中华皮肤科杂志
中華皮膚科雜誌
중화피부과잡지
Chinese Journal of Dermatology
2010年
8期
549-554
,共6页
严昉%项蕾红%李颖%潘展砚%郑志忠
嚴昉%項蕾紅%李穎%潘展硯%鄭誌忠
엄방%항뢰홍%리영%반전연%정지충
痣%色素沉着不足%黑素细胞%黑素类
痣%色素沉著不足%黑素細胞%黑素類
지%색소침착불족%흑소세포%흑소류
Nevus%Hypopigmentation%Melanocytes%Melanins
目的 探讨无色素痣的临床和组织学特征.方法 分析85例无色素痣患者的发病年龄、类型和皮损特点,并对部分患者行皮肤色素测定和反射式共聚焦显微镜(RCM)观察.对其中17例患者的皮损区和正常区皮肤组织进行组织病理检查,透射电镜观察皮损区超微结构.免疫组化法检测皮损区和正常皮肤处酪氨酸酶(TYR)、HMB45、酪氨酸酶相关蛋白1(TRP-1)、TRP-2和CD117表达.结果 85例无色素痣患者中,23例(27.1%)出生时发现皮损,21例(24.7%)出现于3岁以后,最大发病年龄为29岁.皮损分布于躯干部25例(29.4%),颈部13例(15.3%);72例(84.7%)皮损边缘不规则,54例(63.5%)仅有1处皮损.19例无色素痣患者患处的黑素指数(186.56±52.86)和相对黑素指数(80±11)低于正常人皮肤(分别为223.88±63.19和100),高于12例白癜风患者皮损处(分别为128.57±64.31和60±20),差异均具有统计学意义(P＜0.01).反射式共聚焦显微镜示,无色素痣皮损中含黑素细胞数量减少,亮度减低,黑素分布均匀,皮损区与正常皮肤分界区常不清晰.皮损区Fontana-Masson染色示皮损区黑素强度为1810.12±327.96,较正常区(2064.24±260.41)明显减弱.电镜下发现黑素细胞数量减少,黑素小体减少,黑素细胞胞质和树突以及角质形成细胞中可见Ⅱ、Ⅲ期未成熟的黑素小体,角质形成细胞中可见聚集成团的黑素小体.17例患者正常区TYR表达水平为1827.35±307.09,TRP-1为6102.54±1642.64,而皮损区TYR(1477.35±224.05)和TRP-1(5322.33±1565.26)表达下降,正常区与皮损区比较,P均＜0.01;HMB45、TRP-2、CD117表达两处比较差异均无统计学意义.结论 无色素痣是一种早期发病、非家族聚集性、稳定的不规则色素减退性疾病,其皮损中黑素细胞和黑素小体数量均减少,可见未成熟黑素小体.相对黑素指数和反射式共聚焦显微镜检查可作为诊断无色素痣的无创性检测方法.
目的 探討無色素痣的臨床和組織學特徵.方法 分析85例無色素痣患者的髮病年齡、類型和皮損特點,併對部分患者行皮膚色素測定和反射式共聚焦顯微鏡(RCM)觀察.對其中17例患者的皮損區和正常區皮膚組織進行組織病理檢查,透射電鏡觀察皮損區超微結構.免疫組化法檢測皮損區和正常皮膚處酪氨痠酶(TYR)、HMB45、酪氨痠酶相關蛋白1(TRP-1)、TRP-2和CD117錶達.結果 85例無色素痣患者中,23例(27.1%)齣生時髮現皮損,21例(24.7%)齣現于3歲以後,最大髮病年齡為29歲.皮損分佈于軀榦部25例(29.4%),頸部13例(15.3%);72例(84.7%)皮損邊緣不規則,54例(63.5%)僅有1處皮損.19例無色素痣患者患處的黑素指數(186.56±52.86)和相對黑素指數(80±11)低于正常人皮膚(分彆為223.88±63.19和100),高于12例白癜風患者皮損處(分彆為128.57±64.31和60±20),差異均具有統計學意義(P＜0.01).反射式共聚焦顯微鏡示,無色素痣皮損中含黑素細胞數量減少,亮度減低,黑素分佈均勻,皮損區與正常皮膚分界區常不清晰.皮損區Fontana-Masson染色示皮損區黑素彊度為1810.12±327.96,較正常區(2064.24±260.41)明顯減弱.電鏡下髮現黑素細胞數量減少,黑素小體減少,黑素細胞胞質和樹突以及角質形成細胞中可見Ⅱ、Ⅲ期未成熟的黑素小體,角質形成細胞中可見聚集成糰的黑素小體.17例患者正常區TYR錶達水平為1827.35±307.09,TRP-1為6102.54±1642.64,而皮損區TYR(1477.35±224.05)和TRP-1(5322.33±1565.26)錶達下降,正常區與皮損區比較,P均＜0.01;HMB45、TRP-2、CD117錶達兩處比較差異均無統計學意義.結論 無色素痣是一種早期髮病、非傢族聚集性、穩定的不規則色素減退性疾病,其皮損中黑素細胞和黑素小體數量均減少,可見未成熟黑素小體.相對黑素指數和反射式共聚焦顯微鏡檢查可作為診斷無色素痣的無創性檢測方法.
목적 탐토무색소지적림상화조직학특정.방법 분석85례무색소지환자적발병년령、류형화피손특점,병대부분환자행피부색소측정화반사식공취초현미경(RCM)관찰.대기중17례환자적피손구화정상구피부조직진행조직병리검사,투사전경관찰피손구초미결구.면역조화법검측피손구화정상피부처락안산매(TYR)、HMB45、락안산매상관단백1(TRP-1)、TRP-2화CD117표체.결과 85례무색소지환자중,23례(27.1%)출생시발현피손,21례(24.7%)출현우3세이후,최대발병년령위29세.피손분포우구간부25례(29.4%),경부13례(15.3%);72례(84.7%)피손변연불규칙,54례(63.5%)부유1처피손.19례무색소지환자환처적흑소지수(186.56±52.86)화상대흑소지수(80±11)저우정상인피부(분별위223.88±63.19화100),고우12례백전풍환자피손처(분별위128.57±64.31화60±20),차이균구유통계학의의(P＜0.01).반사식공취초현미경시,무색소지피손중함흑소세포수량감소,량도감저,흑소분포균균,피손구여정상피부분계구상불청석.피손구Fontana-Masson염색시피손구흑소강도위1810.12±327.96,교정상구(2064.24±260.41)명현감약.전경하발현흑소세포수량감소,흑소소체감소,흑소세포포질화수돌이급각질형성세포중가견Ⅱ、Ⅲ기미성숙적흑소소체,각질형성세포중가견취집성단적흑소소체.17례환자정상구TYR표체수평위1827.35±307.09,TRP-1위6102.54±1642.64,이피손구TYR(1477.35±224.05)화TRP-1(5322.33±1565.26)표체하강,정상구여피손구비교,P균＜0.01;HMB45、TRP-2、CD117표체량처비교차이균무통계학의의.결론 무색소지시일충조기발병、비가족취집성、은정적불규칙색소감퇴성질병,기피손중흑소세포화흑소소체수량균감소,가견미성숙흑소소체.상대흑소지수화반사식공취초현미경검사가작위진단무색소지적무창성검측방법.
Objective To study the clinical, histopathologic and ultrastructural characteristics of achromic naevus (AN). Methods Clinical data, including sex, age, age of onset, pattern of lesions, involved sites, shape and number of lesions and associated systemic diseases, were collected from 85 patients with AN. Skin melanin index was detected in 34 lesions of 19 patients with AN, 30 lesions of 12 patients with vitiligo and 64 contralateral normal skin islands of the 31 patients. Reflectance confocal microscopy (RCM) was performed to analyze the lesion, normal skin and junctional area between lesional and normal skin of 62 patients with AN. Tissue samples were obtained from lesions and perilesional normal skin of 17 patients with AN and subjected to pathological examination as well as ultrastructural study with transmission electron microscopy; also, skin biopsy specimens were immunostained for tyrosinase, HMB45, tyrosinase-related protein-1 (TRP-1), TRP-2 and CD117. Results Of the 85 patients with AN, 23 (27.1%) developed lesions at birth, and 21 (24.7%) after 3 years of age; 72 (84.7%) had irregularly shaped lesions, 54 (63.5%) had only a single lesion. The mean melanin index and relative melanin index of AN lesions were 186.56 ± 52.86 and 80 ± 11, respectively, significantly lower than those in normal skin islands (223.88 ± 63.19 and 100, both P < 0.01), but higher than those in depigmented lesions from 12 patients with vitiligo (128.57 ± 64.31 and 60 ± 20, both P < 0.01). RCM revealed a decline in the number of melanocytes and brightness of melanin caps, even distribution of melanin in lesions, as well as obscure demarcation between lesions and normal skin from patients with AN. Fontana-Masson stain showed that the melanin content was lower in lesions than in perilesional skin (1810.12 ± 327.96 vs 2064.24 ± 260.41) from patients with AN. Microscopic examination demonstrated a decrease in melanocyte and melanosome number, presence of immature melanocytes at stage Ⅱ and Ⅲ in cytoplasm and dendrites of melanocytes and keratinocytes, aggregated melanosomes in affected keratinocytes in lesions of AN. In 17 patients with AN, the relative expression levels of tyrosinase and TRP-1 were 1827.35 ± 307.09 and 6102.54 ± 1642.64, respectively, in normal skin specimens, significantly higher than those in lesional skin (1477.35 ± 224.05, 5322.33 ± 1565.26, both P< 0.01); no statistical difference was observed in the expression levels of HMB45, TRP-2 or CD117 between lesional and normal skin. Conclusions AN is an early-onset, nonfamilial aggregated, stable leukoderma with irregular margins, and in lesions of AN, the number of both melanocytes and melanosomes is decreased with the presence of immature melanosomes. The measurement of relative melanin index and reflectance confocal microscopy may offer a non-invasive approach to the diagnosis of AN.
