Zeng Weiliang, Guo Lili
Department of Plastic Surgery, The First Affiliated Hospital of Zhengzhou University, 450052, Zhengzhou, China.
Scar Research Center, The First Affiliated Hospital of Zhengzhou University, 450052, Zhengzhou, China.
BMC Surg. 2021 May 4;21(1):234. doi: 10.1186/s12893-021-01229-9.
Cutis verticis gyrate (CVG) is a rare morphologic syndrome that presents with hypertrophy and folding of the scalp. CVG can be classified into three forms: primary essential, primary non-essential, and secondary. Cerebriform intradermal nevus (CIN) is a rare cause of secondary CVG. We are here to report a rare case of CVG with an underlying CIN and discuss the clinical course, treatment options, and critical screening guidelines for these patients.
A 25 year-old male patient presented with a chief complaint of generalized hair loss in the scalp parietaloccipital region for a duration of 1 year and the hair loss area was occasionally accompanied by mild itching. The hair loss started gradually and worsened over time. In addition, he had scalp skin folds resembling the ridge and furrow of the cerebral cortex in the parietaloccipital region since birth. Physical examination revealed hypertrophy and formation of folds in the parietal-occipital area, forming 5 to 6 furrows and ridges. The size of the cerebriform mass was about 12.0 cm × 8.5 cm, without other skin lesions. Diffuse non-scarring hair loss was distributed on the posterior-parietal scalp, mid-parietal scalp and superior-occipital scalp. The diseased tissue of the patient's parietaloccipital area was excised under general anesthesia. The postoperative pathological examination of the tissue excised showed that there were dense intradermal melanocytic nevus, so the patient was diagnosed with secondary CVG caused by CIN. At the 2 year follow-up, there were no obvious changes in the lesions.
CIN must be differentiated from other conditions that manifest as CVG, including primary essential or non-essential CVG and secondary CVG caused by other reasons. Each CIN patient requires a specific decision of whether to excise the lesion surgically or follow a wait-and-see policy, depending on the patient's will and specific condition. Surgical treatment may be performed when there is an aesthetic demand. However, clinical observation and close follow-up is also a good treatment choice for patients with stable disease or mild symptoms.
回旋状头皮（CVG）是一种罕见的形态学综合征，表现为头皮肥厚和褶皱。CVG可分为三种类型：原发性本质性、原发性非本质性和继发性。脑回状真皮内痣（CIN）是继发性CVG的罕见病因。我们在此报告一例罕见的伴有潜在CIN的CVG病例，并讨论这些患者的临床病程、治疗选择和关键筛查指南。
一名25岁男性患者，主要主诉为头皮顶枕部广泛性脱发1年，脱发区偶尔伴有轻度瘙痒。脱发起病逐渐，随时间加重。此外，自出生以来，他在顶枕部有类似大脑皮质沟回的头皮皮肤褶皱。体格检查发现顶枕部肥厚并形成褶皱，形成5至6条沟和嵴。脑回状肿物大小约为12.0 cm×8.5 cm，无其他皮肤病变。弥漫性非瘢痕性脱发分布于顶后部头皮、顶中部头皮和枕上部头皮。患者顶枕部病变组织在全身麻醉下切除。切除组织的术后病理检查显示有密集的真皮内黑素细胞痣，因此患者被诊断为由CIN引起的继发性CVG。在2年的随访中，病变无明显变化。
CIN必须与表现为CVG的其他疾病相鉴别，包括原发性本质性或非本质性CVG以及由其他原因引起的继发性CVG。每个CIN患者都需要根据患者的意愿和具体情况，就是否手术切除病变或采取观望政策做出具体决定。当有美学需求时可进行手术治疗。然而，对于病情稳定或症状轻微的患者，临床观察和密切随访也是一种不错的治疗选择。
