Acral arteriovenous tumor.

A unique, superficial, lobulated, benign vascular tumor of the skin, found in the acral areas of adult males, is described. The combination of three different elements--arterial, venous, and transitional vascular channels--make up the predominant histologic features of this tumor. The transition of fibromuscular channels indicates a venous lesion, but the acral location of the lesions and the structure and staining characteristics suggest that the lesion is a hamartomatous proliferation of the Sucquet-Hoyer canal of the true glomus.     

Acral angiokeratomas in a patient with Turner syndrome

Several types of vascular anomalies have been described in patients with Turner syndrome, including cutaneous lymphatic malformations, vascular anomalies of the heart and aorta, acral venous malformations, and intestinal vascular anomalies. Angiokeratomas have rarely been reported in patients with Turner syndrome. Here, we describe a 14-year-old girl with Turner syndrome who presented with a 2-year history of tender bluish-black keratotic acral papules. Biopsy showed acral skin with focal epidermal acanthosis that was centered on a dilated superficial vessel, consistent with an angiokeratoma. Lysosomal enzyme assays were normal, and she did not demonstrate any other features of a lysosomal storage disorder.     

Persistent hepatic venous plexus in a patient with Sturge-Weber syndrome

Abstract:  A patient with extensive facial and intracranial features of Sturge–Weber syndrome was found to have a persistent primitive hepatic venous plexus, characterized by three additional vessels arising from the inferior vena cava, circulating between liver segments, which formed a common trunk in the supra-hepatic region that flowed into the right atrium. To the best of our knowledge, this hepatic finding has not been previously described in association with Sturge–Weber syndrome.     

Unilateral acquired nevus flammeus in women

Congenital nevus flammeus is a benign vascular tumor characterized by pink to pale red patches that thicken as the patient ages, producing a dull red to reddish blue, cobblestone-textured plaque. We present the cases of 3 women with unilateral acquired nevus flammeus on the cheek whose lesions resolved after minimal treatment with a 585-nm pulsed dye laser. The etiology of acquired nevus flammeus is reviewed and tumor response rates to laser surgery are discussed.     

Meyerson‐Phenomenon hides a nevus flammeus

Facial eczema is a common disease in daily dermatological practice. The cause of facial eczema is often atopic dermatitis or allergic contact dermatitis. Usually, the eczema resolves with correct topical treatment and by avoiding allergic trigger factors. A 49‐year‐old woman presented with persistent eczema on her forehead which recurred over decades despite correct topical therapy. A skin biopsy revealed the astonishing diagnosis of a nevus flammeus with an overlying eczema known as the Meyerson phenomenon. The Meyerson phenomenon is often described in children with nevi flammei suffering from atopic dermatitis – in adults the phenomenon is rarely recognized as a reason for eczema. We show the interesting case of an adult woman with the Meyerson phenomenon on a nevus flammeus and discuss the possible pathomechanisms.     

太田痣并发伊藤痣和鲜红斑痣

报告2例太田痣并发伊藤痣及鲜红斑痣.2例患者均为先天发病,例1为左侧面部太田痣并发双侧肩部、背部、腰部、臀部和上肢伊藤痣及左面颈部、左背部鲜红斑痣:例2为左侧面部太田痣并发肩背部伊藤痣及面部、躯干和四肢多处鲜红斑痣.     

New approach in the laser therapy of nevus flammeus

The technical properties of the flashlamp-pumped dye laser are compared with those of other lasers already used in the therapy of port-wine stains. The advantages of the dye laser seem to be evident. The main reasons are the possibility of free choice of the wavelength and the small width of beam pulse. Short term results are presented. The therapeutical value of this method will be recognized not before lang term results are available.     

儿童鲜红斑痣的激光治疗进展

鲜红斑痣(nevus flammeus),又称葡萄酒色斑(port wine stains),由扩张的毛细血管组成,是最常见的先天性血管畸形,发病率约为0.3%～0.5%,无明显性别差异.损害可发生于体表各部,但以面、颈和头皮多见.通常在开始时为粉红色斑片,随着年龄增长颜色逐渐加深,可呈鲜红或紫红色,皮损渐增厚,在成年期可出现结节.     

Nuchal nevus flammeus as a skin marker of prognosis in alopecia areata

In this work, the incidence of nuchal nevus flammeus was studied in 205 patients suffering from various forms of alopecia areata, as well as in a group of 555 volunteers without alopecia areata examined in our outpatient clinic. The incidence of nuchal nevus flammeus in the totalis-universalis form of alopecia areata was 58.2% (examined patients, n = 79), in ophiasis-extensive forms 22.8% (examined patients, n = 70) and in simple forms of alopecia areata 3.6% (examined patients, n = 56). In the group of 555 volunteers without alopecia areata the incidence of nuchal nevus flammeus was 4.5%. Our results show that nuchal nevus flammeus could be a valuable skin marker indicating a more severe course of alopecia areata.     

Congenital absence of a kidney in a patient with the basal cell nevus syndrome

Basal-cell nevus was diagnosed in a 60-year-old patient who suffered for 20 years from multiple basal-cell carcinomas. Autopsy has revealed congenital absence of the right kidney and ureter after his death at the age of 61.     

A novel epidermal nevus syndrome with congenital cylindromatous turban tumor

BACKGROUND: Epidermal nevi (in the broad sense of epithelial nevi) may give rise to benign or malignant skin tumors. They may also be associated with anomalies of other organ systems in an epidermal nevus syndrome. RESULTS: This article describes a preterm infant with nevus sebaceus of the scalp and face, a large turban tumor with features of malignant cylindroma and multiple non-cutaneous defects. These included skeletal, hematopoietic, hepatobiliary, and urinary anomalies. Severe secondary lesions were present (pulmonary hypoplasia due to oligohydramnios; cerebral infarcts probably related to the turban tumor). Karyotype was normal, and family history was negative. CONCLUSIONS: This unique case is unlike any reported epidermal nevus syndrome. Similarly, there is no prior report of a congenital cylindroma, certainly not as a turban tumor, which implies very rapid growth. The presence of both overgrowth and undergrowth phenomena (e.g. hypoplastic urinary tract and biliary atresia) may reflect dysregulation of paracrine growth factors, presumably due to genetic mutation.     

脉冲染料激光治疗鲜红斑痣后湿疹一例

报道一例脉冲染料激光治疗鲜红斑痣后湿疹。患儿,男,4月,因左侧面部渗出、结痂6天就诊,患者因鲜红斑痣接受脉冲染料激光治疗,第2次治疗后1周治疗区域出现渗出与结痂。诊断为湿疹,予0.1%丁酸氢化可的松乳膏外用,每日2次,5天后湿疹消退。