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.     

伴毛囊黏蛋白沉积的亲毛囊性蕈样肉芽肿

报告1例伴毛囊黏蛋白沉积的亲毛囊性蕈样肉芽肿。患者男,43岁。右侧眉弓、上眼睑斑块、丘疹6个月,眉毛脱落4个月。皮损组织病理:真皮内及毛囊、附属器周围见大量淋巴样细胞团块状浸润,部分细胞异形,部分毛囊上皮可见不典型淋巴样细胞移入,毛囊内充满黏蛋白;阿辛蓝染色阳性。免疫组化显示浸润细胞主要是CD4~+T细胞。T细胞受体基因重排结果为阴性。诊断:伴毛囊黏蛋白沉积的亲毛囊性蕈样肉芽肿。     

毛囊黏蛋白沉积症与蕈样肉芽肿T细胞受体基因重排的对比研究

目的 研究毛囊黏蛋白沉积症与蕈样肉芽肿的关系.方法 从8例特发性毛囊黏蛋白沉积症、3例毛囊黏蛋白沉积症合并蕈样肉芽肿和1例蕈样肉芽肿患者获得病变组织,将其埋于石蜡中.设计T淋巴细胞受体(TCR)可变区引物Vγ1-8/A,Vγ10,Vγ11及β,利用PCR方法分析了12例患者组织中T淋巴细胞受体基因重排的情况.结果 8例特发性毛囊黏蛋白沉积症中1例,3例毛囊黏蛋白沉积症合并蕈样肉芽肿和1例蕈样肉芽肿患者皮损TCR Vγ1-8/A呈单克隆性.结论 对于年龄较大、病程较长的毛囊黏蛋白沉积症患者,应行TCRγ基因重排检查,以排除淋巴瘤.     

亲毛囊性蕈样肉芽肿四例分析

报道4例亲毛囊性蕈样肉芽肿。男3例,女1例,年龄32 ~ 52岁。3例以头面部及颈部受累为主,密集多发性暗红色不规则浸润性斑块、结节、肿块、毛囊性丘疹及痤疮样皮损,躯干、四肢表现为斑片、轻度浸润的斑块、毛囊性丘疹及痤疮样皮损。1例头面部未受累,全身密集毛囊性丘疹。组织病理显示真皮内、血管周围及毛囊周围大量淋巴样细胞为主的团块状浸润,部分细胞异形,浸润细胞移入毛囊上皮是他们共同而显著的特征,而亲表皮现象不明显。其中2例毛囊内充满黏蛋白,阿新蓝染色阳性。免疫组化显示浸润细胞主要为CD4阳性T细胞。对常规用于经典蕈样肉芽肿的治疗反应差,病情难以完全缓解,即使部分缓解后也极易复发。     

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。     

021 伴有毛囊粘蛋白沉积的毛囊性蕈样肉芽肿一例

毛囊性蕈样肉芽肿 (ＦＭＦ)是一种少见的皮肤型Ｔ细胞淋巴瘤 ,以毛囊内及其周围有异形性淋巴细胞浸润为特征 ,一般无表皮受累或毛囊粘蛋白沉积。有文献报道毛囊之间表皮不受累以及无毛囊粘蛋白沉积是ＦＭＦ与伴有毛囊粘蛋白沉积的ＭＦ的主要区别点。该文报道了 1例伴有毛囊粘蛋白沉积的ＦＭＦ。5 0岁男性 ,手足发生瘙痒性、多发性、毛囊性丘疹及黑头粉刺样皮损 6年 ,后逐渐扩展至整个上、下肢。曾系统性口服米诺环素、13 顺维Ａ酸及外用 3%红霉素凝胶 ,效果欠佳。皮肤科情况 :四肢可见点状、毛囊角化性丘疹 ,部分融合成轻微隆起的斑块 ,其间…     

Disseminated papular granuloma annulare

报告1例播散性丘疹型环状肉芽肿.患者男,61岁.因躯干、四肢起大量粟粒大、无症状的丘疹3年余就诊.组织病理检查显示真皮浅层栅栏状肉芽肿形成,中央有局灶性胶原纤维变性,周围可见组织细胞和淋巴细胞浸润.阿新蓝染色示肉芽肿的中央有阳性物质沉积.该病需与毛囊性扁平苔藓、丘疹型结节病、发疹性汗管瘤、光泽苔藓和毛囊性蕈样肉芽肿等病鉴别.     

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.     

毛囊黏蛋白病

报告1例毛囊黏蛋白病。患者男,33岁。全身泛发红斑伴瘙痒13年余,加重近半年,并出现眉毛脱落。皮损组织病理检查示：真皮毛囊周围炎性细胞浸润。阿新蓝染色阳性,毛囊内可见黏蛋白沉积。诊断为毛囊黏蛋白病。     

网状红斑黏蛋白病并发蕈样肉芽肿1例

报告网状红斑黏蛋白病并发蕈样肉芽肿1例。患者女。42岁。因躯干、上肢红斑14年,皮损增多,加重5年就诊。6年前行皮损组织病理学检查示真皮乳头层及网状层有大量黏蛋白样物质沉积,诊断为网状红斑黏蛋白病,入院皮肤科检查：头部、面部、颈部、躯干、四肢见片状轻度浸润性红斑,部分融合成网状,腹部红斑上可见紫红色浸润性斑块。皮损组织病理检查：真皮肉淋巴细胞苔藓浸润,部分细胞异形,少量浸润细胞移入表皮,形成Pautrier样微脓种,免疫组化染色结果示：LCA（＋＋＋）,CD45RO（＋＋＋）,诊断。蕈样内芽肿。     

Purely follicular mycosis fungoides without mucinosis: report of two cases with review of the literature

Follicular lesions can either associate with typical patch-/plaque-type mycosis fungoides or, more rarely, be the only clinical manifestation of the disease. We describe 2 adult patients who presented with alopecia and disseminated follicular erythematous papules, and comedones and cysts, respectively. In both patients, histology showed a folliculotropic infiltrate of atypical lymphocytes that spared the epidermis, in the absence of follicular mucinosis. Molecular genetic analysis confirmed the oligo/monoclonal nature of the T-cell infiltrate. Reported cases of purely follicular mycosis fungoides without mucinosis are reviewed.     

Basaloid folliculolymphoid hyperplasia with alopecia as an expression of mycosis fungoides (CTCL)

Follicular mucinosis, papules, cysts and comedones have been previously described as expressions of follicular involvement by mycosis fungoides. We report a patient with mycosis fungoides who developed extensive alopecia. Multiple scalp biopsies showed perifollicular and intrafollicular infiltrate of lymphocytes but no evident follicular mucinosis. On transverse sections many of the follicles showed an absence of differentiation towards hair sheath, canal, sebaceous gland or hair formation, but instead formed undifferentiated basaloid structures. These basaloid structures showed transition from atrophic telogen follicles to hypertrophic basaloid islands infiltrated by lymphocytes, resembling the pattern previously described in cutaneous lymphadenoma. Immunophenotyping showed a predominance of helper T-cells which, on ultrastructural examination, showed cerebriform nuclei. The unusual histological findings in our case may be analogous to the hyperplasia seen in sweat glands in syringotropic mycosis fungoides (syringolymphoid hyperplasia), and we propose the term basaloid folliculolymphoid hyperplasia to describe this feature. Basaloid follicular hyperplasia has been previously described as a component of follicular mucinosis but may apparently develop in the absence of overt mucinosis.     

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.     

Follicular mycosis fungoides. A histopathologic analysis of nine cases

BACKGROUND: The spectrum of mycosis fungoides is exceedingly broad. Many different variants have been described, based on both clinical appearance and histological pattern. A rare form which shows preferential infiltration of hair follicles by malignant lymphocytes is follicular mycosis fungoides. METHODS: We reviewed our experience with nine cases of follicular mycosis fungoides. RESULTS: The unifying feature was infiltration of the hair follicle epithelium by atypical lymphocytes causing varying degrees of damage to the hair follicles. In some specimens the lymphocytes displayed only minor atypia leading to a misinterpretation as pseudolymphoma. Gene rearrangement studies were particularly helpful for establishing a diagnosis of malignant lymphoma. Additionally, epidermotropism of lymphocytes, eosinophils and mucin deposition were present to varying degrees. Mucin makes the distinction from mycosis fungoides-associated follicular mucinosis difficult. We found both dermal mucin and a follicular mucinosis pattern present at different stages of disease in the same patient. CONCLUSIONS: We suggest the term mycosis fungoides-associated follicular mucinosis should be replaced by follicular mycosis fungoides in future lymphoma classification schemes.     

Adult T‐cell leukemia–lymphoma associated with follicular mucinosis

Follicular mucinosis (alopecia mucinosa) is often associated with malignancies including mycosis fungoides and Sézary syndrome, but not adult T‐cell leukemia–lymphoma (ATLL). We report a 49‐year‐old male patient who had pruritic follicular papules and erythemas clinically, and follicular and perifollicular infiltrates and follicular mucin deposition histopathologically. The patient showed 11% of flower‐shaped atypical lymphocytes in blood examination and positive human T‐cell leukemia virus type 1 antibody in serology, consistent with the chronic type of ATLL. This case seems to be a very rare association of follicular mucinosis and chronic ATLL, suggesting that malignant T cells may have a feature of folliculotropism as well as epidermotropism.     

Follicular mucinosis associated with mycosis fungoides

A 62-year-old man with a 13-year history of mycosis fungoides presented with a 2-month history of alopecia of the scalp. The mycosis fungoides had remained untreated for the previous 3.5 years. A biopsy specimen from the scalp showed follicular mucinosis in association with mycosis fungoides.     

Mycosis fungoides presenting as Ofuji''s papuloerythroderma

We report three patients presented with clinical features of Ofuji's papuloerythroderma (pruritic erythematous papules and extensive erythema sparing all skin folds), however, showing histopathological findings of mycosis fungoides (Pautrier's microabscess, haloed lymphocytes, disproportionate epidermotropism, and wiry collagen bundles). One case was associated with plaque stage of mycosis fungoides and follicular mucinosis. T-cell receptor (TCR) gene rearrangement analysis in the lesional skin tissue demonstrated rearrangement of the gamma chain in all cases. HTLV-1 serology was negative for two patients who conducted HTLV-1 test.We think that Ofuji's papuloerythroderma might be a variant of early mycosis fungoides rather than secondary skin manifestations to certain cutaneous inflammatory diseases.     

Benign Idiopathic versus Mycosis-Fungoides-Associated Follicular Mucinosis

A study was undertaken in an attempt to identify useful histologic criteria that may allow differentiation between benign idiopathic and mycosis-fungoides-associated follicular mucinosis. We chose young patients because no person under 20 years of age with coexisting follicular mucinosis and mycosis fungoides disease has ever been reported. Our three most important observations in benign juvenile idiopathic follicular mucinosis were as follows: The lymphocytic infiltrate was generally confined to follicular, perifollicular, or perivascular zones with no extension of either normal or atypical mononuclear cells into the epidermis or into papillary/reticular dermis. Within follicular epithelium there were dense collections of lymphocytes with occasionally atypical-appearing nuclei in three of the eight patients, but never as Pautrier microabscesses. There was absence of a significant associated plasma cell or eosinophil-containing inflammatory dermal infiltrate. These findings are in contrast to those of older patients with follicular mucinosis and mycosis fungoides.     

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.     

Follicular mucinosis: clinical and histopathologic study

Fifty-nine patients with the histologic diagnosis of follicular mucinosis (alopecia mucinosa) were evaluated retrospectively. Thirty-seven were male and 22 were female; ages ranged from 10 to 76 years. Of 19 patients with mycosis fungoides, 16 had initial lesions of follicular mucinosis on the trunk or extremities. Two patients had Hodgkin's disease and follicular mucinosis; both were younger than 20 years of age. Seven other patients were 20 years of age or younger. Two of the nine adolescent patients had persistent plaques of follicular mucinosis up to 18 years after diagnosis. Evaluation of biopsy specimens for lymphocytes, eosinophils, nonlymphoid cell infiltration, epidermal lymphocytic exocytosis, mucin deposition, and epidermal hyperplasia revealed no predominant feature that differentiated the group with benign disease from the group with mycosis fungoides. We conclude that no single clinical or histopathologic observation predicts which patients with follicular mucinosis will have a benign course and that evaluation of multiple clinical and histologic variables is necessary.