Laugier-Hunziker综合征1例

患者女,59岁。唇、齿龈、舌、颊黏膜及硬腭色素沉着15年,指(趾)甲色素沉着10年,上肢、手足色素沉着1年。无腹部症状,胃肠镜检查无异常。皮肤科情况:唇、齿龈、舌、颊黏膜及硬腭多发、散在或群集的黑褐色色素沉着斑,部分指(趾)甲纵行黑褐色色素沉着斑,左上肢及手足褐色色素沉着斑。左上肢皮损组织病理检查:表皮基底层细胞中色素增多。诊断:Laugier-Hunziker综合征。     

Peutz-Jeghers综合征1例

患者男,32岁。口唇、口腔黏膜、手足出现褐色斑点24年。皮肤科检查示:唇部、颊黏膜、齿龈、双手指规则的黑色色素沉着斑。肠镜示多发息肉。诊断:Peutz-Jeghers综合征。     

Laugier-Hunziker综合征1例

患者女,58岁.因指(趾)甲黑色条状斑6年,唇、齿龈及颊黏膜色素斑1年余就诊.患者6年前无明显诱因出现右手第1～4指甲,左手第1～3、5指甲,右足5个趾甲,左足第4、5趾甲纵形灰黑色条纹.1年多前双唇、双侧颊黏膜及上下齿龈逐渐出现点状或片状圆形或卵圆形棕黑色色素沉着斑.患者无自觉症状,未予治疗.病程中无暖气、呕吐、腹痛、腹泻及便血等症状.患者既往体健,否认系统性疾病史及长期服用免疫抑制剂、四环素类药物及中药等药物史,无药物过敏史、吸烟史及重金属接触史,家族成员无类似疾病患者.体格检查:各系统检查未见明显异常.     

Laugier-Hunziker综合征1例及文献复习

患者女,53岁。唇部、舌周、颊黏膜、牙龈及右足趾甲、色素沉着30余年,呈进行性增多。无腹部症状。大便潜血试验(-),胃镜及结肠镜检查均无异常。皮肤科情况:唇部、舌周边、颊黏膜及牙龈可见灰黑色色素沉着斑,右足部分趾甲可见黑色纵行条带。组织病理未作。诊断:Laugier-Hunziker综合征。     

Laugier-Hunziker综合征一例

患者女,37岁,以口唇起色素沉着斑20余年,颊黏膜、牙龈、指(趾)甲起黑色条状斑2年余来我科门诊就诊.患者20余年前发现唇红缘起点状色素沉着斑,逐渐扩大成片状,且颜色逐渐加深,未在意.2年前,患者发现牙龈起黑色斑,颊黏膜条状色素沉着斑,同时右手2,3,4指甲,左手2,3指甲,右足1,2趾甲见条状纵行黑色条纹.     

Laugier-Hunziker综合征1例及家系调查

报告1例Laugier-Hunziker综合征,并进行家系调查.先证者男,50岁.指(趾)甲色素沉着16年,颊黏膜色素沉着11年.无腹部症状,胃肠镜检查未发现息肉.组织病理检查示基底层黑素增加,真皮乳头色素失禁和少量噬黑素细胞.该家系4代19人中共有1人患本病,其余2人为疑似病例,表现为指(趾)甲不同程度的纵形色素沉着.     

Laugier-Hunziker综合征3例

Laugier-Hunziker 综合征是一种获得性、良性口唇、颊黏膜和指(趾)甲的色素疾病,在临床上非常少见,笔者于2005 年2月-2009年1 月诊治3 例,现总结报告如下. 例1.女,52 岁.因口唇色素斑5 年,于2005 年2 月前来深圳市第六人民医院就诊.5 年前开始于下唇红部出现黑褐色斑,后无意中发现颊黏膜、牙龈亦有类似皮损,局部无明显自觉症状,近5 年来皮损稍增大、色素加深,近1 年发现指(趾)甲纵行黑色条带,未予任何治疗.     

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.     

扁平苔藓样慢性移植物抗宿主病1例

<正>1病历摘要患者男,40岁。因躯干和四肢紫红斑、口腔溃疡4个月余,指、趾甲损害3个月,于2013年4月就诊于我科。患者4个月前于胸部发生红斑鳞屑及色素沉着,并出现口腔溃疡和牙龈出血,皮疹渐发展至躯干、上肢。3个月前指(趾)甲周围出现红斑,甲纵嵴,甲脆裂。皮疹渐发展至面部、唇部、下肢,呈弥漫分布。既往史:患者     

Laugier-Hunziker综合征一例

<正>Laugier-Hunziker综合征(Laugier-Hunziker syndrome,LHS)是由Laugier和Hunziker两人首先报道的一种唇、口腔黏膜和指(趾)甲色素沉着性疾病。本文报道1例LHS患者。临床资料患者,女,72岁。主因发现唇部及舌体散在黑斑2月余入院。2个月前无明显诱因,患者下唇部及舌体前端出现点状或片状黑褐色斑点,后迅速增多,无任何自觉症状,无恶心、呕吐,无腹痛、便血     

Pseudo-melanoma revealing Laugier-Hunziker syndrome

A 47-year-old Arabic woman presented with a few months history of a 20 × 10 mm brown-black interdigital macule, irregular in shape and outline (Fig. 1). The patient had no previous personal or familial history of dysplastic nevi, melanoma, or gastrointestinal polyposis. She was receiving fluindione, furosemide, digoxin. and lisinopril dihydrate for chronic atrial fibrillation. She had a mitral valvular bioprothesis secondary to rheumatic heart disease.
On physical examination there were no enlarged lymph nodes. Multiple pigmented macules were noted on the lower lip and buccal mucosa (Fig. 2). Genital mucosa showed no pigmentary abnormalities. There were also many pigmented longitudinal streaks involving five finger- and two toenails without Hutchinson's sign, two brownish interdigital macules of the fingers (Fig. 3), and dark brown spots around the nails. Clinical examination of her two sisters and three children did not demonstrate mucocutaneous pigmentation.
Investigations showed normal renal, hepatic, and thyroid function. Human immunodeficiency virus serology was negative. Two-punch biopsy 4×4 mm of the right darkness and left edges of the interdigital lesion confirmed the diagnosis of Laugier-Hunziker syndrome with basal hypermelanosis without melanocytic proliferation or cytologic atypia (Fig. 5). After 2 years follow-up, clinical and histologic examination was not modified.     

Laugier-Hunziker综合征

报告8例Laugier-Hunziker综合征,其中男2例、女6例,年龄38～62岁。所有患者下唇均有色素沉着斑。4例患者皮损累及颊黏膜,3例患者累及上唇,3例患者累及指、趾甲,2例患者累及牙龈。3例患者的皮损组织病理检查显示基底细胞色素增加,真皮乳头噬黑素细胞数量增多。5例患者唇部皮损采用波长752nm、能量密度6.0～8.2J/cm2、光斑直径2.4mm的Q开关紫翠宝石激光器(PhotoGenicaT10)治疗1次,均取得明显的疗效,皮损大部分消退。     

Pigmented nail streaks may indicate Laugier-Hunziker syndrome

Laugier-Hunziker syndrome is a rare, benign pigmentation disorder previously described only once in the United States. The syndrome is acquired in early or mid-adult life and is characterized by multiple longitudinal hyperpigmented bands on the nails and pigmented macules of the lips and buccal mucosa. Peutz-Jeghers syndrome and Addison's disease can present with pigment abnormalities similar to those characteristic of Laugier-Hunziker syndrome.     

Laugier-Hunziker syndrome: a clinical, histopathologic, and ultrastructural study of four cases and review of the literature.

Four cases of Laugier-Hunziker syndrome are described. In all patients (two men and two women between 39 and 57 years of age) pigmentation of the lower lip and hard palate was found. in addition, two patients had involvement of the buccal mucosa; another patient also had pigmentation of the upper lip, the gums, the soft palate, and the fingers of both hands. Histopathologic examination demonstrated an accumulation of melanin in the basal layer keratinocytes and an increase in the number of melanophages in the papillary dermis. Ultrastructural study showed the presence of numerous mature melanosomes in the cytoplasm of the keratinocytes of the basal layer and of the melanophages in the papillary dermis. Alterations of the melanocytes were not observed.     

NAIL AND SKIN HYPERPIGMENTATION ASSOCIATED WITH HYDROXYUREA THERAPY FOR POLYCYTHEMIA VERA

A 63-year-old black woman presented with a complaint of nail and skin darkening for 5 months. Past medical history included polycythemia vera, hypertension, angina, and hi-atal hernia. For several years the patient's medications had included isosorbide dinitrate, iron sulfate, propranolol, and sucralfate. The only recent new medication was hydrox-yurea (500 mg to 1.5 g daily), which the patient had started for polycythemia vera 8 months prior to presentation. She now complained of asymptomatic hyperpigmentation of the finger and toenails, which had begun proximally and extended distally over time. She also noted increased pig-mentation of her face, neck, arms, and buccal mucosa.
Physical examination revealed pigmentary changes in all the nails. Dyschromia varied from blue-grey to brown-black. Three different patterns of pigmentation were noted: trans-verse and longitudinal bands that alternated with areas of lighter discoloration (Fig. 1) and diffuse hyperpigmentation (Fig. 2). The nail plates and the proximal and lateral nail folds had a normal morphology aside from the color changes. Macular brown pigmentation was present on the patient's face, neck, lower arms, palms, and buccal mucosa (Fig. 3). The patient noted that the pigmentation in all of these areas began after the hydroxyurea was started; those areas of skin and mucosa had previously been normal. Lab-oratory results were significant for a mild anemia, hemoglo-bin 11.4 g/dL, and hematocrit 35.2%. The patient refused nail or skin biopsy. The patient also noted increased "hardness" of the nails, which made it difficult for her to cut them.
Microscopic observation of sections prepared from nail clippings revealed a keratinjzed layer with scattered finely granular brownish pigment. Fontana Masson and Schroml's stains for melanin were positive, and Pearl's stain for iron was negative.     

The Laugier—Hunziker syndrome—a clinical review of six cases

The Laugier-Hunziker syndrome is an acquired, benign, macular hyperpigmentation of the lips and buccal mucosa. The nails are often involved with the development of melanonychia. Twenty-two previous cases have been recorded in the literature. We present details of six Caucasian patients with the Laugier-Hunziker syndrome who are the first recorded from Britain. They all had acquired, macular hyperpigmentation of the lips and buccal mucosa. In five of these patients longitudinal pigmented bands were found on the nails. None had other family members affected. Although this is the first report of British patients with this syndrome, we believe that the condition is probably more common than is generally recognized.     

A case of Laugier's disease

A 60-year-old male patients with an extremely rare condition, Laugier's disease (idiopathic lenticular pigmentation of buccal and labial mucosa) is described. The clinical picture of the disease is presented, as are results of examination and differential diagnosis of the dermatosis. Possible predisposing (dark skin integument, quite a number of pigmented nevi) and provoking (chronic and acute insolation) factors may be important in elucidating the disease origin.     

Hereditary diffuse hyperpigmentation

A healthy white female of normal intelligence aged 33 years, who presented with scalp hair thinning, showed congenital mottling of the skin more marked over the trunk and with epidermal atrophy on histology, smooth fingertips, brittle finger nails some split at the free end, and warty palmar keratoses. She gave a history of frictional blisters over the heels in the first few years of life only. Blood picture, sweating, buccal mucosa and teeth were normal. Her son, aged 7 years, showed similar mottled skin over the trunk and this was first noticed at the age of 6 months. He also had a tendency to form blisters over the heels up to 21 years of age. At least four other male members of the family had the abnormality of pigmentation and other changes but unfortunately all persistently refused to permit examination. Inheritance was in keeping with an autosomal dominant pattern.     

Atypical moles in a patient undergoing chemotherapy with oral 5-fluorouracil prodrug

We present a patient with multiple pigmented lesions on the palms, soles, oral mucosa and nails after chemotherapy with oral 5-fluorouracil (5-FU) prodrug. Dermoscopically, most of the macules showed similar features, with pigmentation present predominantly on the crista superficialis, while a large, dark macule also showed pigmentation along the sulcus superficialis with irregular hyperpigmentation and depigmentation, suggesting malignancy. However, histologically, both types of lesion showed basal hyperpigmentation and the presence of a small number of large atypical melanocytes. We diagnosed these lesions as pigment flecks induced by 5-FU, and the pigmented lesions gradually diminished after the cessation of chemotherapy. Our findings suggested that immunosuppression and 5-FU led to the development of the atypical pigmented lesions.