毛囊黏蛋白病研究进展

毛囊黏蛋白病是以毛囊内黏蛋白沉积为组织病理特征的较罕见的疾病。该病分为原发性和继发性2种形式，其中继发性毛囊黏蛋白病常见于皮肤T细胞相关淋巴瘤，如蕈样肉芽肿。原发性和继发性毛囊黏蛋白病在临床表现上有许多重合之处，临床上需要鉴别诊断这2种类型的毛囊黏蛋白病。由于不同类型毛囊黏蛋白病的治疗反应和预后大相径庭，了解其鉴别诊断及相应处理有助于及时采取积极的治疗方案以控制疾病进展，同时可有效避免过度治疗。该文对毛囊黏蛋白病的不同表型、发病机制及可能的治疗方案等最新研究进展作一综述，为临床工作提供参考。     

内分泌、代谢、营养障碍性皮肤病

类脂质渐进性坏死；结节性皮肤狼疮性黏蛋白病；毛囊黏蛋白病；烟酸缺乏症伴严重神经症状1例；     

毛囊黏蛋白病1例

毛囊黏蛋白病临床少见，最近诊治1例，报道如下     

毛囊黏蛋白病二例并文献复习

报道2例毛囊黏蛋白病并进行文献复习.患者1,男,44岁,颈项部局限性浸润性红斑伴疼痛2个月,组织病理示:真皮毛囊水肿,毛囊内外淋巴组织细胞浸润,个别嗜酸粒细胞.阿新蓝染色:毛囊黏蛋白阳性.患者2,男,32岁,左面部浸润性暗红斑45天,组织病理示:表皮大致正常,毛囊上皮水肿,淡蓝色黏液样物质沉积,毛囊内及毛囊周围较多嗜酸...     

环状肉芽肿并毛囊黏蛋白病1例

患者男,36岁,左侧颈部环状斑块伴痒麻感3个月余。皮损组织病理示:真皮内多处胶原纤维变性灶,其间见黏蛋白沉积,周边淋巴细胞浸润,毛囊见黏蛋白变性;黏蛋白阿辛蓝染色阳性;免疫组织化学:CD3阳性、CD4阳性、CD8及TiA1散在阳性。诊断:环状肉芽肿并发毛囊黏蛋白病。手术切除后无复发。     

毛囊黏蛋白病的临床及组织病理特点

目的:分析14例毛囊黏蛋白病患者的临床和组织病理表现.方法:对14例毛囊黏蛋白病患者临床资料进行回顾性分析,包括临床、组织病理、误诊及治疗情况.结果:该病多见于中青年男性,皮损好发于头部;14例患者均有毛囊周围细胞浸润和黏蛋白沉积(占100％);对羟氯喹的治疗效果均不好;14例中误诊为8例(占57％).12例患者为慢性良性型,2例属于恶性型.结论:该病皮损主要分布头部,基本皮疹为浸润的斑块和结节.皮损组织病理检查显示毛囊及其周围有黏蛋白聚积,阿新蓝染色阳性提示诊断毛囊黏蛋白病的线索.     

儿童毛囊黏蛋白病

报告1例儿童毛囊黏蛋白病.患儿男,14岁.因眉间皮肤起浸润性红斑伴眉毛部分脱落半年余就诊.皮肤科检查:眉间偏左侧一约2.5 cm × 3.0 cm浸润性红斑,其上覆少许片状鳞屑,皮损累及处眉毛缺失.皮损组织病理检查:表皮轻度角化不全,棘层增生,真皮毛囊上皮水肿、破坏,少许淋巴细胞移入,胶原间血管壁增厚,周围淋巴细胞及嗜酸性粒细胞浸润;阿新蓝染色示毛囊上皮内较多黏液样物质沉积.诊断:毛囊黏蛋白病.给予复方倍他米松注射液局部封闭治疗后,皮损消退.     

毛囊黏蛋白病

报告1例毛囊黏蛋白病。患者女,32岁。头部、面部及背部红斑伴脱发半年。头部可见3个甲盖大的局限性脱发区,并伴局部脱屑,面部及背部可见大小不等的红斑伴脱屑。皮损组织病理检查示表皮轻度角化过度伴角化不全,棘层增厚,皮突不规则向下伸长;真皮血管及毛囊周围轻度淋巴细胞浸润,毛囊明显变性,局部形成空腔,有黏液样物质沉积。黏蛋白染色(+),抗酸染色(-)。免疫组化:扁豆凝集素(LCA)(+)、CD3(灶性+)、CD20(灶性+)、CD4(-)及CD75(-)。T细胞抗原受体(TCR)γ基因重排呈多克隆特性。诊断:毛囊黏蛋白病。治疗:予口服硒脂糖、维生素E及咪唑斯汀,外用地塞米松及氧化锌对症治疗。治疗4个月后皮疹消退,近8年患者随访无复发。     

毛囊黏蛋白病1例

患者男,13岁。头皮起红疹伴脱发半年。皮肤科情况:后枕部见一1.5cm×1.5cm红色圆形斑块,其上有细小脱屑,头发大部分脱落。皮损组织病理毛囊口角栓形成,真皮内毛囊皮脂腺细胞中大量淡兰色物质沉积,血管周围不等量炎细胞浸润;阿西蓝染色阳性。诊断:毛囊黏蛋白病。     

网状红斑黏蛋白病1例

报告1例网状红斑黏蛋白病。患者男，49岁。头面部、胸背部红斑、丘疹伴瘙痒10年，日晒后皮损无明显改变。组织病理检查示真皮乳头层，网状层上部血管及毛囊周围淋巴细浸润，真皮胶原束之间有阿新蓝染色阳性的黏蛋白沉积。     

毛囊粘蛋白病1例

患者男,21岁。头部浸润性斑块伴脱发8年。组织病理示毛囊周围可见粘蛋白样物质沉积,甲苯胺蓝染色呈紫红色。诊断:毛囊粘蛋白病。     

Pilotropic T-cell lymphoma without mucinosis: 5 new cases]

BACKGROUND: Pilotropic cutaneous T-cell lymphomas without mucinosis are rare, with 27 cases previously reported. Diagnosis and classification may be difficult. The clinical course and histopathological and immunohistochemical findings in 5 patients are described. PATIENTS AND METHODS: Patients were selected from the register of the French Study Group for cutaneous lymphomas. The criteria for inclusion were clinical pilofollicular manifestations and histological features of pilotropic T-cell lymphoma without mucinosis. RESULTS: Five patients were selected. The most frequent clinical manifestations were follicular keratosis, alopecia and follicular papules. Typical lesions of mycosis fongoides were present for several years in 3 patients, and lymphomatoid papulosis preexisted in one patient. Histopathological analysis showed an infiltrate composed of CD3+ and CD4+ atypical lymphocytes involving the follicular epithelium with alteration of the hair follicle walls. Epidermotropism was associated with pilotropism and situated near the follicular lesions or farther apart. Alcian blue stains results were negative in all specimens. PCR studies showed the presence of a T-cell clone in the skin lesions in all cases. COMMENTS: Diagnosis of pilotropic cutaneous T cell lymphomas without mucinosis may be difficult in case of discrete epidermotropism, minimal infiltrate or involvement of the follicular epithelium. Pilotropism could define a particular variant of T-cell lymphomas.     

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.     

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 mucinosis - Case report

Follicular mucinosis, also known as alopecia mucinosa, is a cutaneous mucinosis histologically characterized by accumulation of dermal type mucin in the pilosebaceous follicle and sebaceous glands. It presents in two forms, a primary or idiopathic form and a secondary form associated with various benign or malignant processes. Among the malignant processes, the main association is with mycosis fungoides. The frequent overlap of clinical, histopathological, immunohistochemical and molecular biology characteristics makes the correct classification of these conditions difficult, therefore a long follow-up of all cases is recommended. We report the case of an adolescent with disseminated lesions and discuss the difficulty of early identification of secondary follicular mucinosis associated with cutaneous lymphoma.     

Follicular mycosis fungoides

We describe a patient with follicular mycosis fungoides (MF), a rare folliculotropic variant of cutaneous T-cell lymphoma (CTCL). Follicular involvement in CTCL usually presents clinically as alopecia mucinosa associated histologically with follicular mucinosis. Follicular MF differs from alopecia mucinosa/follicular mucinosis associated with MF with regard to its clinical presentation, histology and, presumably, prognosis. Our patient presented with the characteristic findings of follicular MF, i.e. infiltrated plaques showing numerous enlarged, comedo-like follicular infundibula; histology was dominated by exclusive folliculotropism of atypical lymphocytes sometimes forming follicular Pautrier's microabscesses, and by lack of epidermotropism and follicular mucinosis. Despite photochemotherapy and treatment with oral retinoids and interferon alpha, the patient's follicular MF rapidly developed into a progressive CTCL with large tumorous lesions, but responded to electron beam therapy. The course of our patient's disease confirms the notion that follicular MF may be associated with a worse prognosis than classical MF. However, electron beam irradiation induced remission of follicular MF that was maintained by a combination therapy consisting of extracorporeal photopheresis and interferon alfa.     

Prominent follicular mucinosis with diffuse scalp alopecia resembling alopecia areata

A 56‐year‐old Caucasian female presented with a 2‐month history of alopecia. On examination, she had diffuse hair loss of her scalp with some discrete patches of nonscarring alopecia. Histopathology revealed an inflammatory nonscarring alopecia with prominent follicular mucinosis and findings suggestive of alopecia areata. The patient's alopecia completely resolved with oral prednisone. The histopathologic findings and clinical presentation are most consistent with a diagnosis of alopecia areata with follicular mucinosis, although the differential diagnosis is broad. As follicular mucinosis may be associated with both benign and malignant conditions, it is important to be cautious regarding the clinical diagnosis when this reaction pattern is observed histopathologically.     

Follicular mucinosis in alopecia areata.

The pathological features of alopecia areata were described in association with those of follicular mucinosis in the scalp pathology of an 18-year-old woman. The immunohistochemical picture of the inflammatory infiltrate showed a high CD4/CD8 ratio (25:1), which was significantly different from the CD4/CD8 ratio in patients with alopecia areata. The abundance of helper/inducer cells along with the involvement of the upper part of the hair follicles might explain the development of follicular mucinosis in this case. Different possibilities were discussed, but the clinical presentation and the follow-up favored the clinical and pathological interpretation of alopecia areata with incidental findings of follicular mucinosis.     

Nodular Clear Cell Hidradenoma: A Case Report with Immunohistochemistry, Cytogenetics and Flow Cytometry

CD44 is the cell surface receptor for hyaluronate (HA). We demonstrated that the lack of CD44 expression in murine keratinocytes leads to an abnormal HA accumulation in the dermis, indicating an important role of CD44 in local HA metabolism in mouse skin. We also observed a decrease in the expression of epidermal CD44 in patients of lichen sclerosus et atrophicus which is potentially responsable for the dermal deposition of HA. We also showed that HA accumulation was associated with decreased expression of CD44 in epithelial proliferations in perifollicular solitary cutaneous myxoma. In this study we examined the follicular expression of CD44 and HA in the skin biopsy specimens of seven patients with follicular mucinosis by using CD44‐specific antibodies and biotinylated HA‐binding protein (HABP), respectively. No difference of CD44 expression was observed in the follicular keratinocytes when compared with those of unaffected interfollicular epidermis. The follicular zones of mucin deposition were strongly positive for HA. A weak interkeratinocyte staining pattern for HA was also observed in the interfollicular epidermis. However, HABP staining revealed a stronger reactivity in the follicular keratinocytes surrounding the mucin‐accumulated areas compared to the interfollicular keratinocytes, indicating an active secretion of HA by follicle cells in follicular mucinosis.