Primary essential cutis verticis gyrata in an adult female patient: a case report

Cutis verticis gyrata (CVG) is a very rare morphological condition of the scalp characterized by ridges and furrows resembling the surface of the brain. Primary or idiopathic CVG occurs in the absence of underlying soft-tissue abnormalities and is often associated with neuropsychiatric disorders. Secondary CVG occurs as a result of a local inflammatory or neoplastic process of the scalp or a systemic illness that produce pathologic changes in the scalp structure. The choice of treatment of CVG is surgical repair which depends on the size and location of the lesion, the underlying disorder, and the wishes of the patient, including primary repair, serial excision, skin grafting, local flaps and tissue expansion. In this case report, we describe the first female patient in the published work with primary essential CVG that appeared at 30 years of age. Because the patient had no cosmetic or functional complaint, no surgical intervention was attempted. Primary essential CVG, a very uncommon disorder, may be encountered in females after the third decade. The classification of CVG is essential to properly diagnose and treat patients who present with these unusual scalp lesions.     

Cerebriform intradermal naevus (a rare form of secondary cutis verticis gyrata)

BACKGROUND: Acquired cerebriform intradermal naevus (CIN) is a rare form of pseudo cutis verticis gyrata. CASE REPORT: A case of acquired CIN of the scalp in a 46-year-old male patient is presented. The clinical and histopathological presentations of CIN are described and the therapeutic possibilities are discussed. CONCLUSIONS: In each individual case the physician must decide whether to do surgery or follow a wait-and-see policy. There is little risk of malignant change of CIN, and surgical excision of such lesions often involves evident mutilation.     

Cutis verticis gyrata secondary to acne scleroticans capitis

We report the case of a 35-year-old patient with secondary cutis verticis gyrata (CVG) that histologically presented as acne scleroticans capitis. Clinically, the diagnosis of acne conglobata was made. The CVG developed under systemic corticosteroid treatment of an ulcerative colitis. Corticosteroids were discontinued and a therapeutic attempt was made with isotretinoin. Although under this treatment the conglobate acne healed, the CVG remained unaltered. Dermatosurgical intervention by a scalp reduction plasty finally resulted in a marked improvement of symptoms and acceptable cosmetic outcome.     

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.     

色素异常性皮肤淀粉样变病一家系报道

患者,男,61岁。全身皮肤出现色素沉着及减退斑10年余。其父、兄与其有相同的皮疹特征。皮损组织病理示：表皮角化过度,部分上皮脚融合,棘层轻度增生,真皮浅层小血管可见淋巴细胞浸润,部分真皮乳头层可见嗜伊红团块状物质,其间可见裂隙。真皮乳头层团块状物质刚果红染色阳性。结合临床及实验室检查,诊断为色素异常性皮肤淀粉样变病。     

以消化道症状为主要表现的原发性系统性淀粉样变病1例

患者男,45岁。纳差8个月,反复腹泻1个月。患者以消化道症状为首发症状,进一步检查发现双上眼睑及四肢见片状紫癜,巨舌,并累及心脏、喉部、心包、胸膜、腹膜。结合直肠组织病理学表现黏膜下层伊红染无定形物沉积,甲基紫、刚果红染色阳性,尿κ、λ链异常。诊断:原发性系统性淀粉样变病。     

原发性系统性淀粉样变病1例

报告1例原发性系统性淀粉样变病。患者男，39岁。1年前肩关节突然出现酸痛，症状渐渐加重，双手掌皮肤发硬，肢体上抬及下蹲困难，舌不能外伸，吞咽时有梗阻感。曾经患有胸椎椎体骨髓瘤。皮肤科检查示面颈部、双侧腋下、腹部、阴茎、双大腿内侧泛发对称性的蜡样半球形丘疹，有融合，呈肤色或琥珀色。口腔黏膜肥厚，舌弥漫性浸润、质硬，呈巨舌。皮损组织病理检查示表皮萎缩，真皮与皮下组织淀粉样物质沉积，甲基紫染色阳性，诊断为原发性系统性淀粉样变病。给予泼尼松和雷公藤治疗，症状明显改善。     

新生儿皮肤再生不良1例

报告1例新生儿皮肤再生不良,患儿男,出生1h,生后即被发现左上肢皮肤缺损,分娩时无外伤史,给予0.1%利凡诺溶液及莫匹罗星软膏外用,10d后缺损处被新生组织填充,表面光滑,略凹陷,颜色较周围正常皮肤暗。     

原发性皮肤毛霉病

报告1例原发性皮肤毛霉病.患者男,57岁.右前臂埋置静脉套管针17 d后局部出现红肿,迅速坏死并扩大.患者患2型糖尿病及慢性肾功能不全.皮肤科检查示右前臂大片坏死区,上覆黑色焦痂及渗出.取黑痂及渗液行真菌镜检,镜下可见大量粗大、无分隔、垂直分枝的菌丝,培养第2天即有棉花糖样菌落生长,初步诊断为皮肤毛霉病.皮损行组织病理检查,PAS染色示真皮及皮下组织可见较多粗大、无分隔菌丝,确诊为皮肤毛霉病.给予两性霉素B脂质体泵入、坏死组织清创术、术后外敷两性霉素B脂质体溶液及外用人成纤维细胞生长因子.患者伤口愈合良好,随访4个月无复发.     

获得性局限性皮肤松弛症一例

A 24-year-old woman presented with redundant skin on her face and neck for 2 years. Physical examination revealed that the loose of skin is noticeable on her face, neck and upper eyelids, resulting in a prematurely aged appearance. Fatigue test was negative. The histopathology showed the reduction in elastic fi￣bers and the abnormally distorted shapes of elastic fibers. The diagnosis of acquired localized cutis laxa was made. © 2019 Shandong Yinbao Technology Co. Ltd. All rights reserved.     

原发性皮肤弥漫性大B细胞淋巴瘤一例

患者,男,51岁。左小腿红斑、结节、斑块伴疼痛3个月。组织病理及免疫组化确诊为原发性皮肤弥漫性大B细胞淋巴瘤,腿型。     

Frontal rhytidectomy as surgical treatment for pachydermoperiostosis: a case report

The thickened folded skin of Touraine-Solente-Golé syndrome can result in cosmetic and functional deformities. The treatment of pachydermoperiostosis is usually centered around improving the cosmetic appearance through plastic surgery. We describe the case of a 27-year-old male who had pachydermoperiostosis with a leonine facies that was managed with frontal rhytidectomy. A greatly improved cosmetic appearance was achieved with this procedure.     

原发于头皮的皮肤淀粉样变一例

原发性皮肤淀粉样变皮损常见于双侧胫前、背部及大腿,也可见于阴茎及面颈部,原发于头皮者少见,诊治具有一定的难度。现报道1例我科诊治原发于头皮的皮肤淀粉样变患者。给予0.1%维A酸外用头皮,治疗2个月,皮损变化不明显,瘙痒缓解,目前进一步随访中。     

Primary osteoma cutis

A 13-month-old male infant with primary osteoma cutis was reported. There was no diagnostic sign of Albright's hereditary osteodystrophy in either this patient or his family. Laboratory investigations showed no abnormalities except moderate obesity (kaup index=22).     

原发性皮肤边缘区B细胞淋巴瘤

患者男,30岁.背部散在红色结节3年余.皮损组织病理检查:真皮内淋巴细胞样细胞呈片状岛屿状浸润增生,部分细胞可见异形性,表真皮间可见无浸润带,表皮大致正常.免疫组化结果:中心细胞样小淋巴细胞Bcl-2、CD20、CD79a、mum-1(++),Kap-pa:Lambda＞10;CD5、CD10、Bc1-6(-);结节周...     

急性髓细胞性白血病(M4型)

报告1例急性髓细胞性皮肤白血病(M4型).患者女,48岁.全身出现丘疹、红色结节14d,伴剧烈瘙痒.体格检查:全身泛发大小不等的红色丘疹、结节,质韧,无压痛.皮损组织病理检查:真皮内弥漫淋巴样细胞浸润,有明显异形及较多核分裂象.免疫组化组织病理检查:CD68阳性(灶性),MPO阳性(少量).骨髓穿刺:白血病细胞大量增生,免疫标记:CD68、CD11b、MPO及HLA-DR均阳性.诊断:急性髓细胞性白血病(M4型).患者经过2次DA(伊达比星、阿糖胞苷)方案化疗后,再次行骨髓穿刺示缓解,但皮损仍有复发.     

Asymmetrical nonscalp aplasia cutis congenita: a case report

Aplasia cutis congenita or congenital absence of the skin is a rare and serious congenital disease; its etiopathogenesis remains unclear. In this condition, localized or widespread areas of skin are absent at birth. A newborn suffering from an unusual aplasia cutis congenita located asymmetrically on the nonscalp, without blistering, was presented. This patient was completely healed with conservative treatment.     

皮肤弹性过度伴免疫学检查异常1例

报告皮肤弹性过度伴细胞免疫缺陷1例。患儿女，13岁。出生后即有皮肤弹性过度，脓疱疮反复发作4年。实验室检查发现有缺铁性贫血，皮损组织病理检查无特异性，脓液细菌培养出金黄色葡萄球菌，淋巴细胞亚群检查CD3 32%，CD427%，CD8 20%，CD4/CD8 1.35。先后给予免疫调节剂，系统及外月抗生素，抗贫血药等治疗。患儿细胞免疫缺陷得到纠正，深脓疱疮及缺铁性贫血治愈。     

先天性头皮发育不全一例

患儿,男,出生后头顶部皮肤可见一处类圆形缺损。诊断为先天性皮肤发育不全(ACC),ACC是一种以局部或广泛皮肤缺损为主要临床表现的先天性疾病,可累及身体的任何部位,最常见于头皮,发病机制尚不明确,可以独立发病或伴随其他遗传性疾病。目前ACC治疗方法主要包括保守治疗和手术治疗,但存在争议。