毛发疾病

20140862 53例瘢痕性秃发的临床和病理特点分析/戚世玲（中山大学第一医院皮肤科）,赵莹,张小婷…∥中华皮肤科杂志.-2013,46（10）.-731-735 回顾性分析53例瘢痕性脱发患者临床资料,分析其组织病理、皮肤镜征象、治疗和预后的特点。结果：瘢痕性脱发以脱发、毛囊开口消失、毛囊皮脂腺单位数目减少或消失为共同特征。     

雄秃样纤维性秃发一例

【摘要】 报道1例雄秃样纤维性秃发（FAPD）及其临床病理、皮肤镜和TrichoScan特征,以提高对FAPD的认识。患者男,23岁,前额及头顶部进行性脱发10年,伴局部头发变细、软,偶有头皮瘙痒。皮肤科检查：前额至头顶部头发弥漫性稀疏,前额发际线后移,局部发质细软,脱发区可见部分毛囊角化性丘疹、毛囊周围红斑,未见明显鳞屑。TrichoScan检查：毛发密度明显降低,毳毛比例增加。皮肤镜检查：部分毛囊开口消失,融合性白点征。头皮组织病理检查：毛囊漏斗部、峡部淋巴细胞苔藓样浸润,毛囊周围同心层状纤维化,毛囊结构破坏,毛囊性微瘢痕形成,残留毛囊直径变异度明显增加,可见部分毳毛。诊断：FAPD。FAPD临床易误诊为雄激素性秃发,需及早诊断并治疗。     

前额纤维性脱发7例的临床特征及文献回顾

目的探讨前额纤维性脱发患者的临床表现、皮肤镜下特征、病理特点及疗效。方法收集本院毛发专科门诊2014年12月1日-2016年10月1日前额纤维性脱发患者7例,回顾分析其流行病学资料、临床表现、皮肤镜表现、病理特点及治疗效果。结果 7例患者均为女性,发病年龄19~54岁。所有患者均有前额、颞部发际线后退表现,眉毛脱失似与绝经相关。皮肤镜下,所有患者均有毛囊口消失、毛周红斑的征象,3例具有毛周角化。5例病理均提示毛囊周围淋巴细胞浸润伴纤维增生,5例患者接受口服羟氯喹、甲泼尼龙,外用卤米松或0.1%他克莫司乳膏治疗,其发际线后退速度减慢或停止。结论前额纤维性脱发为前额和颞部发际线处的瘢痕性脱发,好发于绝经期女性,亦可侵及眉毛和腋毛,治疗不能导致毛囊的再生,但可减缓病情进展。     

原发性瘢痕性脱发的研究进展

原发性瘢痕性脱发是一组以毛囊为中心受到永久性和炎症性破坏为特征的脱发性疾病,病因不明。按病理特点分为淋巴细胞性(如盘状红斑狼疮脱发和毛囊扁平苔藓)、中性粒细胞性(如脱发性毛囊炎和分割性蜂窝织炎/毛囊炎)、混合性(如瘢痕疙瘩性毛囊炎/痤疮)和非特异性4类。本病有不可复性,如不能及时而有效的接受治疗,进展到瘢痕晚期会导致永久脱发。治疗可阻止病变发展,但不能彻底根治,故本病易反复发作。本文对原发性瘢痕性脱发的诊断、治疗和发病机制等进展进行综述。     

棘状秃发性毛囊炎

报告1例棘状秃发性毛囊炎.患者男,27岁.因头皮起毛囊性丘疹伴脱发7年就诊.皮肤科检查:头皮片状不规则脱发.有萎缩性瘢痕,头皮、面部、颈部、躯干、四肢广泛分布粟粒大毛囊性丘疹.组织病理检查可见角化过度,表皮萎缩变薄,真皮内胶原纤维增生,呈瘢痕样改变,部分毛囊破坏,周围淋巴细胞、浆细胞及多核巨细胞浸润,符合棘状秃发性毛囊炎改变.     

棘状秃发性毛发角化病

报告1例棘状秃发性毛发角化病.患者男,22岁.因头皮弥漫性毛囊角化性丘疹伴脱发10余年就诊.皮肤科检查:头皮弥漫性瘢痕性脱发和毛囊角化性丘疹,并可见散在分布细小和稀疏的头发.组织病理检查可见表皮毛囊角化过度和轻度的毛囊周围纤维化,毛囊和血管周围有少量慢性炎性细胞浸润.结合临床和组织病理检查,符合棘状秃发性毛发角化病的诊断.临床上,该病需与棘状秃发性毛囊炎、毛囊性鱼鳞病伴脱发和畏光综合征(IFAP)、角膜炎-鱼鳞病-耳聋综合征(KID综合征)和无萎缩脱毛性毛发角化病等病鉴别.     

红斑狼疮脱发的研究进展

脱发是红斑狼疮(LE)患者的常见临床表现之一,且与疾病活动性有关,可分为瘢痕性脱发与非瘢痕性脱发两大类,前者为盘状红斑狼疮(DLE)脱发,后者可分为狼疮发、斑状脱发、弥漫性脱发。不同类型的LE脱发在临床、皮肤镜和组织病理学表现上各有特点。红斑狼疮非瘢痕性脱发患者的常见皮肤镜表现包括毛细血管扩张、毛干直径差异明显及色素减退、头皮色素沉着、白点征、褐色毛周征等。LE患者的非瘢痕性斑状脱发在临床和皮肤镜表现有异于斑秃。LE脱发的治疗要及时和适宜,尤其是早期DLE脱发患者治疗后毛发可部分再生,非瘢痕性脱发患者可基本痊愈。     

皮肤镜在毛发疾病诊断中的应用

皮肤镜是一种皮肤显微镜,具有无创性、放大性和便携性,但皮肤镜并不单纯是放大镜:借助于偏振光的原理,皮肤镜在放大皮肤表面形态的同时,能够观察到皮下真皮浅层的血管分布、毛囊结构等,在毛发疾病中可用于观察毛囊型及毛囊间型的皮肤镜特点,以及毛干和毛根处头皮情况等相关内容。常见的脱发疾病有其特异性或非特异性的皮肤镜征象,掌握这些皮肤镜征象将有助于常见脱发疾病的诊断和鉴别诊断,如非瘢痕性斑状脱发的鉴别诊断、急性弥漫性脱发的鉴别诊断等;而斑秃患者的黑点征、感叹号发与断发的存在与否对临床治疗具有重要的指导意义。     

棘状秃发性毛囊炎

报告1例棘状秃发性毛囊炎.患者男,41岁.头面部反复出现红色脓疱性丘疹伴疼痛、脱发近41年.家族中其大姐有类似疾病.毛囊性丘疹和脓疱在青春期加重,并伴有畏光、结膜干燥、睑缘炎.皮肤科检查:头皮弥漫性秃发,可见网状瘢痕形成和色素脱失.眉弓、上唇和下颌散在分布许多红色丘疹,有的丘疹顶端有脓疱.双侧眉毛、腋毛和阴毛大部分缺失,局部可见散在毛囊角化性丘疹.皮损组织病理检查:表皮萎缩,真皮可见毛囊,其周围有较多淋巴细胞、组织细胞浸润,并有纤维组织增生.结合临床和组织病理表现,符合棘状秃发性毛囊炎的诊断.     

毛发疾病皮肤镜诊断专家共识

正毛发疾病的皮肤镜诊断具有无创性和快捷性,能够提供毛囊单位在皮面开口处、皮表微细结构、毛干形态、毛细血管和发根形态的信息。而且常见的脱发疾病具有与病理改变相关联的特征性皮肤镜征象,可用于脱发疾病的诊断和鉴别诊断工作,在一定程度上减少活检和病理检查的机率。本共识详细阐述了常见脱发疾病的皮肤镜征象,重点在于提供瘢痕性和非瘢痕性脱发诊断和鉴别诊断的依据。     

Cicatricial alopecia as a manifestation of different dermatoses

There are numerous dermatoses which may cause cicatricial alopecia when localized on the scalp, such as chronic discoid lupus erythematosus (DLE), lichen planus, graft-versus-host disease, dermatomyositis, scleroderma, cicatricial pemphigoid, porphyria cutanea tarda, follicular mucinosis, perifolliculitis capitis abscedens, lichen sclerosus et atrophicus, necrobiosis lipoidica, sarcoidosis, etc. Histologically, cicatricial alopecia is characterized by dermal scarring, along with absent or reduced hair follicles and reduced number of erector pili muscles. According to working classification of cicatricial alopecia by the North American Hair Society, primary cicatricial alopecia may be divided into the following categories: lymphocytic group (e.g., DLE, lichen planopilaris, classic pseudopelade (Brocq), central centrifugal cicatricial alopecia); neutrophilic group (e.g., folliculitis decalvans, dissecting cellulitis); and mixed group (e.g., folliculitis keloidalis). Over a 5-year period, 36 patients with cicatricial alopecia were hospitalized at our Department: DLE (n = 27), pseudopelade Brocq (n = 3), mucinosis follicularis (n = 2), and lichen planopilaris, folliculitis decalvans, folliculitis abscedens and folliculitis keloidalis (one patient each). Clinical evaluation was compared with histopathologic analysis of follicular architecture, as well as with the type, localization and extent of inflammatory infiltrate. Scalp biopsy was considered mandatory in all cases. Our experience indicates the need of more complex research to extend the knowledge about the etiopathogenesis and treatment options for cicatricial alopecia. We hope that this type of alopecia may attract more attention and research in the future.     

Dermoscopy guided scalp biopsy in cicatricial alopecia

Background Scalp biopsies are crucial for the diagnosis of cicatricial alopecia. However, the pathologic interpretation may not be diagnostic if biopsy is not obtained from the correct site. This is particularly relevant for cicatricial alopecia as the disease may be focal and disease activity difficult to appreciate by the naked eye. Objective To report a new simple technique to select the optimal biopsy site in cicatricial alopecia. Methods In the last 2 years we performed dermoscopy guided scalp biopsies using handled dermatoscopes in 80 patients with different forms of cicatricial alopecia. Biopsy site was selected based on presence of the following dermatoscopic features: perifollicular concentric white scales in lichen planopilaris, frontal fibrosing alopecia (FFA) and discoid lupus erythematosus (DLE); hair tufts in folliculitis decalvans, hairs surrounded by a peripilar grey‐white halo in central centrifugal cicatricial alopecia and follicular red dots or keratotic plugs in DLE. Results The dermoscopy guided biopsies yielded a definitive pathological diagnosis in 95% of the cases. Comment The advantage of this method is that it is a fast, precise way to identify even individually affected follicles in early or focal cicatricial alopecia. It also allows for the morphologic characterization of particular follicular structures.     

Primary cicatricial alopecias: clinicopathology of 112 cases

BACKGROUND: Cicatricial alopecias represent a diverse group of diseases characterized by a lack of follicular ostia and irreversible alopecia. There is limited literature on the epidemiology and therapeutics of cicatricial alopecias. OBJECTIVE: The aim of this study was to review the epidemiology, clinical characteristics, and treatment of inflammatory cicatricial alopecias in a mixed ethnic population referred to a university hair clinic. METHODS: The study population consisted of 112 patients seen during a 5-year period with acquired primary cicatricial alopecias. This represented 3.2% of the total number of trichologic consultations seen at the University of British Columbia Hair Clinic, Vancouver, British Columbia, Canada. RESULTS: The ratio of lymphocytic to neutrophilic cicatricial alopecias was 4:1. Lymphocytic cicatricial alopecias had a tendency to affect middle-aged women, whereas neutrophilic cicatricial alopecias had a predilection for middle-aged men. CONCLUSIONS: An accurate diagnosis of cicatricial alopecia is achieved through careful clinicopathologic evaluation. We suggest that a scalp biopsy is mandatory in all cases. Multiple biopsies may be necessary for some affected individuals to achieve a definitive diagnosis as a result of a highly variable clinical course. An aggressive multiple modality therapeutic approach is often necessary to prevent further irreversible follicular destruction, implying cicatrical alopecia should be considered a trichologic emergency. Current therapeutic options for lymphocytic cicatricial alopecia include corticosteroids, antimalarials, and isotretinoin versus antibiotics, corticosteroids, and isotretinoin for neutrophilic cicatricial alopecias.     

Tufted hair folliculitis: a pattern of scarring alopecia?

We present a patient with pemphigus vulgaris who, over the years, experienced the development of tufted hair folliculitis as a result of scalp involvement. Multiple hairs emerged from widely dilated follicular ostia surrounded by indurated, scarred skin. Histopathologic findings were typical for tufted hair folliculitis. We believe that because a specific host response to scalp injury might be crucial to the development of this rare disorder, it should be regarded as a type of scarring alopecia.     

Management of primary cicatricial alopecias: options for treatment

Primary cicatricial alopecias (PCAs) are a poorly understood group of disorders that result in permanent hair loss. Clinically, they are characterized not only by permanent loss of hair shafts but also of visible follicular ostia along with other visible changes in skin surface morphology, while their histopathological hallmark usually (although not always) is the replacement of follicular structures with scar-like fibrous tissue. As hair follicle neogenesis in adult human scalp skin is not yet a readily available treatment option for patients with cicatricial alopecias, the aim of treatment, currently, remains to reduce symptoms and to slow or stop PCA progression, namely the scarring process. Early treatment is the key to minimizing the extent of permanent alopecia. However, inconsistent terminology, poorly defined clinical end-points and a lack of good quality clinical trials have long made management of these conditions very challenging. As one important step towards improving the management of this under-investigated and under-serviced group of dermatoses, the current review presents evidence-based guidance for treatment, with identification of the strength of evidence, and a brief overview of clinical features of each condition. Wherever only insufficient evidence-based advice on PCA management can be given at present, this is indicated so as to highlight important gaps in our clinical knowledge that call for concerted efforts to close these in the near future.     

Value of direct immunofluorescence for differential diagnosis of cicatricial alopecia

BACKGROUND: There are diverse causes of cicatricial alopecia characterized by lack of follicular ostia and irreversible loss of hair. While clinical differentiation between the causes may be difficult, particularly with regard to lichen planus (LP), lupus erythematosus (LE) and pseudopelade of Brocq (PB), it has been suggested that both histopathologic examination and direct immunofluorescence studies (DIF) are necessary for an accurate diagnosis. OBJECTIVE: The aim of this study was to evaluate the diagnostic value of DIF studies in addition to histopathology in patients with cicatricial alopecia as a clinical feature. METHODS: 136 scalp biopsy specimens received for histopathology and DIF during a 5-year period were reviewed. RESULTS: Definitive diagnosis was achieved by careful evaluation of scalp biopsies. The most prevalent diagnoses in order of frequency were LP (26%), LE (21%) and folliculitis decalvans (20%). PB was diagnosed in 10%. In most cases, the diagnosis could be made on the basis of histopathology and independently of DIF. Characteristic DIF patterns showed high specificity, but low sensitivity for LP, and high specificity and sensitivity for LE. The DIF pattern in PB showed no difference to LP. CONCLUSIONS: Histopathology permits diagnosis in the majority of cicatricial alopecias. DIF is of value in histopathologically inconclusive cases, particularly when LE is in question.