线状和漩涡状痣样过度黑素沉着病1例

患儿女,10岁。躯干及四肢色素沉着斑10年。皮肤科情况:躯干及四肢可见沿Blaschko线分布的线状、漩涡状褐色色素沉着斑片,边界清楚,表面无鳞屑和萎缩。皮损组织病理示:表皮轻度角化过度,基底层色素增加,灶性基底层液化变性,真皮浅中层小血管周围少量淋巴细胞、噬黑色素细胞浸润。诊断:线状和漩涡状痣样过度黑素沉着病。     

线状回旋状色素沉着1例

线状回旋状色素沉着临床主要以沿Blaschko线分布的不对称性线状或回旋状色素沉着为特点。皮损出现前，无明显的炎症或其他皮肤改变。现报道一例女性患者，15岁，自2岁起于颈部和躯干部出现不对称褐色色素沉着斑，伴有线状或回旋状分布的融合性色素斑片。组织病理学检查显示；表皮基底层黑素细胞和色素颗粒明显增加，未见色素失禁。     

有线状色素沉着表现的皮肤病

临床上一些色紊l生皮肤病表现为线状色素沉着,一些虽然不是色素性皮肤病,但也会出现线状色素沉着。该文综述了有线状色素沉着表现的皮肤病,如线状和漩涡状痣样过度黑素沉着病、线状Becker痣、线状持久性色素异常性红斑、带状黄褐斑、线状色素性紫癜性皮病、线状色素性扁平苔藓、线状皮肤红斑狼疮、线状硬皮病、Moulin线状萎缩性皮病等。     

线状银屑病误诊为线状表皮痣1例

报道1例线状银屑病误诊病例。患儿男,12岁,上下肢条索状红色斑块伴有鳞屑2年。曾于当地医院行病理活检诊断为"炎性线状表皮痣",外用治疗效果不佳。于我院行病理及免疫组化检查,确诊为"线状银屑病",抗银屑病治疗有效。同时分析误诊原因。     

单侧分布线状和漩涡状痣样过度黑素沉着病

报告1例单侧分布线状和漩涡状痣样过度黑素沉着病.患儿男,10岁.右侧躯干、四肢出现线状及旋涡状色素沉着斑8年,而部、掌跖、指(趾)甲、口腔黏膜、外阴未见类似皮损,患儿出现弱视5年.     

成人炎性线状疣状表皮痣1例

患者男,40岁。手部角化性丘疹5年。查体见左手手背、中指及无名指背角化性丘疹,沿Blaschko线分布至肘关节,表面角化性鳞屑;无名指及中指远端指关节变形不能伸展。组织病理符合炎性线状疣状表皮痣。     

回旋形线状鱼鳞病一家系调查

报告1例回旋形线状鱼鳞病患者的家系调查,该家系3代14人中仅有母女2人患病。2例患者均表现为出生后不久全身出现泛发性环形红斑和鳞屑,掌跖角化过度,无毛发异常。组织病理学检查显示:表皮轻度疣状增生,颗粒层变薄,棘层肥厚,角化过度等非特异性改变。     

线状和漩涡状痣样过度黑素沉着病

报告1例线状和漩涡状痣样过度黑素沉着病。患者女,17岁。出生2周后周身即出现色素斑,并排列成条纹状或漩涡状,皮损面积广泛,无黏膜、眼睛、掌跖受累,损害至成年仍未消退。组织病理学检查示基底层色素增加和黑素细胞增多,无色素失禁。本病易与色素失禁症和早期线状表皮痣相混淆。     

线状和漩涡状痣样过度黑素沉着病一例

患者男,20岁.出生后全身开始出现色素斑,逐渐增多并排列成条纹状或漩涡状.无黏膜、眼睛、掌跖受累,损害至成年仍未消退.皮肤科检查:皮损分布广泛,躯干、四肢皆为淡褐色或深棕色条状或漩涡状排列的色素沉着斑纹,沿Blaschko线分布,斑纹较宽,在躯干部略呈漩涡状,弧形,四肢呈条状.组织病理检查:基底层色素增加,无色素失禁.诊断为线状和漩涡状痣样过度黑素沉着病.给予Q开关532 nm激光治疗1次,尚无明显效果,仍在随访中.本病需与色素失禁症和早期线状表皮痣相鉴别.     

炎症性线状疣状表皮痣1例

患者女,47岁。左下肢皮损伴瘙痒半年余。左下肢带状分布鳞屑性红色斑丘疹、丘疹及斑块。皮损组织病理示:表皮角化过度伴角化不全,水平方向上正角化和角化不全交替出现,角化不全处下方的颗粒层消失,表皮银屑病样增生,真皮血管周中等量淋巴细胞为主浸润。诊断为炎症性线状疣状表皮痣。     

Linear and whorled nevoid hypermelanosis complicated with inflammatory linear verrucous epidermal nevus and ichthyosis vulgaris

We report a 17-year-old girl who presented with linear and whorled melanosis following Blaschko lines mainly on her trunk. Ichthyosiform lesions and linear scaling erythemas were observed respectively on her lower limbs and the dorsa of her hands, left knee, ankle and foot. No abnormality was found in systemic examination and blood tests. A biopsy specimen of pigmentation of her back showed there was increased pigmentation within the basal keratinocytes, with focal incontinentia pigmenti. Notable lymphangiectasis could also be seen in the mid part of dermis, which had never previously been described. The biopsy of scaling erythemas of her hand showed dominant hyperkeratosis with focal parakeratosis, acanthosis and papillomatous hyperplasia in the epidermis. The diagnosis of linear and whorled nevoid hypermelanosis (LWNH) complicated with inflammatory linear verrucous epidermal nevus and ichthyosis vulgaris was made in accordance with clinical and pathological manifestations. LWNH ought to be differentiated from incontinentia pigmenti and hypomelanosis of Ito.     

婴儿线状和漩涡状痣样过度黑素沉着病一例

报告1 例婴儿线状和漩涡状痣样过度黑素沉着病。患者女, 5月。出生4 周后周身即出现色素斑, 并排列成条纹状或漩涡状, 皮损面积广泛, 无黏膜、眼睛、掌跖受累, 生长发育无异常。组织病理学检查示基底层色素增加和黑素细胞增多, 无色素失禁。本病常被误诊为色素失禁症。     

Whorled scarring alopecia: a rare phenomenon in incontinentia pigmenti?

Incontinentia pigmenti is a rare X-linked dominant disease that affects the ectodermal tissues and is usually lethal in males. Two girls, 1 Malay and 1 Chinese, with incontinentia pigmenti of the Bloch-Sulzberger type had a whorled pattern of scarring alopecia. This phenomenon, hitherto unreported in association with this disorder, corresponded to the lines of Blaschko. The mother and maternal grandmother of the Malay girl also had whorled scarring alopecia. This phenomenon is permanent and can be used as a marker to ascertain affected adult women who may no longer have cutaneous manifestations. X chromosome inactivation in females during early embryogenesis results in a mosaic population of cells, which explains the linear and patchy cutaneous manifestations of incontinentia pigmenti.     

Linear and whorled nevoid hypermelanosis

Linear and whorled nevoid hypermelanosis (LWNHM) is a reticulate pigmentary disorder with a sporadic occurrence, representing genetic mosaicism. It is characterised by hyperpigmented macules in a reticulate pattern along Blaschko's lines, sparing the mucous membranes and stabilising after one to two years. It may be associated with various neurological abnormalities. The disorder may resemble incontinentia pigmenti, epidermal nevus, or zebra-like hyperpigmentation clinically. We report LWMNHM in a 15-year-old girl with progressively increasing streaks of reticulate hyperpigmented macules arranged in a whorled pattern over the trunk and extremities, which appeared soon after birth. There was no history of any preceding eruption or any associated systemic abnormality. Histopathological examination revealed basal cell hyperpigmentation without any pigmentary incontinence. CT scan of the brain was normal.     

Linear and whorled naevoid hypermelanosis in a patient with trisomy 4 mosaicism

Linear whorled naevoid hypermelanosis (LWNH) is a rare skin condition, characterized by swirls and whorls of hyperpigmented macules distributed in a reticulate pattern along the lines of Blaschko. We report a 2‐year‐old boy who presented with linear and whorled hyperpigmentation on his trunk and limbs, following the lines of Blaschko. Hyperkinesia and developmental speech‐language impairment were also present. A biopsy specimen showed increased pigmentation within the basal keratinocytes without incontinentia pigmenti. No chromosomal abnormality was found in peripheral blood samples. Chromosomal analysis of skin fibroblasts detected trisomy 4 mosaicism. This case shows for the first time an association of LWNH with trisomy 4 mosaicism. LWNH should not be considered a single entity, but a cutaneous expression of mosaicism.     

Linear and whorled nevoid hypermelanosis

Two cases are presented of congenital linear and whorled hypermelanosis. Hyperpigmented macules in streaky configurations along Blaschko's lines appeared gradually after birth. Histologic examination revealed prominent epidermal melanocytes and irregular basal layer hyperpigmentation with normal melanosomes. This condition must be differentiated from incontinentia pigmenti, early systematized epidermal nevus, extensive hypomelanosis of Ito, and chimerism. Other similar case reports from the literature suggest that incidence is sporadic and may be associated with more serious congenital anomalies. The patterning is the inverse to that found in hypomelanosis of Ito. Developmental somatic mosaicism may be responsible for this patterned hypermelanosis.     

Incontinentia pigmenti: variable disease expression within an affected family

We report a florid case of incontinentia pigmenti in a neonate in which linear vesiculobullous, verrucous and pigmented lesions were present simultaneously at birth. Histology of a vesiculobullous lesion showed vesiculation with numerous eosinophils in the epidermis, and a sparse infiltrate in the dermis with pigmentary incontinence. The mother of our patient described a streaky linear rash on her legs during her own childhood which resolved spontaneously, in addition to partial anodontia, suggesting that she too has the disease, although previously undiagnosed. This emphasises the variable disease expression and the importance of recognising this condition so that patients can be followed up with regard to complications, and genetic counselling can be offered. The issue of prenatal diagnosis is discussed.     

线状和漩涡状痣样过度黑素沉着病伴骨骼异常一例

患者，男，15岁。因全身出现线状、漩涡状色素沉着15年就诊。出生时左侧臀部可见局部突起，学步时发现跛行。X线检查示：右下肢较对侧缩短。诊断为线状和痣样过度黑素沉着病合并骨骼异常。