Department of Dermatology, University of California, 1701 Divisadero St, 3rd Floor, Box 0316, San Francisco, CA 94115, USA.
Br J Dermatol. 2010 Jun;162(6):1337-41. doi: 10.1111/j.1365-2133.2010.09702.x. Epub 2010 Feb 15.
There is little published information about segmental hypo- and hyperpigmentation pigmentation disorder (SegPD) although it is a relatively common problem in paediatric dermatology.
To define the spectrum of disease, clinical presentation and associations in cases of SegPD and to clarify further the terminology in defining patterned hypo- and hyperpigmentation in children.
This was a retrospective review of cases in an academic paediatric dermatology practice. Thirty-nine patients referred for dermatological evaluation were diagnosed with SegPD. Demographic and clinical features, and distribution and frequency of extracutaneous abnormalities were measured.
Twenty female and 19 male patients were included in the study; 33 out of the 39 were referred specifically for a pigmentation abnormality. The mean age at onset was 3·4 months (median age 0·25 months). Family history was positive in two patients. Most (30/39; 77%) had segmental hyperpigmentation whereas nine of 39 (23%) had hypopigmentation. Patches were more often delineated at the ventral midline (32/39) than on the dorsal midline (7/39). The distribution of lesions was as follows: areas of the torso were most often affected (77%) and when the face, neck, arms and legs were affected pigmentation usually extended onto the torso; six patients had SegPD localized to the face. Only three of the 39 patients had extracutaneous abnormalities - atrial septal defect, strabismus with retinal hypopigmentation and a bronchogenic cyst - but the relationship to SegPD was uncertain and none had neurological abnormalities.
SegPD is a relatively common pigmentary anomaly and most affected individuals are otherwise healthy. We propose reviving the term 'segmental pigmentation disorder' coined by Metzker and colleagues to describe children with segmental and block-like hypo-/hyperpigmentation with midline demarcation.
尽管节段性色素减退和色素沉着障碍（SegPD）在儿科皮肤病学中相对常见，但有关其的文献却很少。
定义 SegPD 的疾病谱、临床表现和相关因素，并进一步阐明儿童节段性色素减退和色素沉着的术语。
这是一项在学术性儿科皮肤科实践中的回顾性研究。对 39 例因皮肤病就诊的患者进行了 SegPD 诊断。测量了人口统计学和临床特征，以及皮肤外异常的分布和频率。
本研究纳入了 20 名女性和 19 名男性患者，其中 39 例患者因色素异常就诊。发病的平均年龄为 3.4 个月（中位数年龄为 0.25 个月）。有 2 例患者有家族史。大多数（30/39；77%）为节段性色素沉着过度，而 39 例中有 9 例（23%）为色素减退。斑片更多见于腹中线（32/39）而非背中线（7/39）。皮损分布如下：躯干区域最常受累（77%），当面部、颈部、手臂和腿部受累时，色素沉着通常延伸至躯干；6 例患者的 SegPD 局限于面部。39 例患者中只有 3 例有皮肤外异常——房间隔缺损、斜视伴视网膜色素减退和支气管囊肿，但与 SegPD 的关系不确定，也没有神经异常。
SegPD 是一种相对常见的色素异常，大多数受累个体的健康状况良好。我们建议重新使用 Metzker 等人提出的术语“节段性色素障碍”来描述具有中线分界的节段性和块状色素减退/沉着的儿童。
