获得性指状纤维角化瘤7例临床分析

目的:加深对获得性指状纤维角化瘤临床及组织病理特点的认识.方法:回顾性分析2008-2011年间我科确诊为获得性指状纤维角化瘤患者的临床表现、组织病理特点和治疗反应.结果:7例患者平均发病年龄(35.6±10.1)岁(26～52岁),平均病程(3.3±4.0)年(0.5～10年),男女比例1∶1.3.皮损表现为单发、大小不等、肤色的增生性肿物,呈圆顶状或柱状.主要累及指、趾部,也可见于手掌及甲沟等部位.组织病理学检查:表皮角化过度,颗粒层增厚,棘层不规则肥厚,表皮突增宽、延长;真皮乳头大量扩张毛细血管及粗大纤维增生,与真皮乳头平行.结论:获得性指状纤维角化瘤为单发增生性肿物,常发生在指、趾部,也可累及肢端其他部位.皮损组织病理检查有特征性改变,切除可治愈.     

获得性肢端纤维角皮瘤七例临床分析

回顾性分析2016-2019年间我科确诊为获得性肢端纤维角皮瘤的7例患者，平均发病年龄（30.25±15.17）岁，平均病程（4.15±5.12）年，男女比例4∶3。皮损表现为发生于肢端的无症状的单发丘疹状、柱状或角化斑块状增生物、直径多＜1 cm，呈正常皮色或黄褐色。组织病理学特征性表现为胶原束与表皮垂直。皮肤镜特征因皮损部位、生长模式、胶原及毛细血管形成的不同而表现不同。     

获得性指(趾)部纤维角化瘤

患者男,76岁,因右足拇趾内侧皮疹10年余来我科就诊.10年前患者感觉右足拇趾内侧皮肤肥厚,逐渐向内侧缓慢生长,无痛痒等不适,近半年来行走时感觉疼痛.既往健康,家族中无遗传病及类似病史.体检:一般情况可,各系统检查未见明显异常.皮肤科情况:右足拇趾内侧结节,约2.7 cm×1.5 cm × 0.9 cm,肤色,表面光滑,质地较硬(图1).予以外科手术切除.结节组织病理检查:表皮角化过度,棘层肥厚,表皮突延伸、增宽;真皮可见大量增生的胶原纤维及成纤维细胞,血管增生、扩张(图2,3).根据患者临床表现和皮损组织病理检查,诊断:获得性指(趾)部纤维角化瘤.     

跖部获得性指（趾）状纤维角皮瘤一例

报告1例获得性指（趾）状纤维角皮瘤。患者男,21岁,右跖部多枚角化性肤色丘疹15年,无瘙痒、疼痛,渐增多、增大。查体示：右跖部至少15枚大小不一的肤色角化性丘疹,集聚成斑块状,三角尖样突起,跖面扁平、质软,突起边缘明显角化、透明状,每一丘疹背面,质硬、光滑,甲周纤维瘤样外观。手术切除。组织病理示：表皮角化过度,棘层肥厚,两丘疹间的乳头分枝状;真皮大量致密、增宽的成熟胶原纤维束,与皮疹长轴平行,间有少量成纤维细胞,未见血管结构,形成皮损的中心部分;皮下组织正常;未见空疱化细胞。     

婴儿指部纤维瘤病

报告1例婴儿指部纤维瘤病.患儿男,21个月.左手无名指和小指末节出现结节,皮肤萎缩20个月余.系统检查无异常.皮损组织病理检查:表皮角化过度伴角化不全,棘层肥厚,表皮突延长、增宽.真皮成纤维细胞及胶原增生,胞质内可见嗜伊红包涵体.Masson染色胞质中可见圆形深紫色的包涵体.结合临床诊断为婴儿指部纤维瘤病.     

疣状肢端角化症的临床及病理特点研究

目的：探讨疣状肢端角化症的临床及病理特点。方法：分析本文及近40年来13篇文献报道的17例肢端角化症先证者的临床及病理资料。结果：发病年龄，10例（59％）〈20岁，7例（41％）〉25岁；有家族史者10例（59％）。皮损主要分布于双手背、足背及前臂、小腿伸侧。皮损表现16例（94％）为扁平疣样损害，1例表现为寻常疣样损害，6例（35％）伴有掌跖角化，5例（29％）伴有指趾甲受累，3例（18％）伴有毛囊角化病。皮损组织病理均表现为表皮明显角化过度、颗粒层及棘层增厚、乳头瘤样增生及塔尖样表皮局限性隆起等特征性变化，无角化不全及空泡化细胞。结论：疣状肢端角化症大部分患者有家族史，常于加岁前发病。皮损主要分布四肢伸侧，基本皮疹为扁平疣样的褐色扁平丘疹。皮损病理显示表皮明显角化过度、乳头瘤样增生及塔尖样表皮局限性隆起等特征性变化，是诊断疣状肢端角化症的主要依据。     

获得性肢端纤维角皮瘤一例

52岁女性患者,右手示指丘疹逐渐增大1年余。皮肤科情况:右手示指末端指间关节指腹侧单发丘疹,半球形,黄豆大小,肤色,隆起于皮面,表面无鳞屑,触之较硬、无压痛。组织病理示:表皮角化过度,棘层肥厚,表皮突增宽下延。真皮中央为致密的胶原纤维束,周围有血管包绕,血管垂直于表皮,诊断:获得性肢端纤维角皮瘤。     

对称性肢端角化病62例临床分析

目的 分析对称性肢端角化病患者的临床表现、人口学资料、组织病理学特点。方法 收集2003年5月以来在我院门诊就诊的对称性肢端角化病患者62例,详细了解其人口学资料及家族史,并进行全身体格检查,部分患者行皮损组织病理检查。结果 62例患者中男55例,女7例,发病年龄4 ～ 53岁,平均年龄24.02岁;病程15 d至10年,平均为26.65个月;主要临床表现为对称性褐色角化性斑片,皮损接触水后变白,主要分布在指背、手背、手腕、掌侧缘、踝部、膝部、肘部,冬季自然缓解;真菌检查阴性;组织病理学表现为表皮角化过度,棘层增厚,轻度乳头瘤样增生,真皮少量淋巴细胞。结论 对称性肢端角化病以对称性肢端角化为主要临床表现,伴有明显季节性变化。     

获得性对称性肢端角化病1例

患者男,38岁。双手、足起褐色角化性斑片5年。皮损特点为双手及双足背部褐色角化性斑片,接触水后局部变白,冬季明显缓解。患者有鱼鳞病史。父母非近亲结婚,也均有鱼鳞病史。皮损组织病理示:表皮角化过度,棘层增生,真皮浅层血管周围淋巴细胞浸润。诊断:获得性对称性肢端角化病。     

对称性肢端角化病九例分析

目的 探讨9例对称性肢端角化病患者的相关危险因素、临床表现、治疗及疗效.方法 门诊收集9例对称性肢端角化病患者的临床资料.对皮损进行拍照,真菌镜检、组织病理检查.予外用药治疗并随访.结果 男8例,女1例,平均年龄28.2岁,平均发病年龄24.6岁,病程2个月至16年；皮损对称分布,双手背、腕全部累及,踝次之；手腕部皮损分布呈圈状；夏季重、冬秋季多数自愈；大部分患者与塑胶或橡胶接触有关,部分合并寻常性鱼鳞病；真菌镜检均阴性,组织病理学表现为表皮角化过度,棘层肥厚,轻微乳头瘤样增生；尿素维E乳膏、丙酸氯倍他索乳膏按1∶1调用明显有效.结论 寻常性鱼鳞病患者存在对称性肢端角化病的易患因素,遇水变白可能是水、空气参与的一种物理现象.外用糖皮质激素可作为第一线药物选择.     

22例甲下黑素瘤临床及组织病理学分析

目的:探讨甲下黑素瘤的临床及组织病理学特点。方法:回顾分析西京医院皮肤科2003—2009年间诊断的22例甲下黑素瘤患者临床及组织病理学特点。结果:全部患者均为成人,其中男9例,女13例。发生于拇指9例,示指5例,发生于第一趾4例,发生于其他指(趾)4例。16例出现甲周皮肤颜色变黑,10例表现为甲板毁损或溃疡、结节性损害,6例为单纯甲黑线或黑甲。组织病理资料分析结果表明10例为原位黑素瘤,12例为浸润性黑素瘤。结论:甲下黑素瘤好发于成年人,尤其是中老年患者,拇指、示指以及第一趾是其好发部位。早期甲下黑素瘤表现为皮损较宽、颜色不均的甲黑线或弥漫性黑甲,晚期甲下黑素瘤累及甲周皮肤,或形成结节溃疡性损害。甲下原位黑素瘤表现为甲母质或甲床部位单个黑素细胞增生,黑素细胞位置较高(基底层以上),核染色质深,细胞树突明显,合成颗粒较粗大的黑素。甲下浸润性黑素瘤累及真皮内,呈浸润性生长,细胞异形性明显。     

Clinical and histopathological characters of 22 cases of subungual melanoma北大核心

Objective: To analyze the clinical and histological characters of subungual melanoma. Methods: Retrospective analysis of 22 cases of subungual melanoma was made at the Department of Dermatology, Xijing Hospital from 2003 to 2009. Results: The clinical data showed all patients were adult (9 males, 13 females). Nine lesions were located on thumbs, 5 on index fingers, 4 on the first toes and 4 distributed on other fingers or toes. Sixteen cases showed darkening of skin around nail, 10 damage of nail plate, nodules or ulceration, 6 cases presented as melanonychia. Histological data showed 10 cases were melanoma in situ and 12 invasive melanoma. Conclusion: Subungual melanoma always appears in adults, especially in the senile. Thumbs, index fingers and the first toes were the most predilection sites. Its early stage always presented as broad melanonychia with uneven color. Its late stage always involves skin around the nail, or forms nodules or ulceration. Subungual melanoma in situ showed proliferations of single cells distributed at very high level in matrix or nail bed. The neoplasm cells always show dense chromatin, prominent dendrites and contained coarse pigment. Subungal invasive melanoma involves dermis and shows infiltrative growth pattern, maturation of melanocytes is not existed and cellular polymorphism is prominent.     

Electronmicroscopic Observation of the Basement Membrane Zone in Focal Dermal Hypoplasia

A 3-month-old boy had reticulated achromic and atrophic skin lesions on the right buttock and thigh, as well as partial syndactyly of the third and fourth toes of the right foot. With hematoxylin and eosin stain, an epidermal defect, abnormally located fat cell lobules, and absence of the upper and midportions of the dermis were observed, conforming to a typical histopathologic picture of focal dermal hypoplasia. Electron microscopic examination of the atrophic site showed loose collagen bundles, collagen fibers with loss of regular bands, abnormal fibroblasts, and disruption of the basement membrane zone. These defects in the basement membrane zone strongly suggest that abnormal formation of type IV collagen is associated with focal dermal hypoplasia, and that this abnormal formation of collagen is correlated with the clinical sign of skin atrophy.     

先天性厚甲症伴多发性皮角1例

患者男,18岁。甲及掌跖部皮损18年。皮损特征表现为:20甲高度增厚、掌跖部严重角化、毛囊角化,尤以肘膝部明显、乳头角化及面部多发性皮角样损害。皮肤活检示:表皮高度角化过度及灶性角化不全,真皮少量炎症细胞浸润。诊断:先天性厚甲症;多发性皮角。     

Recurring digital fibromas of infancy

Recurring digital fibromas are distinguished by their occurrence in infants, their almost exclusive predilection for the fingers and toes, their tendency to recur and their characteristic inclusion bodies. At the Royal Alexandra Hospital for Children (RAHC) thirty cases were seen in thirty-six years. The patients were twenty females and ten males, aged 0–7 years, including two with congenital lesions. The fingers and toes were equally affected, and one unusual case involved the side of the hand. Multiple lesions occurred in 50% of patients, more often on the fingers, especially on adjacent fingers (six cases) and two patients had bilateral lesions. Although some lesions had infiltrated down to the periosteum, no metastases occurred in our series, or in the cases in the literature. As growth rate may be alarming, some of these tumours have been treated aggressively, but evidence suggests that a conservative approach should be followed.     

Ganglion of the distal interphalangeal joint (myxoid cyst): therapy by identification and repair of the leak of joint fluid

BACKGROUND: Digital myxoid cysts are a relatively common pathology in the skin, representing a ganglion of the adjacent distal interphalangeal joint. Success of treatment is largely proportional to the destructiveness of the therapy and postoperative morbidity. We studied an effective, minimally traumatic surgical treatment in which tissue is not removed and morbidity is low. DESIGN: Open, nonrandomized trial of therapy. Methylene blue dye was injected into the distal interphalangeal joint. A skin flap was designed around the cyst and raised to identify the dye-filled communication between joint and cyst. The communication was sutured and the flap was replaced with no tissue excision. SETTING: Two university dermatology departments. PATIENTS: Fifty-four subjects with 47 cysts involving fingers or thumbs and 7 involving toes. Previous therapies in 37 patients had resulted in relapse. MAIN OUTCOME MEASURES: Clinical assessment at 2 and 8 months. RESULTS: We treated 34 women and 20 men (mean age, 60.4 years; range, 45-83 years). Communication between cyst and joint was identified by means of methylene blue injection in 48 patients (89%). At 8 months, 48 patients remained cured with no visible scarring. Of these, nail dystrophy associated with the cyst preoperatively (n = 35) resolved in all but 1 patient (97%). Six patients had relapses (5 within 4 months). Of these, 3 were on the toes. Cure rate on toes was 4 of 7 (57%) and on fingers, 44 of 47 (94%). In 2 patients, pain persisted for 4 months and then resolved. Limitation of joint mobility resolved after 2 months in 1 subject. CONCLUSIONS: Ligature of myxoid cyst origin at the joint capsule is an effective treatment and does not require excision. Myxoid cysts on toes are more likely to relapse than those on fingers.     

Subtotal C4 deficiency and SLE-like disease

Extremely low C4 values were found in a 65-year-old man with relapsing arthritis and skin lesions of many years duration of the scalp, face, hands and feet together with painful ulcerations of the toes and fingers. The discovery was made during an exacerbation, but the deficiency of C4 persisted in repeated controls after remission. The clinical findings in connection with these low C4 values are in congruence with the diagnosis of inherited deficiency of C4.     

A case of sclerodermatous graft-versus-host disease following autologous peripheral blood stem cell transplantation

We report a 65-year-old woman with chronic graft-versus-host disease (GVHD) who developed severely sclerotic skin on the fingers, hand and trunk following autologous peripheral blood stem cell transplantation (APBSCT). The patient had suffered from breast cancer and been treated with surgery and chemotherapy. She showed pancytopenia and was treated with APBCST. Four years after APBSCT, she developed the severe sclerotic changes on the fingers, hands, extremities and trunk. The skin biopsy showed a flattened epidermis and a proliferation of collagen bundles in the dermis. No anti-nucleolar DNA titers were detected in the serum. She was diagnosed with chronic GVHD. Despite treatment with oral prednisolone, the skin sclerotic change developed and the breast cancer recurred. She died due to pericarditis. This is a rare case of sclerodermatous GVHD following APBSCT. The serum interleukin (IL)-12 levels were examined during the treatment.     

Verrucous form of chilblain lupus erythematosus

A 45-year-old woman had symmetrical livid plaques with yellowish hyperkeratoses for 5 years, which progressed on to the fingers and toes and on the soles of the feet. Two years later creamy, whitish areas and maceration appeared on the buccal mucosa and the lips. A skin biopsy revealed massive collagen hyaline degeneration in the perivascular area, hyperkeratosis and hypergranulosis, small lymphocyte infiltrates with several melanophages and extravasates of erythrocytes in the upper corium in perivascular areas and hydropic degeneration of basal keratinocytes. The findings using direct immunofluorescence were compatible with lupus erythematosus (LE). Laboratory investigation showed a slight leucopenia and thrombopenia, a slightly elevated erythrocyte sedimentation rate, hypocomplementaemia C3 and C4, a high titre of rheumatoid factor and antinuclear antibodies positivity of extractable nuclear antigen. The results reflected probably the development of a systemic form of the disease. The patient was successfully managed by methylprednisolone and hydroxychloroquine. After 1 year of therapy, a new skin biopsy revealed a substantial reduction of hyperkeratosis and hyaline degeneration of collagen tissue in the perivascular areas. The combination of the extensive hyperkeratosis and hyalinization thus seems to be features of the long-lasting, untreated lesions in chilblain LE.     

ULTRASTRUCTURAL STUDIES ON PIGMENTED MACULES OF PEUTZ-JEGHERS SYNDROME

Light and electron microscopic studies were carried out on pigmented macules on the lips, fingers and toes of the patients with Peutz-Jeghers syndrome. Our studies indicated basal pigmentation was noticed in the basal cell layer of the epidermis, but no differences were recognized between the lesions and normal skin in either the number of melanocyte or the length of the dendrite of melanocyte in the lips. In fingers and toes, few melanin granules or melanosomes could be found in the basal cells. Numerous dendrites of melanocyte filled with melanin granules or melanosomes were seen in the intercellular spaces of the keratinocytes. The dendrites of melanocytes in the lesions were much longer and more branched than those in the normal skin. However, the population of melanocytes in the both areas was almost identical. Some melanosomes in the dendrites of the upper epidermis seemed to be degraded and exhibited a positive acid phosphatase reaction. We may assume that melanosome transfer from melanocytes or melanosome receptivity of the keratinocytes remarkably decreased in the lesions on the fingers and toes of Peutz-Jeghers syndrome and then melanosomes had accumulated in the dendrites and some of them are being degraded.