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。     

成人色素减退性蕈样肉芽肿1例并文献复习

患者男性,25岁,周身色素脱失斑15个月。专科查体：全身泛发大小不一色素脱失斑,部分皮疹沿皮纹分布,上覆糠秕状鳞屑,境界欠清。皮肤病理：表皮灶性角化不全,真皮浅层血管周围及胶原间可见淋巴细胞为主的浸润,淋巴细胞周边空晕,可见亲表皮现象。免疫组化：CD3、CD4、CD8(+),CD20散在（+）,CD30（-）。诊断：色素减退性蕈样肉芽肿（HMF）。     

儿童色素减退型蕈样肉芽肿1例

 报告色素减退型蕈样肉芽肿1例。患儿男,4岁。全身散发绿豆大小色素减退斑半年余,加重1个月。皮肤科检查：颈部、胸、背及四肢可见多发类圆形或圆形色素减退斑,境界清楚,未见融合,部分白斑中央见暗红色丘疹及斑片,表面干燥,覆盖白色鳞屑。左手背有两颗高于皮面黑色斑丘疹。皮损组织病理：表皮灶性角化不全,基底细胞液化变性,淋巴样细胞进入表皮内,少许核扭曲,见空晕,真皮浅层较多淋巴组织细胞苔藓样浸润,少许细胞轻异性。免疫组化提示CD20散在少量细胞（+）、CD79a散在少量细胞（+）、CD3（+）、CD4少量细胞（+）、CD5（+）、CD7大部分细胞（+）、CD8（+）、CD4与CD8比例为1 ∶ 4;CD30约30%细胞（+）、CD68散在少量细胞（+）、TIA 1（+）、Ki 67约30%细胞（+）;CD7表达减弱。EBER原位杂交：（-）。根据临床表现、组织病理和免疫荧光,诊断为色素减退型蕈样肉芽肿。予盐酸氮芥溶液治疗,后失访。     

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.     

婴儿弥漫性皮肤肥大细胞增生症

报告1例弥漫性皮肤肥大细胞增生症。患儿女,10个月。躯干水疱及大疱5个月。皮肤科检查：全身皮肤呈橘黄色、纹理粗糙、颜色较暗,背部见水疱簇集性分布,尼氏征阴性。背部散在分布色素减退斑,色素减退区域摩擦后可见皮肤发红。额部、右侧颞部可见风团。背部皮损组织病理检查：表皮内陈旧性水疱,真皮内肥大细胞弥漫性浸润,肥大细胞呈圆形或梭形,细胞质丰富,呈嗜碱性。免疫组化：CD117(+),CD2及CD25为（-）。诊断：弥漫性皮肤肥大细胞增生症。     

儿童色素减退型蕈样肉芽肿1例

患儿女,5岁2个月,皮肤色素减退斑3年。皮肤色素减退斑进行性增多,边界不清。皮肤科情况:全身泛发大小不一的色素减退斑,界限不清,累及躯干面积约70%~80%。皮损组织病理示:真皮浅层及表皮内较多单一的淋巴细胞浸润,核稍深染,有空晕,表皮内形成Pautrier微脓肿,部分淋巴细胞沿基底层排列。免疫组织化学示:CD3(2+)、CD8(3+)、Ki-67(5%+)。诊断:色素减退型蕈样肉芽肿。窄谱中波紫外线治疗3个月后大部分色素减退斑恢复正常肤色。     

色素减退性蕈样肉芽肿1例

患者男,39岁.躯干出现浅白色斑疹5年.查体见躯干和四肢近端有大小不等的色素减退斑.皮损组织病理示表皮及真皮上层淋巴细胞浸润,并具异型性;免疫组化CD4(+),CD7(-),CD8(2+),CD45RO(3+).结合临床与组织病理诊断为色素减退性蕈样肉芽肿.     

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。     

丘疹性蕈样肉芽肿一例

患者男,27岁,因左胸部、左腋窝丘疹3年就诊,无自觉症状,皮损持续不退。组织病理检查示棘层轻度增生肥厚,皮突延长,真皮浅层苔藓样淋巴样细胞浸润,部分移入表皮,形成Pautrier微脓肿。免疫组化示LCA（+）、CD45RO（+）、CD3（+）、CD4（+）、CD8散在（+）、CD20（-）、CD68（-）,CD30（-）。皮肤激光共聚焦显微镜显示棘层中散在有轻度折光的椭圆或圆形细胞影,直径4 ~ 8 μm。诊断：丘疹性蕈样肉芽肿。治疗：外用丙酸氟替卡松乳膏、异维A酸凝胶,窄波紫外线照射,丘疹变软,色泽变淡。     

色素减退型蕈样肉芽肿1例

患者女,45岁,2年前无明显原因躯干出现色素减退斑,无自觉症状,2年内色素减退斑逐渐增多且累及颈部、四肢。皮肤科情况:全身泛发形状不规则、大小不一的色素减退性浸润性斑片,边界较模糊。皮损组织病理示:表皮内可见多个核大、深染、周围有空晕的异型淋巴样细胞,亲表皮现象比较明显。免疫组织化学提示表皮内CD8⁺T淋巴细胞浸润。诊断:色素减退型蕈样肉芽肿。     

Inflammatory vitiligo-like macules that simulate hypopigmented mycosis fungoides

Two cases of an inflammatory vitiligo-like condition that simulated mycosis fungoides are reported. Both patients presented acquired hypopigmented macules sharply limited by an erythematous and papular border. The clinical aspect was suggestive of inflammatory vitiligo. Mycosis fungoides was suspected on skin specimens showing a dense band-like lymphocytic infiltrate with discrete nuclear atypias and marked exocytosis. This infiltrate was made of CD3 positive lymphocytes. CD8 positive lymphocytes were numerous in one case, few in the other. There was a loss of melanocytes in the lesional skin and absence of dominant T-cell clones in both cases. No repigmentation was observed after PUVA or local chemotherapy. The authors emphasized that erythematous and papular borders surrounding hypopigmented macules, CD8 positive lymphocytic infiltrate, absence of T-cell clonal rearrangement are helpful to rule out mycosis fungoides.     

Leucoderma associated with flares of erythrodermic cutaneous T-cell lymphomas: four cases. The French Study Group of Cutaneous Lymphomas

We describe four patients with erythrodermic cutaneous T-cell lymphomas (two with erythrodermic mycosis fungoides, and two with Sézary syndrome) who presented with extensive hypopigmented lesions that occurred during flares of their cutaneous disease. These cases must be distinguished from previously described hypopigmented mycosis fungoides where hypopigmented lesions were the sole manifestation of the lymphoma. In two cases a biopsy was performed on hypopigmented skin, showing an infiltrate of atypical lymphocytes with epidermotropism and absence of melanocytes, as in vitiligo. It is suggested that the hypopigmentation could be due to the cytotoxicity of tumour or reactional lymphocytes directed against melanocytes.     

Vitiligo vs. hypopigmented mycosis fungoides (histopathological and immunohistochemical study, univariate analysis)

Vitiligo is a common skin disease characterized by the presence of well circumscribed, depigmented milky white macules devoid of identifiable melanocytes. On the other hand, hypopigmented mycosis fungoides (MF) is a rare variant of MF which presents clinically as persistent hypopigmented macules and patches. Both disorders show a predominance of CD8+ T cells in tissue samples and hence the differentiation between the two diseases on clinical, histopathological and even immunohistochemical grounds may offer great difficulty. The aim of this work is to identity certain histopathological clues which might help to differentiate between the two diseases. The study included 54 patients (26 vitiligo patients and 28 patients with Hypopigmented MF). Skin biopsies were taken and examined by hematoxylin and eosin and CD3, CD4 and CD8 markers were performed for ten vitiligo and nine MF patients. We have found that epidermotropism, hydropic degeneration of basal cells, partial loss of pigment, preservation of some melanocytes, presence of lymphocytes within the papillary dermis, increased density of the dermal infiltrate and wiry fibrosis of the papillary dermal collagen were detected with a significantly higher incidence in hypopigmented MF rather than vitiligo (P-values < 0.0001, < 0.00011, < 0.00011, = 0.001, = 0.008 and = 0.001 respectively). On the other hand, focal thickening of the basement membrane, complete loss of pigmentation, total absence of melanocytes, as well as absence or sparsness of lymphocytes in the dermal papillae were seen much more frequently in vitiligo. Statistical analysis of these differences was significant with P-values < 0.00011, < 0.00011, < 0.00011, = 0.008 respectively, regarding these pathological criteria. We conclude that differentiation of hypopigmented MF from vitiligo is possible by relying on the histopathological clues described in this study. This is particularly useful in areas of the world where cost benefit is crucial.     

皮肤色素减退性淋巴增殖性疾病41例临床病理分析

目的:探讨色素减退性蕈样肉芽肿（HMF）与色素减退性界面T细胞异常增生（HITCD）的临床与病理特征。方法:收集2015年1月至2020年9月在杭州市第三人民医院皮肤科就诊、具有完整临床病理资料的皮肤色素减退性淋巴增殖性疾病患者41例,分析临床病理及免疫表型特征。符合正态分布的定量数据使用 t检验,分类数据...     

Hypopigmented mycosis fungoides: a review of its clinical features and pathophysiology

Several distinct clinical forms of mycosis fungoides have been described. Hypopigmented mycosis fungoides should be regarded as a subtype of mycosis fungoides, insofar as it presents some peculiar characteristics that contrast with the clinical features of the classical form. Most patients with hypopigmented mycosis fungoides are younger than patients typically diagnosed with classical mycosis fungoides. In addition to typical dark-skinned individuals impairment, hypopigmented mycosis fungoides has also been described in Asian patients. The prognosis for hypopigmented mycosis fungoides is much better than for classical mycosis fungoides: hypopigmented mycosis fungoides is diagnosed when there are only patches of affected skin, and lesions usually will not progress beyond terminal stages, although they can persist for many years. Diagnosis should involve clinicopathologic correlation: skin biopsy analysis often reveals intense epidermotropism, characterized by haloed, large, and atypical CD8+ lymphocytes with convoluted nuclei, in contrast to mild to moderate dermal lymphocytic infiltrate. These CD8+ cells, which participate in T helper 1-mediated immune responses, prevent evolution to mycosis fungoides plaques and tumors and could be considered the main cause of the inhibition of melanogenesis. Therefore, hypopigmentation could be considered a marker of good prognosis for mycosis fungoides.     

Hypopigmented interface T‐cell dyscrasia: A form of cutaneous T‐cell dyscrasia distinct from hypopigmented mycosis fungoides

Hypopigmentation in cutaneous T‐cell lymphoproliferative disease should not always be equated with hypopigmented mycosis fungoides (MF). A form of hypopigmented pre‐lymphomatous T‐cell dyscrasia falling under the designation of the so‐called hypopigmented interface variant of T‐cell dyscrasia has recently been proposed. The aim of the present study was to establish hypopigmented interface T‐cell dyscrasia as its own entity apart from other T‐cell dyscrasias and MF using a patient case series. Twenty four cases of hypopigmented interface T‐cell dyscrasia were identified in the dermatopathology database of Weill Medical College of Cornell University. There were 17 females and seven males (mean age, 36 years). In children and adolescents, the patients were most commonly of African American extraction. Truncal photo‐protected areas manifesting as large solitary patches or multiple smaller macules were characteristic; disease progression to MF occurred in only one patient. The lesions responded to topical steroids and light therapy. The pathology was defined by a cell poor interface associated with degeneration of keratinocytes and melanocytes, and by lymphocytes whose nuclei showed low‐grade cerebriform atypia, and which expressed a significant reduction in CD7 and CD62L expression. In 50% of the cases, the implicated cell type was of the CD8 subset. Clonality was not identified. Hypopigmented interface T‐cell dyscrasia is a distinct entity separate from and rarely progressive to MF.     

Pathogenetic mechanisms of vitiligo in a patient with Sézary syndrome

Patients exhibiting association between vitiligo and cutaneous T-cell lymphoma (CTCL) remain rare and it is not known whether some T-cell subpopulations of CTCL in the skin are able to recognize specific melanocytic epitopes and thus induce vitiligo. The aim of our study was to determine whether T cells specific to melanocyte differentiation antigens were detectable among tumour-infiltrating lymphocytes (TIL) in the hypopigmented skin of a patient with Sézary syndrome (SS). A 71-year-old patient presented with SS and developed vitiligo during the course of her disease. Immunohistochemical studies showed staining with HMB45 and MelanA antibodies in the pigmented skin biopsy, whereas no staining was observed in the hypopigmented skin biopsy. To analyse responses to melanocyte differentiation antigens, we used a transient COS transfection assay that permits an estimation of CD8 T-cell responses against a large number of HLA/antigen combinations. This technique allowed the detection of melanocyte differentiation antigen-specific T lymphocytes, directed mainly against Melan-A/MART1 antigen in the HLA-A*23 context. Our study supports the concept that vitiligo that has developed during the evolution of a CTCL is related to the presence of a T-lymphocyte subpopulation reactive against melanocyte differentiation antigens (mainly Melan-A/MART1) present in skin lesions. The role of interferon in the induction of this T-lymphocyte subpopulation is discussed.