巨大型疣状增生与显著角化过度型汗孔角化症1例

报告1例巨大型疣状增生与显著角化过度型汗孔角化症。患者女,48岁,臀部、双下肢丘疹、斑块伴剧痒5年余。体检：腰臀部见密集多发绿豆至蚕豆大小红色丘疹、斑块,双臀部皮疹融合成巨大型疣状增生;双大腿内、屈侧密集孤立粟米至黄豆大褐色角化性丘疹。皮损组织病理检查：表皮角化过度,可见角化不全的圆锥状板形成,棘层增厚。根据临床症状及皮肤组织病理学改变,诊断为汗孔角化症。     

毛囊角化病ATP2A2基因突变二例及家系调查

【摘要】 例1男，16岁，面部、颈部及双腋下见密集褐色毛囊角化性丘疹，部分融合成斑块，局部可见疣状增生；母亲与其有相似的病史及临床表现。例2男，21岁，头面部、颈部、躯干、双腋下及臀部见弥漫性毛囊角化性丘疹，部分融合成片，局部可见疣状增生；家族成员均无类似症状。例2颈部皮损组织病理：表皮角化过度伴灶状角化不全，棘层部分区域棘刺松解并有腔隙形成，可见绒毛、圆体和谷粒细胞，真皮浅层炎症细胞浸润。2例患者及其父母基因检测：例1及母亲ATP2A2基因存在第15外显子c.2300A>G错义突变；例2第15外显子与第15内含子交界处存在c.2097+5G>A 剪切区域突变。2例患者其他家族成员未见上述突变。     

泛发性毛囊角化病1例

患者男,22岁。自6岁始面部出现针尖至米粒大灰褐色或近正常肤色毛囊性散在分布的坚实小丘疹,逐渐增多、变大,泛发全身。部分融合成斑块,可见疣状增生。左肩部皮损组织病理示:角化不全,毛囊角栓形成,可见圆体和谷粒、裂隙和馅窝,有绒毛突入馅窝,表皮乳头瘤样增生,慢性炎症细胞浸润。诊断:毛囊角化病。     

沿Blaschko线分布的儿童汗孔角化症一例

13岁男性患儿,右侧腰腹部褐色丘疹10年、增多伴瘙痒6个月。皮肤科情况:右侧腰腹部密集绿豆大小、暗褐色、疣状增生性丘疹,丘疹中央轻度萎缩,边缘堤状隆起,无鳞屑,皮损整体呈S形沿Blaschko线分布。组织病理示:表皮凹窝处角化不全柱,其下方颗粒层消失,真皮浅层中等量淋巴细胞浸润。诊断:汗孔角化症(沿Blaschko线分布)。     

Fox-Fordyce病1例

患者女,24岁。双侧腋窝丘疹伴瘙痒3个月。皮肤科情况:双侧腋窝密集分布丘疹,质韧,孤立,互不融合,腋毛稀疏。皮损病理组织示:角化亢进、角化不全,表皮棘层细胞间及毛囊漏斗部上皮细胞间可见水肿。真皮毛囊及汗腺周围多少不等淋巴细胞及多量嗜酸性细胞浸润。诊断:Fox-Fordyce病。     

伴有足跖部广泛皮损的线状疣状痣1例

患者男,11岁。右下腹、右下肢线形疣状皮损11年,右足趾疣状增生9年。患者出生后1月即发现右下腹至右下肢内侧有一条略高出皮面、淡红色、表面粗糙皮疹;整个右足跖皮肤粗糙,无不适。2岁后下腹、下肢皮损逐渐增厚呈密集粟粒状。右足跖呈疣状增生伴皲裂、渗血及发臭。患儿系第一胎足月顺产,生长发育智力正常,父母非近亲婚配,家族中无类似病史。体检:系统检查未见异常。皮肤科情况:右下腹至右下肢内侧有一条淡红色、密集乳头状瘤样、过度角化性丘疹连续排列成条状,境界清,无炎症反应。皮疹延续至右足跖,整个右足跖疣状增生、干燥、过度角化伴重…     

黑头粉刺样痣综合征1例并文献复习

患者女,20岁,左足趾、足背红斑肿胀伴瘙痒2个月,加重10 d.追问病史,患者自幼头面颈部及左侧肢体就有带状分布的密集成片的黑色毛囊性丘疹,并伴左手拇指畸形、先天性巨结肠及肠梗阻病史.皮损组织病理示:表皮角化过度,可见多发毛囊漏斗样开口和表皮样囊肿,伸向真皮深层,毛囊漏斗部显著扩张,形成宽而深凹陷,凹陷内充满大量角化性...     

表现为浅表脂肪瘤样痣的毛囊皮脂腺囊性错构瘤1例

患者女,36岁。颈部丘疹30余年。颈部可见密集呈片状分布的皮色圆顶丘疹,约绿豆大小,质韧。皮损组织病理示:角化过度,表皮大致正常,真皮内可见多数大的分化成熟的皮脂腺体,开口于毛囊漏斗部,腺体周围间质有裂隙,真皮内胶原增生致密。诊断:毛囊皮脂腺囊性错构瘤。     

嗜酸性脓疱性毛囊炎

报告1例嗜酸性脓疱性毛囊炎.患者男,20岁.躯干、四肢出现毛囊性丘疹、脱屑1个月.皮肤科检查颈部、躯干及四肢弥漫红色及暗红色斑片,其上密集分布粟粒大毛囊性丘疹,部分丘疹顶端可见针尖大脓疱.血常规及骨髓检查均示嗜酸性粒细胞增多.皮损组织病理检查示表皮角化过度,棘细胞层不规则增厚,毛囊及真皮内有大量以嗜酸性粒细胞为主的炎性细胞浸润,可见毛囊内嗜酸性粒细胞小脓肿.     

显著角化过度型汗孔角化症1例

患者男，67岁。周身起丘疹、斑块28年。家族中其父亲及次子有同样皮损。皮疹呈环形或不规则形隆起的褐色角化斑，中央平坦，边缘呈堤状，部分皮损中央见角化性疣状增生及血痂。组织病理学符合汗孔诊断角化症。     

毛囊闭锁性三联征一例及家系分析

目的 探讨毛囊闭锁性三联征的临床和遗传学特点。方法 对1例毛囊闭锁性三联征继发鳞状细胞癌的特殊病例,在临床检查的基础上,进行了家系调查和系谱分析。结果 此家系共有13例,于20岁左右发病,先证者临床特点及实验室检查符合毛囊闭锁性三联征的诊断。结论 遗传因素是不可忽视的主要因素,本病可能为常染色体显性遗传病。     

Follicular Psoriasis: Differentiation from Pityriasis Rubra Pilaris—An Illustrative Case and Review of the Literature

The follicular presentation of psoriasis is a well‐described but uncommon variant. In some cases, follicular psoriasis may clinically and histopathologically mimic pityriasis rubra pilaris. There are several reports discussing the resemblance of widespread follicular psoriasis in children to pityriasis rubra pilaris. We describe a case of follicular psoriasis in a 16‐year‐old black girl with acrally distributed follicular hyperkeratotic papules with associated keratoderma of her plantar surfaces resembling pityriasis rubra pilaris.     

Folliculotropic T-cell lymphocytosis (mucin-poor follicular mucinosis)

A 48-year-old man presented with multiple asymptomatic patches of hair loss over his trunk and limbs associated with focal keratotic follicular plugs. Multiple skin biopsies showed a panfollicular lymphocytic infiltrate associated with follicular hyperkeratinization, minimal follicular spongiosis, focal basaloid follicular hyperplasia but no overt follicular mucinosis. The lymphocytes were small and there was no atypia. Immunoperoxidase stains showed that the follicular lymphocytes were T cells and predominantly CD4 positive with HLADr (LN3) expressed on their surface. There were insufficient clinical or histopathological features to make a diagnosis of folliculotropic T-cell lymphoma. This case currently may be classified best as folliculotropic T-cell lymphocytosis and may represent a mucin-poor counterpart of follicular mucinosis. Such cases may pursue an indolent course or may evolve to folliculotropic T-cell lymphoma, mycosis fungoides or anaplastic lymphoma. The term folliculotropic T-cell lymphocytosis may be useful for similar cases lacking clinical or histological criteria for lymphoma and lacking follicular mucinosis.     

Multiple tumors of follicular infundibulum with sweat duct differentiation

A 67-year-old man is reported with multiple tumors of follicular infundibulum containing ducts. Approximately 30 hypopigmented, scaling macules and minimally elevated papules were present on the face. Skin biopsy specimens from 5 representative lesions revealed similar findings. There was a proliferation of ramifying strands of pale-staining keratinocytes in the upper dermis showing connections with follicular infundibula of vellus follicles and epidermis. There was evidence of hair follicle differentiation with small follicular bulbs, papillary mesenchymal bodies, keratocysts, and occasional hair shafts in the tumor. These findings are characteristic of prior reports of TFI. Ducts were also present within the epithelial cords. Carcinoembryonic antigen, gross cystic disease fluid protein-15, epithelial membrane antigen, and S-100 protein were identified within the tumor. We theorize that the ductal elements within these TFI reflect the multipotential differentiating capacity of portions of infundibular epithelium.
Horn TD, Vennos EM, Bernstein BD, Cooper PH. Multiple tumors of follicular infundibulum with sweat duct differentiation.     

Basaloid folliculolymphoid hyperplasia: a distinctive finding in follicular mycosis fungoides

Follicular mycosis fungoides is an increasingly recognized subtype of mycosis fungoides. There is great variability in the clinical and histologic presentation of this variant of mycosis fungoides. Epithelial hyperplasia may occur in cutaneous lymphomas, and basaloid induction of the epidermis is a recognized phenomenon in cutaneous neoplasms. We have identified three patients with follicular mycosis fungoides with basaloid folliculolymphoid hyperplasia, a finding that has received little attention in the literature. The patients were all men with prominent follicular papules on the face. We believe that these changes are a distinctive histologic pattern of follicular mycosis fungoides. Hence, we describe the clinical-pathologic findings of basaloid folliculolymphoid hyperplasia in these three patients with follicular mycosis fungoides.     

Trichostasis spinulosa: itchy follicular papules in young adults

Trichostasis spinulosa is a common inapparent follicular disorder of retained bundles of vellus hairs. There exist two variants: the classical variant presenting with a non-itching, comedo-like lesion on the face in the elderly, and the pruritic variant presenting with itching, follicular papules located on the limbs in young adults. Here we present the microscopic study of the pruritic variant of trichostasis spinulosa in two patients and provide a review of the literature.     

Follicular Mucinosis in a Male Adolescent with a History of Acute Myelogenous Leukemia and Graft‐versus‐Host Disease

Although many cases of follicular mucinosis are idiopathic, numerous others are associated with mycosis fungoides or, rarely, other neoplastic or inflammatory disorders. There are only three reported cases, all in adults, of follicular mucinosis arising in association with acute myelogenous leukemia, two of which involved mycosis fungoides–associated follicular mucinosis, including one case in which the patient had a preceding bone marrow transplant. We present the first reported case of follicular mucinosis arising in an adolescent with acute myelogenous leukemia and acute graft‐versus‐host disease after an allogeneic bone marrow transplantation.     

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.     

Benign keratosis with a spectrum of follicular differentiation: a case series and investigation of a potential role of human papilloma virus

BACKGROUND: A variety of dermatopathologic entities are histologically defined by the presence of follicular differentiation. Follicular differentiation confined to the epidermis may follow induction from dermal mesenchymal proliferations, as in a nevus sebaceus of Jadassohn, or represent endogenous proliferations such as the tumor of the follicular infundibulum or trichilemmoma. METHODS: We report on five cases of a histologically distinct form of benign keratosis showing variable follicular differentiation. Clinicopathologic correlation and analysis of a potential human papilloma virus pathogenesis was investigated. RESULTS: Each of the cases arose on the trunk or extremities of three men and two women with a mean age at presentation of 66.6 years. All of the lesions showed variable follicular differentiation, with germinative basaloid cells, matrical cells with matrical keratinization, inner root sheath with trichohyalin granules, or glycogenated lower outer root sheath. Immunohistochemical staining for human papilloma virus was negative in each case. CONCLUSIONS: There exists a distinct entity, histologically defined as a keratosis with variable follicular differentiation, which has not been previously described. These lesions do not appear to be pathogenically related to human papilloma virus infection.