婴幼儿先天性色素痣126例临床及病理特征分析

【摘要】 目的 总结婴幼儿先天性色素痣的临床及病理特征。方法 回顾性分析2015年1月至2020年1月在西京皮肤医院确诊的126例婴幼儿先天性色素痣患儿的临床及病理资料。计数资料比较采用χ2检验。结果 126例婴幼儿先天性色素痣患儿中,男68例,女58例;86.5%的患儿出生即有皮损;57.9%就诊年龄2 ~ 3岁。皮损发生部位包括头面部（76例,60.3%）、躯干（24例,19.1%）、四肢 （26例,20.6%）。36例（28.6%）为先天性小痣,68例（54.0%）为M1型中型痣,13例（10.3%）为M2型,9例（7.1%）为巨痣。121例（96.0%）皮损单发,5例（4.0%）多发,44例（34.9%）痣伴粗毛,15例（11.9%）伴丘疹/增生性结节,6例（4.8%）有卫星灶。病理亚型包括混合痣120例（95.2%）、皮内痣4例（3.2%）和交界痣2例（1.6%）。38例（30.1%）镜下皮损深度< 1 mm,61例（48.4%）1 ~ 2 mm,25例（19.8%） > 2 mm,45例（35.7%）浸润至皮下脂肪层或更深。126例色素痣皮损中,常见病理特征包括痣组织成熟现象（100%,不包括2例交界痣）,角质层色素颗粒（42.1%）,分布紊乱/不对称（63.5%）,表皮痣细胞散在分布（72.2%）和呈Paget样扩散（53.2%）,真皮可见噬黑素细胞（56.4%）,痣细胞沿毛囊皮脂腺分布（65.1%）等。特殊病理特征包括痣细胞嵌入血管/淋巴管腔内（33.3%）、痣细胞松解（35.7%）、纤维瘤样改变（19.8%）、累及立毛肌（24.6%）、肥大细胞浸润（23.8%）等。不同临床表现的婴幼儿先天性色素痣病理模式：浸润深度 > 2 mm、角质层色素颗粒和角质层柱状色素颗粒在巨痣中的发生率明显高于其他大小皮损（χ2 = 7.93、10.76、5.89,均P < 0.05）;浸润深度 > 2 mm、表皮海绵水肿伴痣细胞散在分布、痣细胞巢沿毛囊皮脂腺分布、纤维瘤样改变、肥大细胞浸润在伴有粗毛皮损中的发生率明显高于不伴粗毛者（χ2 = 28.29、8.11、6.22、7.92、8.19,均P < 0.01）;表皮痣细胞呈Paget样扩散、痣细胞有异型性在伴丘疹/增生性结节的皮损中的发生率高于不伴丘疹增生性结节的皮损（χ2 = 4.92、6.30,均P < 0.05）。结论 婴幼儿先天性色素痣的临床及组织病理具有独特性,细胞常见不典型性,确诊及治疗选择需要密切结合临床与病理特征。     

皮脂腺痣126例临床病理分析

目的探讨皮脂腺痣不同年龄阶段的临床表现、组织病理学特点及肿瘤发生率。方法收集126例皮脂腺痣患者,按年龄分成三组进行回顾性分析。结果婴儿儿童期(<10岁)10例,皮损单发,好发于头皮和面部,略高出皮面,表面光滑无毛发,病理为表皮轻度肥厚,真皮皮脂腺发育不全,并有少量幼稚毛囊;青春发育期(10～20岁)80例,76例单发,4例多发,皮损明显隆起,疣状,黄色,病理为表皮角化过度,棘层肥厚呈乳头瘤样增生,真皮不同程度的皮脂腺增生,部分病例可见幼稚毛囊和/或异位大汗腺;成人期(>20岁)36例,有3例多发,临床与病理表现同青春发育期。在总共126例患者中有幼稚毛囊者65例(51.59%),异位大汗腺36例(28.57%),有7例(5.56%)并发肿瘤,其中2例(1.59%)为基底细胞上皮瘤。结论①婴儿儿童期的临床表现和病理形态与青春发育期及成人期不同。②皮脂腺痣并发肿瘤的概率为5.56%,远低于传统10%～40%的认识。     

皮脂腺痣17例临床病理分析

目的　探讨皮脂腺痣与表皮及皮肤附属器错构瘤样结构的关系。方法　收集 17例皮脂腺痣 ,分析、总结其临床病史 ,病理学改变特点。结果　 14例 (82 %)初生时发病 ,男女之比为 1.3 0∶1(9∶8) ,本病主要表现为毛囊发育不良和皮脂腺增生 ,4例伴有表皮痣 ,1例伴有基底细胞癌 ,1例伴表皮样囊肿 ,6例伴有大汗腺异位。结论　皮脂腺痣是一种错构瘤样增生 ,常伴有其他表皮或皮肤附属器错构瘤样结构存在。     

768例黑素细胞痣及类似可疑损害切除活检的回顾性分析

目的：了解黑素细胞痣诊断准确性，病理特征，去除方法及复发等问题。方法：回顾分析我院1990年－2000年诊断或疑诊黑素细胞痣的768例活检病理资料，结果：诊断为黑素细胞痣447例，疑诊为黑素细胞痣296例，黑素细胞痣恶变待排25例，共有30例（3．91％）病理诊断为恶性肿瘤，其中恶性黑素瘤6例（0．78％），基底细胞癌22例（2．86％），Bowen病2例（0．26％），结论：黑素细胞痣的诊断应予重视，在有疑问时，手术切除并做病理检查十分必要。     

Spitz样肿瘤320例临床及组织病理分析

【摘要】 目的 总结Spitz样肿瘤的临床及组织病理特征。方法 回顾2005年1月至2020年1月西京皮肤医院确诊的320例Spitz样肿瘤患者的临床及病理资料。结果 320例患者中,男141例,女179例,年龄0 ~ 65（12.5 ± 11.7）岁,病程1个月至30年;其中,Spitz痣307例,不典型Spitz肿瘤（AST）8例,Spitz痣样黑素瘤（SM）5例。皮损多为单发,可见于头面部、躯干和四肢,边界均清楚。307例Spitz痣皮损以黑色（132例,43.0%）和红色（108例,35.1%）为主,多数色素均匀（262例,85.3%）且表面平滑（272例,88.6%）。Spitz痣存在特殊临床亚型,11例 （3.6%）发生在斑痣上,11例 （3.6%）呈簇发性,6例（2.0%）播散性,7例（2.3%）结节性,1例（0.3%）为瘢痕疙瘩样。Spitz痣特征性病理表现包括表皮内痣细胞呈Paget样扩散（123例,40.1%）,真表皮交界处出现Kamino小体（74例,24.1%）,痣细胞呈水平带状（177例,57.8%）及楔形分布（118例,38.4%）,痣细胞巢周围出现裂隙（177例,57.8%）,可见生理性核分裂象（117例,38.1%）,核染色质均细腻。根据特殊组织病理表现,Spitz痣又分为色素性上皮样Spitz痣（9例,2.9%）、结缔组织增生性Spitz痣（13例,4.2%）、血管瘤样Spitz痣（8例,2.6%）、疣状Spitz痣（12例,3.9%）、黏液样Spitz痣（10例,3.3%）、晕痣样Spitz痣（4例,1.3%） 等。4例AST皮损为黑色,7例色素均匀,3例皮损表面粗糙;特征病理表现包括细胞均有轻度至中度的异型性,均可见核分裂象（7例为2 ~ 6个/mm2）,5例核染色质粗糙。3例SM皮损呈红色,4例色素不均匀,3例表面粗糙;特征病理表现包括黑素细胞呈Paget样扩散（3例）,瘤细胞呈无极性浸润性生长且均未见成熟现象,均有明显异型性,并可见病理性核分裂象（3例, > 6个/mm2）,核染色质均粗糙且核膜明显着色。结论 Spitz样肿瘤的临床及组织病理表现具有特征性,Spitz痣的临床及病理亚型繁多,AST同时具有Spitz痣和黑素瘤的临床及组织学特征。     

发育不良性痣11例临床病理学分析

目的 探讨发育不良性痣的临床病理学特点。方法 对11例临床表现为色素性损害的手术标本行H-E染色，结合临床指标及评分进行研究和分析。结果 临床表现：皮损直径≥5mm者8例，多发性损害者7例，边界模糊者4例，外形不规则者6例，痣表面色素不匀者4例，基底色红者6例。光镜下：交界痣3例，复合痣8例。发育不良性痣较为特异的组织学表现为真表皮交界处雀斑样增生，痣细胞巢增生紊乱倾向于形成“桥型”融合。表皮下方的非典型黑素细胞在基底层呈“Paget”样蔓延，真表皮交界处的黑素细胞向周围延伸，并超过真皮内的痣细胞成分。非典型黑素细胞：细胞核较角质形成细胞核大，多形性，出现核仁，深染。结论 临床和组织病理相结合是诊断发育不良性痣的可行性标准，仅根据组织学的非典型性不能诊断发育不良性痣。     

头皮黄色疣状斑块伴灰黑色结节——皮脂腺痣并发基底细胞癌

诊断:皮脂腺痣并发基底细胞癌. 皮损组织病理:表皮角化过度,表皮突延长,皮脂腺增生,且无对应之毛囊.真皮深部及皮下脂肪内可见汗腺:另见界限清楚的肿瘤团块,部分与表皮相连.肿瘤团块中及周围混有单个或巢状皮脂腺分化细胞.团块由基底样细胞组成,周边细胞排列成栅栏状,可见收缩间隙.     

巨大先天性黑素细胞痣1例

先天性黑素细胞痣（congenital melanocytic nevus,CMN）虽然出生时即有，但无遗传性，其走私较后天性黑素细胞痣大，直径＞20cm者则称为巨大先天性黑素细胞痣。现将我科诊治的1例头皮巨大先天性黑素细胞痣报告如下。     

结缔组织增生性Spitz痣和色素性梭形细胞痣临床及组织病理学特征

目的:分析结缔组织增生性Spitz痣和色索性梭形细胞痣的临床及组织病理学特点.方法:回顾性分析确诊的8例结缔组织增生性Spitz痣和9例色素性梭形细胞痣患者的临床和组织病理学特征.结果:结缔组织增生性Spitz痣表现为梭形或上皮样痣细胞增生并伴有显著的胶原硬化和均质化,色素性梭形细胞痣表现为真、表皮交界处梭形细胞增生并伴有纤细的色素颗粒沉积.结论:结缔组织增生性Spitz痣和色素性梭形细胞痣是Spitz痣中较少见的特殊类型.其中色素性梭形细胞痣需要与黑素瘤相鉴别.     

皮脂腺痣并发皮脂腺瘤

报告1例皮脂腺痣并发皮脂腺瘤.患儿男,13岁.头顶部一椭圆形淡黄色疣状新生物13年.皮肤科检查:头顶部见一约2cm×3cm椭圆形淡黄色斑块,边界清楚,表面呈轻度疣状.皮损组织病理检查:表皮棘层增生肥厚,轻度乳头瘤样增生,真皮浅中部见增生的皮脂腺及不成熟的毛囊结构,并可见异位大汗腺,真皮中部还可见多个大小不等肿瘤团块,肿瘤团块由基底样细胞和少许成熟的皮脂腺细胞组成,但未形成皮脂腺小叶样结构,团块周边细胞未见栅状排列及收缩间隙.诊断为皮脂腺痣并发皮脂腺瘤.     

Dynamic evolution of a scalp congenital melanocytic nevus with poliosis and cutis verticis gyrata

Cutis verticis gyrata (CVG), characterized by cerebriform overgrowth of the scalp, is rarely observed in congenital melanocytic nevi (CMN). We describe a 13-year-old male with autism and a large CMN of the scalp with numerous satellite nevi whose scalp nevus exhibited evolution with poliosis and CVG. Given the potential association of CVG (independent of CMN) with seizures, neuropsychiatric, and ophthalmologic disorders, and nevus-associated CVG (cerebriform intradermal nevus) with melanoma, multidisciplinary evaluation of CMN patients with CVG is important to guide management and treatment.     

Unna's and Miescher's nevi: two different types of intradermal nevus: hypothesis concerning their histogenesis

In 1991, we tentatively introduced the classification of Ackerman and Magana-García for acquired melanocytic nevi in our laboratory. We soon realized that every acquired intradermal melanocytic nevus might be easily classified into either Unna's or Miescher's patterns and that this classification had both clinical implications and significant histological differences. The decisive discriminative feature between Unna's and Miescher's nevi is that Unna's nevus is an almost purely adventitial lesion confined to expanded papillary dermis and, many times, to the perifollicular dermis too. In Miescher's nevus melanocytes diffusely infiltrate both adventitial and reticular dermis in a wedge-shaped pattern. With these concepts in mind, every acquired intradermal melanocytic nevus can be easily classified as either Unna's or Miescher's. We studied 751 acquired melanocytic nevi; 458 (61%) of them were intradermal; of these, 234 were Unna's nevi and 224 were Miescher's nevi. Eighty- three per cent of the nevi from the head and neck were intradermal nevi, whereas on the trunk and limbs junction and compound nevi were the most frequent (56%). When intradermal nevi were divided into Unna's and Miescher's patterns, it resulted that 91% of Miescher's nevi located on the face and 94% of intradermal nevi on the face were Miescher's nevi. In contradistinction, 87% of the Unna's nevi located on the neck, trunk, and limbs, and 96% of intradermal nevi from these locations were Unna's nevi. Only on the scalp was there no clear predominance of one type of intradermal nevus. A series of other histological characteristics were significantly predominant (P = 0.000) in either Unna's or Miescher's nevi. Unna's nevi had more: junctional nests above the intradermal component (40% versus 20%), a radial pattern of intradermal nests (38% versus 0%), vascular-like clefts lined by nevus cells (48% versus 4%), and in depth maturation (94% versus 0%). Miescher's nevi had more: pilosebaceous follicles within the nevus (100% versus 51%), subnevis folliculitis (12% versus 1%), large isolated melanocytes along the basal epidermal layer (47% versus 11%), multinucleated nevocytes (89% versus 44%), and adipocytes within the nevus (53% versus 11%). In conclusion, Unna's and Miescher's nevi are 2 subsets of acquired melanocytic nevus with clinical implications and significant histological differences. A histogenetic hypothesis is proposed on the basis of their histological structure.     

Desmoplastic trichoepithelioma and intradermal nevus: a combined malformation

We studied 13 desmoplastic trichoepitheliomas associated with intradermal nevi. Ten intradermal nevi were found among 76 new cases of desmoplastic trichoepithelioma (13%); three additional examples of the combined malformation were seen in consultation. Clinically, desmoplastic trichoepithelioma associated with an intradermal nevus was typically a small, firm or hard, sometimes annular, nodule on the face, particularly the cheek, of a relatively young woman. Microscopically, the combined malformation contained narrow strands of basaloid cells and keratinous cysts in a desmoplastic stroma, intimately mixed with intradermal nests of nevocytes. Melanocytic nevi have been associated with epidermal hyperplasia resembling seborrheic keratoses, follicular cysts, trichostasis spinulosa, syringomas, basal cell carcinomas, and hair follicle formation on the soles. The frequency of the occurrence of intradermal nevus with desmoplastic trichoepithelioma and the close anatomic association of the two elements may indicate that this combined malformation is another example of epithelial induction by melanocytic nevi.     

Dysplastic changes in different types of melanocytic nevi. A unifying concept

We observed histopathologic changes previously described in dysplastic melanocytic nevi in association with a dermal component characteristic of other types of melanocytic nevi or overlapping with features of other varieties of nevi. In order to determine the frequency of these changes, we studied 2,164 cases of compound melanocytic nevi that fulfilled the histopathologic criteria for the diagnosis of compound dysplastic nevus, including architectural pattern, cytologic features, and mesenchymal changes. Of the 2,164 compound dysplastic melanocytic nevi, 1,895 (87.6%) had the histopathologic characteristics previously described for dysplastic nevus, 179 (8.3%) showed a dermal component with a congenital pattern, 67 (3.1%) demonstrated epidermal and dermal characteristics of Spitz's nevus, 8 (0.3%) had features of a combined blue nevus, 13 (0.6%) had a halo phenomenon and 2 (0.1%) showed dermal neuronevus. By considering these nevi as variants of dysplastic nevi, one may apply a unified conceptual basis for their nomenclature. In order to completely describe the appearance of the nevus, we named them by adding the term "dysplastic", to their main histopathologic subtype. Accordingly, six different varieties of dysplastic nevi were identified: 1) dysplastic nevus (original); 2) dysplastic nevus with a congenital pattern; 3) dysplastic Spitz's nevus; 4) dysplastic combined blue nevus; 5) dysplastic halo nevus; and 6) dysplastic neuronevus. In summary, we conclude that the histopathologic criteria previously reported for the diagnosis of dysplastic nevi may be found in association with a dermal component characteristic of other types of melanocytic nevi or may have overlapping features with other variants of nevi.     

Histogenesis of congenital and acquired melanocytic nevi based on histological study of lesion size and thickness

The histogenesis of melanocytic nevi is poorly understood. It is important to determine the differences and similarities in histogenesis between congenital and acquired nevi. To clarify the histogenic differences between acquired melanocytic nevi (AMN) and congenital melanocytic nevi (CMN), diameter and depth of nevus cells (tumor thickness) were examined in histological specimens from 80 cases of CMN and 71 cases of AMN, and these nevi were classified according to Mark's pathological CMN criteria. In all cases, giant CMN nevus cells were found in the lower marginal portion of excised specimens. The mean diameter and lesional thickness were significantly higher in CMN than in AMN. AMN diameter showed a significant correlation (r = 0.567, P < 0.05) with lesional thickness, while no such relation was observed in CMN. In addition, a significant correlation between lesion diameter and thickness was observed in small (<10 mm) non-Mark's type CMN (r = 0.626, P < 0.05). CMN may be classified into three subtypes: (i) caused by increased proliferation of melanoblasts during the course of migration from the neural crest to the epidermis; (ii) proliferation of nevus cells after arrival at the epidermis, and nevus cell distribution affected by adnexa and dermal differentiation; and (iii) arising after completion of skin development before birth.     

Histologic spectrum of melanocytic nevi removed from patients > 60 years of age

BACKGROUND: The histology of melanocytic nevi removed from older patients often differs from that of nevi from younger adults. According to the literature, the most common nevus in older individuals is the intradermal nevus, and purely junctional nevi are rare and should alert the pathologist to a possible melanoma precursor. OBJECTIVE: To evaluate the histologic features of melanocytic nevi removed from patients > 60 year of age. METHODS: Biopsies of nevi (N=215) from 172 patients > 60 years (mean age 69+/-7 years) were examined retrospectively by three dermatopathologists, a consensus diagnosis was rendered, and the spectrum of histologic features was documented. RESULTS: Junctional melanocytic nevi were frequently diagnosed in older patients (21% of cases) and a lentiginous, often heavily pigmented growth pattern was common (12% of nevi). Severely atypical (dysplastic) changes were found in 6% of nevi removed from older patients. CONCLUSIONS: We conclude that benign junctional nevi are relatively common in older patients and that a lentiginous, heavily pigmented growth pattern, traditionally associated with younger individuals, is often seen in both junctional and compound nevi in this older age group. This pattern must be differentiated from dysplastic nevus and melanoma in situ, which they may clinically resemble.     

Proliferation, apoptosis, and survivin expression in a spectrum of melanocytic nevi

BACKGROUND: Apoptosis is important for maintenance of tissue homeostasis and often dysregulated in cutaneous neoplasms. The apoptosis inhibitor survivin is expressed in melanoma and non-melanoma skin cancers and benign keratinocytic lesions. Its expression has not been studied in melanocytic nevi. OBJECTIVE: We determined the expression pattern of survivin in benign melanocytic nevi in comparison to markers of proliferation and apoptosis. METHODS: Six cases of each of the following melanocytic nevi were retrieved from a dermatopathology archive: compound dysplastic nevus, intradermal nevus, compound nevus, neurotized intradermal nevus, and Spitz nevus. Survivin expression was evaluated by in situ hybridization. Apoptotic and proliferation indices were calculated by counting immunoreactive cells in terminal deoxynucleotidyl transferase (TdT)-mediated dUTP nick end labeling and proliferating cell nuclear antigen immunostained sections, respectively. RESULTS: All nevi, regardless of histologic type, expressed survivin. Compound melanocytic lesions expressed survivin in both epidermal and dermal compartments. The apoptotic rate was low for dysplastic, compound, and Spitz nevi, and apoptotic cells were not identified in any neurotized nevus. The proliferative index was highest for Spitz nevi, while all other nevi demonstrated rare positive cells. CONCLUSIONS: Survivin is consistently expressed in benign melanocytic lesions, while apoptotic cells are rarely identified, suggesting the dysregulation of apoptotic pathways with the accumulation of cells in these neoplasms.     

Four Cases of Lobulated Intradermal Nevus: A Sign of Aging Melanocytic Nevus

Melanocytic nevi are subject to change with age in both clinical and histopathologic findings. In 1991, Cho et al. first reported three cases of lobulated intradermal nevi and suggested their cases represented an unusual form of regressing melanocytic nevus. Herein we report four cases of lobulated intradermal nevus and review previous literature.     

Balloon cell nevus in children: Report of three cases of the scalp and review

Balloon cell nevus (BCN) is a histopathological variant of cutaneous acquired melanocytic nevi characterized by junctional and/or dermal nests of large cells with a clear and foamy cytoplasm which has rarely been described in children. Three cases of BCN firstly reported on the scalp in two pediatric patients are presented along with a literature review. Dermoscopy is particularly indicated in those pigmented lesions showing a yellowish hue, in ruling out in real time those disorders that may clinically be similar such as xanthogranuloma and sebaceous nevus, and to suggest the diagnosis of BCN. The final diagnosis, however, is established by histopathological examination.