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。     

Laugier-Hunziker syndrome

Laugier-Hunziker syndrome is a benign pigmentary disorder which manifests as macular hyperpigmentation of the lips and buccal mucosa. Some patients have longitudinal pigmented bands of nails. The syndrome has no systemic associations. Two patients of this rare syndrome are reported. Disorders producing similar pigmentary changes which must be differentiated are discussed.     

Pigmented nail disorders

Nail pigmentation is defined by the presence of melanin in the nail plate. It most frequently has the appearance of a longitudinal pigmented band, called longitudinal mel-anonychia (LM). LM is an important aspect of ungual pathology because it can be the first sign of nail apparatus melanoma. The aim of this article is to help distinguish those types of melanonychia that are worrisome and should lead to biopsy from those that are reasonably reassuring and need only be followed clinically. Histology, differential diagnosis, and biopsy techniques also are considered.     

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.     

Dermoscopic findings in Laugier-Hunziker syndrome

BACKGROUND: Laugier-Hunziker syndrome (LHS) is a rare, acquired mucocutaneous hyperpigmentation often associated with longitudinal melanonychia. The clinical behavior of mucocutaneous pigmented lesions ranges from benign to highly malignant. Therefore, in most cases, the clinical diagnosis should be confirmed by further diagnostic methods. Dermoscopy is a noninvasive technique that has been used to make more accurate diagnoses of pigmented skin lesions. Nevertheless, to our knowledge, the dermoscopic features of the pigmented lesions in LHS have not been described previously. Herein, we report a case of LHS together with its dermoscopic features. OBSERVATIONS: The clinical examination revealed macular hyperpigmentation on the oral and genital mucosa, conjunctiva, and palmoplantar region together with longitudinal melanonychia. Dermoscopic examination of mucosal lesions on the patient's lips and vulva revealed a parallel pattern. Longitudinal homogeneous pigmentation was observed on the toenails. The pigmented macules on the palms and the sole showed a parallel furrow pattern. A skin biopsy sample taken from the labial lesion was compatible with a diagnosis of mucosal melanosis. CONCLUSIONS: By means of this case report, the dermoscopic features of the pigmented lesions in LHS are described for the first time, which facilitates diagnosis with a noninvasive technique. Future reports highlighting the dermoscopic features of this syndrome may simplify the diagnosis of LHS, which is thought to be underdiagnosed.     

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.     

Familial acquired pigmented streaks in the nail

A 40-year-old man developed multiple longitudinal pigmented streaks in his nails. His sister was also found to have a single streak on a fingernail. Neither had any history of drug ingestion or recent illness and examination failed to reveal any other signs in the skin or mucosal surfaces. A biopsy of the nail matrix at the base of one streak showed no evidence of a naevus or lentigo, the principal finding being pigmentary incontinence and excess macrophages.     

Pigmented nail with atypical melanocytic hyperplasia

We report two cases showing black discoloration of the thumb nail which were histologically found to be acral lentiginous melanoma (ALM) in situ. A pigmented subungual lesion is more frequently malignant than benign and it is generally believed that diagnosis of subungual melanoma during the radial-growth phase is very difficult. Our cases are particularly interesting because atypical melanocytic hyperplasia was confined to the epidermis despite the lesion being present for a long time.     

Pigmented nail streak associated with Bowen's disease of the nail matrix

We described a 59-year-old male physician with Bowen's disease occurring on the nail matrix of his right 5th finger. The rapid growth of the pigmented nail streak accompanied by nail deformity led us to consider the possibility of subungual melanoma clinically. Histologic features, however, were compatible with those of Bowen's disease accompanied by melanocytes with melanin-rich long dendrites in the nail matrix. We speculate that his occupational exposure to X-rays for 25 years played an important role in the pathomechanism of the present case.     

Asymptomatic lower lip hyperpigmentation from Laugier-Hunziker syndrome

Laugier-Hunziker syndrome is a rare mucocutaneous pigmentary disorder. It is considered benign, but other mucocutaneous pigmentation disorders are in the differential diagnosis and should be ruled out. This report describes a woman with pigmentation of the labial mucosa of the lower lip who was successfully treated with a Q-switched 532-nm laser.     

A Laugier-Hunziker syndrome associated with esophageal melanocytosis

A 62-year-old Japanese woman came to our clinic because of melanotic macules on the lip, palatoglossal arch, lingual margin and palm. Endoscopic examination revealed a melanotic macule on the midesophageal mucosa but no polyposis in the gastrointestinal tract. Histologically, the specimens taken from the labial, esophageal and palmar lesions showed an acanthosis and basal hyperpigmentation in the epithelium. The patient had not taken any medication which could lead to pigmentation. As far as we know, this is the first case report of an esophageal melanocytic macule which occurred in a patient with Laugier-Hunziker syndrome. When confronted with an isolated pigment spot, we emphasize the necessity of systematic examinations for others. Because the pathologic relationship between Laugier-Hunziker syndrome and the esophageal melanocytic lesion is not proven, further studies should clarify this issue.     

Pigment streaks in the nail plate due to secondary malignant melanoma

We describe two patients with pigment streaks in the nail plate due to secondary malignant melanoma in the nail matrix.     

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.