymphocytes. However, in other areas there were only findings of conventional MF without granuloma. Granulomatous MF may be associated with poikiloderma.     

Granulomatous mycosis fungoides - A diagnostic challenge

Granulomatous mycosis fungoides is a rare subtype of T-cell cutaneous lymphoma. Due to its clinical heterogenicity the diagnosis is delayed and based on histopathological and immuno-histochemical findings, sometimes requiring gene rearrangement studies for confirmation. We report the case of a patient who was submitted to several biopsies before diagnostic conclusion.     

Granulomatous mycosis fungoides with hypohidrosis mimicking lepromatous leprosy

Granulomatous mycosis fungoides (GMF) is a rare type of cutaneous T cell lymphoma. A 38-year-old married male presented with decreased sweating all over the body for last 8 years, progressive redness and scaling over body for 2 years and multiple noduloulcerative lesions over the body for 1 year. Cutaneous examination revealed generalized erythema and scaling with poikilodermatous changes over chest and upper back along with multiple noduloulcerative lesions. Skin biopsy from a nodular lesion revealed dense granulomatous infiltrate of atypical lymphocytes with epidermotropism and sparing of appendages. Diagnosis of GMF was made. Computed tomographic scan of thorax, abdomen and pelvis revealed axillary and inguinal lymphadenopathy. Immunohistochemistry revealed leukocyte common antigen and CD3 positivity suggestive of T cell origin. Patient was started on CHOP (Cyclophosphamide, Hydroxydaunorubicin, Oncovin and Prednisolone) regimen of chemotherapy with marked improvement after three cycles of chemotherapy. This case had some clinical resemblance to lepromatous leprosy.     

Granulomatous mycosis fungoides with extensive chest wall involvement

A 40-year-old woman presented with a 5-year history of a mass overlying her right pectoralis major muscle. Histopathology of the lesion revealed a florid granulomatous infiltrate including an atypical lymphocytic component with marked epidermotropism consistent with granulomatous mycosis fungoides. Staging investigations demonstrated the tumour to be localized to the right chest. Consequently, the patient was treated with radiotherapy (50 Gy) to the lesion with good clinical effect. However, she soon developed a clinically palpable lesion on the left chest outside the radiotherapy field. Positron emission tomography scanning demonstrated an extensive left-sided chest wall tumour and also residual tumour on the right. This left-sided lesion failed to respond to systemic chemotherapy. Further radiotherapy (50 Gy) has recently been administered to the left chest lesion; the response is being monitored. While granulomatous inflammation has been previously described in cutaneous T-cell lymphomas, it is rare and is often associated with a delay in the diagnosis and difficulty with clinical staging. The clinical presentation can be extremely variable and consequently, diagnosis rests with histological features, immunohistochemical studies and gene rearrangement analysis.     

Granulomatous mycosis fungoides presenting as an acquired ichthyosis

We report a case of a 69-year-old gentleman who presented with a 3-month history of unexplained fevers and malaise who developed generalized pruritus, alopecia and an ichthyosiform erythematous eruption on his forearms, legs, chest and back. Skin histology, immunophenotyping and molecular features were consistent with granulomatous mycosis fungoides. He has been successfully treated with twice weekly PUVA photochemotherapy.     

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.