Epidemiology of congenital pigmented naevi:

Genetic and environmental risk factors were investigated in a sample of 989 non-malformed newborn infants with skin pigmented naevi (PN), and in 989 non-malformed non-PN, control babies. The samples were obtained from a population of 531,831 livebirths, from fifty-nine South-American maternity hospitals. No significant differences were observed between the PN and control groups for the following risk factors: sex, twinning, parental consanguinity, socioeconomic level, parental ages, birth order, fetal presentation, type of delivery, postnatal mortality, and first trimester of pregnancy histories for maternal chronic illnesses or immunizations, radiation exposure, drug intake, and vaginal bleeding. PN infants were associated with high frequencies of Black racial ancestry, positive prenatal history for maternal acute illnesses, and high mean values for length of gestation and birth weight. The observed association of PN with maternal acute illnesses could be a spurious one, due to partial dependence upon Black racial ancestry, and to maternal memory bias. The analysis of the intrauterine growth curves showed that PN infants have a low prematurity rate, and a high mean birth weight within the postmature group, lacking the normal post-term weight loss pattern shown by the control newborn group. This observation suggests that congenital PN appear in the latest stages of intrauterine development. No evidence was obtained to allow us to consider congenital PN as true developmental anomalies.     

Distribution of diffuse plexiform neurofibroma on the body surface in patients with neurofibromatosis 1

Neurofibromatosis 1 (NF1) is a genetic disease characterized by cutaneous, neurological and osseous abnormalities. Approximately 20% of patients develop plexiform neurofibroma (PN), resulting in impaired quality of life. To evaluate distribution of diffuse PN on the body surface, a retrospective study was conducted for 354 patients with NF1 from 2007 to 2018 in Japan. We investigated a total of 40 patients with clinically apparent superficial diffuse PN. In the cases evaluated, 57.4% of the diffuse PN were located on the trunk, 19.2% the head and neck, 12.8% the lower limbs and 10.6% the upper limbs. Remarkably, 75.0% of the diffuse PN were located on the dorsal side. The frequency was significantly higher on the trunk than on the head and neck (P = 0.026). Our findings provide useful information for giving attention to the high possibility of diffuse PN on the dorsal side before progression in childhood and for future treatment in NF1.     

Detection of atypical texture features in early malignant melanoma

Background: The presence of an atypical (irregular) pigment network (APN) can indicate a diagnosis of melanoma. This study sought to analyze the APN with texture measures.
Methods: For 106 dermoscopy images including 28 melanomas and 78 benign dysplastic nevi, the areas of APN were selected manually. Ten texture measures in the CVIPtools image analysis system were applied.
Results: Of the 10 texture measures used, correlation average provided the highest discrimination accuracy, an average of 95.4%. Discrimination of melanomas was optimal at a pixel distance of 20 for the 768 × 512 images, consistent with a melanocytic lesion texel size estimate of 4–5 texels per mm.
Conclusion: Texture analysis, in particular correlation average at an optimized pixel spacing, may afford automatic detection of an irregular pigment network in early malignant melanoma.     

Congenital localized absence of skin associated with blistering of the skin and mucous membranes: Bart's syndrome

A newborn girl presented with a congenital localized absence of skin on both legs. At the age of eight months, blistering of the scalp, upper limbs, and oral mucous membranes occurred, which was nonscarring and recurrent. This case is reported because of its rarity.     

Accessory scrotum attached to a peduncular perineal lipoma

In this study, we report on the case of a newborn boy diagnosed after birth with an accessory scrotum attached to a peduncular type of perineal lipoma without any other associated congenital anomalies. The neonate underwent a simple surgical excision of the lipoma and accessory scrotum in the first month of life, and his postoperative course was uneventful. Histologic examination revealed normal scrotal skin and adipose tissue. Accessory scrotum has a high incidence of association with perineal lipoma (83% of reported cases) and other urogenital and anorectal anomalies, but urogenital or anorectal anomalies were not seen in our patient.     

A newborn with nodular ulcerated lesion on a giant congenital nevus

The proliferative nodule is a lesion that develops within a congenital nevus. It is usually small (less than 5 mm), sometimes multiple, with a slow growth rate, and has a black or dark brown, smooth, shiny surface. It usually involutes spontaneously. We report a newborn infant who, at birth, had a giant congenital nevus with a nodular, ulcerative, hemorrhagic lesion within it. Physical and neurologic examinations were normal. Radiologic studies at birth and subsequently were normal. A fragment of the lesion was biopsied and histologic findings were compatible with a diagnosis of proliferative nodule in a giant congenital nevus. The rest of the nodule regressed spontaneously after 4 months.     

Generalized Lichen nitidus associated with Down's syndrome: case report

Lichen nitidus is a disease of unknown etiology, characterized by flesh-colored, shiny papules of 1-2 mm and generally asymptomatic or with mild pruritus. The most common sites of occurrence are genitalia, upper limbs, trunk and abdomen. The generalized form is rare. This is the fourth reported case of lichen nitidus associated with Down Syndrome.     

Scarring Resulting from Chickenpox

Nine hundred and eighty-six children (519 boys, 467 girls) who had chickenpox at least 1 year previously were examined for the presence of scars resulting from this disease. Ninety-six (18.5%) boys and 88 (18.8%) girls had chickenpox scars, giving rise to an overall prevalence of 18.7%. The scars were found on the face in 75 (40.8%), neck 2 (1.1%), shoulders 8 (4.3%), upper limbs 15 (8.2%), anterior thorax 50 (27.2%), abdomen 106 (57.6%), back 65 (35.3%), buttocks 9 (4.9%), and lower limbs 12 (6.5%) affected children. The mean number of scars in the 184 children was 2.8 (standard deviation 1.9). The scars were hyperpigmented in 32, hypopigmented in 160, depressed in 38, and hypertrophic in 32 children. Keloids were noted in two children.     

Dermoscopic naevus patterns in people at high versus moderate/low melanoma risk in Queensland

Introduction: Dermoscopic understanding of naevus characteristics is essential baseline knowledge for identifying early malignant changes. Method: This cross‐sectional study includes 34 patients (56% female, mean age 48 years) at high risk of melanoma (personal or a first degree family member with history of melanoma) and 31 moderate/low melanoma risk volunteers (55% female, mean age 37 years) recruited at the Princess Alexandra Hospital, Brisbane, between October 2009 and March 2010. Participants received full body and individual dermoscopic imaging of clinically significant naevi (≥2 mm on the back of male/female and lower limbs of female and ≥5 mm at other body sites). Dermoscopic patterns of naevi were compared between people at high versus moderate/low melanoma risk according to age and body site. Results: In both high and moderate/low risk groups, globular naevi predominated on the head/neck and abdomen/chest, reticular and non‐specific naevi on the back, and non‐specific pattern on the upper and lower limbs. Non‐specific naevi were the most common in all age groups. In both risk groups, globular naevi were more frequent in the younger age bracket, and reticular naevi were more frequent in the older age bracket. Mixed naevus patterns were infrequent and were more common in the younger age brackets of both risk groups. Conclusion: Our preliminary data shows that dermoscopic naevus patterns were similar for age and body site in people at different levels of melanoma risk, suggesting high melanoma risk does not influence dermoscopic naevus patterns.     

Lateral displacement of the nipples and unilateral renal hypoplasia: another mammo-renal syndrome?⋆

Background of study Polythelia is a cutaneous marker of congenital diseases of the kidneys. Lateral displacement of the nipples (e.g. laterally located to their respective midclavicular lines) is considered a further sign of renal disorders, particularly of bilateral renal hypoplasia, as reported by Fleisher in a detailed study on seven infants. Subject We observed a 45-year-old white man with his morphologically normal nipples displaced on the lateral aspects of the trunk without any other consistent clinical features such as accessory nipples or areolae, gonadal dysgenesis, face or car anomalies. Aims and methods In order to reveal congenital abnormalities of the kidneys and the urinary collecting systems the following investigations were undertaken: renal ultrasound, intravenous pyelography. abdomen CT and NMR scans, urethrocystography, transurethral cystoscopy, urinalysis, laboratory tests including karyotype, ECG and cardiovascular evaluation. Results Instrumental findings disclosed a hypoplastic left kidney in an ectopic site, located below the right kidney (cross type of renal ectopy) without any connection between their urinary- collecting systems. Complex malformations of the urinary tract such as an orthotopic narrow right ureteral orifice and ridge, a large-sized diverticulum on the left wall of the bladder were also present. Moreover, a tumour on the upper pole of the right kidney, 20 mm in diameter, with a histological picture of renal-cell carcinoma was detected. Conclusions A lateral displacement of the nipples is a clue for the presence of renal hypoplasia both bilateral and unilateral. The significance of this association is unclear and its occurrence is probably underestimated.     

Pustular psoriasis of pregnancy in a patient whose dermatosis showed features of acute generalized exanthematous pustulosis

A 23‐year‐old pregnant woman at 38 weeks of gestation presented with a 2‐day history of a red eruption on her abdomen and upper thighs. The patient had applied baby oil to her abdomen a few days earlier, but she denied using any other topical products. Her only medications were prenatal vitamins and iron, which she had been taking since the beginning of her pregnancy. The clinical impression was contact dermatitis, and she was started on topical Sarna lotion (containing 0.5% camphor and 0.5% menthol) after she declined topical steroids. Three days later, she presented to the dermatology clinic with worsening pruritus, mild diarrhea, and subjective chills. On physical examination, there were diffuse erythematous plaques studded with 1–2‐mm pustules on the upper extremities, chest, back, abdomen, and upper thighs ( Fig. 1a–c ). The pustules were distributed diffusely throughout the plaques with no obvious circinate pattern.

Figure 1 Open in figure viewer PowerPoint (a) Diffuse erythematous plaques on the abdomen and proximal upper thighs. (b) Abdomen showing coalescing pustules. (c) Proximal upper thigh showing clustered 1–2‐mm pustules on an erythematous plaque     

成人表皮痣综合征1例

 报告1例成人表皮痣综合征。患者男,28岁,因皮疹、智力低下、多动28年,间断抽搐27年就诊。皮肤科检查：额部、眼周、颈部可见弥漫性密集的淡褐至褐黑色乳头瘤样角化性丘疹,触之坚硬,似高起鱼鳞病、先天性良性黑棘皮病样。躯干、双上肢、四肢皮损角质增厚,较额部、眼周、颈部薄,可见弧形带状或旋涡状排列,不规则斑片状或斑点状色素脱失斑,与增生性皮损及正常皮肤相互交错。颈部皮损组织病理检查示纤维上皮性息肉改变,胸部皮损组织病理检查示表皮痣样改变。脑电图：脑电图异常伴高中波幅尖波及4~7 Hz的θ节律短阵出现。诊断为表皮痣综合征。     

先天性色素痣伴增生性结节10例临床病理分析

【摘要】 目的 探讨先天性色素痣伴增生性结节的临床特点及组织病理特征。方法 收集第四军医大学西京皮肤医院2015—2019年经临床和病理确诊的10例先天性色素痣伴增生性结节患者的临床及病理资料，并进行回顾性分析。结果 10例患者年龄2～45岁（平均15岁），9例增生性结节发生于婴儿，1例发生于成人。皮损位于四肢4例，头面部3例，躯干2例，躯干及四肢同时受累1例。临床表现为黑色斑片或斑块中出现1个或多个结节，6例增生性结节为多发，4例为单发，单个结节直径0.2～1.5 cm，仅1例出现溃疡。组织病理检查显示增生性结节内黑素细胞均存在成熟现象，核分裂象少，细胞无明显异型性，无坏死现象，免疫组化检查显示痣细胞弥漫表达Melan-A，不表达或仅部分表达HMB45，Ki67增殖指数 < 5%。结论 先天性色素痣伴增生性结节可发生于四肢、头面部及躯干；临床表现为原先天性色素痣皮损上的单发或多发结节；病理上增生性结节内黑素细胞可见成熟现象，免疫组化HMB45及Ki67染色有助于诊断，其预后有待长期随访。     

全身转移性黑素瘤1例

患者女,60岁。头面、躯干、四肢大小不等黑红色结节,无痛痒3年,半年内泛发全身。皮肤科情况:头面、躯干、四肢见大小不等高粱粒至黄豆大小结节,呈皮色、暗红色及黑色。左膝关节上方见一直径约2cm×2cm大小肿块,其上见4～5个大小不等皮色至黑色结节,部分结节表面结痂。皮损组织病理示:真皮内弥漫性肿瘤样细胞浸润,此肿瘤样细胞核大、核仁明显,部分肿瘤样细胞呈巢状排列,肿瘤组织内见坏死。免疫组织化学染色显示肿瘤细胞LCA(-),CK20(-),S100蛋白及HMB45弥漫强阳性,Vimentin弥漫阳性。诊断:黑素瘤。     

大疱性蕈样肉芽肿

报告1例大疱性蕈样肉芽肿.患者男,40岁.因躯干、四肢红斑伴瘙痒6年,出现斑块半年、水疱1个月就诊.皮肤科检查:面部、躯干及四肢泛发红斑和斑块,部分斑块浸润明显.右下腹斑块上可见0.5 cm×2 ca的水疱.皮损组织病理检查:真皮浅层明显水肿,部分区域可见表皮下疱形成,真皮浅层可见异形明显的单一核细胞呈带状浸润.部分单一核细胞侵入表皮,形成Pautrier微脓肿.免疫组化染色结果示LCA( )、CD4( )、CD45RO( ).结合临床、免疫组化和组织病理检查结果,诊断为大疱性蕈样肉芽肿.     

Phlebectomy--cosmetic indications

Ambulatory phlebectomy is a safe, aesthetic, effective and economical operative technique, which enables venous extraction with a special hook, assuring complete and definitive eradication of the veins. Minimal skin incisions (1-3 mm), or even needle punctures, usually result in little or no scarring. This aesthetic method is particularly indicated to treat unsightly varicose veins, such as reticular veins (popliteal fold, lateral thigh and leg), groin pudendal veins, veins of the ankles and the dorsal venous network of the foot. Curettage of telangiectasias is a less well-known technique, but it is also effective in removing networks of thick blue spider veins. Ambulatory phlebectomy of body areas other than the lower limbs include dilated periorbital, temporal or frontal venous networks and venous dilatation of the abdomen, upper limbs or dorsa of the hands.     

Cutis marmorata telangiectatica congenita: a prospective study of 27 cases and review of the literature with proposal of diagnostic criteria

Background.  Cutis marmorata telangiectatica congenita (CMTC) is a congenital vascular anomaly of unknown aetiology. About 300 cases have been reported in the literature. The rate of associated anomalies varies between 20% and 70%.
Methods.  We report a series of 27 children with CMTC, 18 of whom were followed-up prospectively for a median of 22 months (range 2 months–5.3 years).
Results.  Both genders were equally affected (13 male/14 female). The legs were involved in 20 cases (74%), the arms in 10 (37%), the face in 4 (15%) and the trunk in 18 (67%). There were 20 (74%) patients who presented with involvement of both trunk and limbs, a further 20 patients had lesions affecting the limb on only one side of the body, and 7 children (26%) had bilateral lesions; 1 child had generalized CMTC lesions. The involved areas covered a mean of 18% of body surface area (range 3–90). Associated anomalies were found in 15 patients (56%), with some exhibiting more than one. There was body asymmetry (hypertropy or hypotrophy of the affected limb) in nine patients (33%), seven patients had a variety of other malformations (congenital glaucoma, syndactyly, lipoma, macrocephaly, renal hypoplasia, Kartagener's syndrome), and other vascular lesions were present in four patients (15%). There was no correlation between the extent of skin lesions and likelihood of associated anomalies. On follow-up, fading of skin lesions was noted in 67% of our patients.
Conclusion.  Body asymmetry is the most common anomaly associated with CMTC; other associations might be pure chance. In order to separate CMTC from other vascular malformations, notably Klippel–Trénaunay syndrome, we suggest diagnostic criteria for their differentiation.     

恙虫病45例临床特征分析

目的：分析恙虫病患者的临床特征。方法：回顾性分析2012年8月至2016年3月在我们皮肤科就诊的45例恙虫病患者的临床资料。结果：潜伏期1-3周。主要表现发热（100.00%,45/45）、头痛（28.89%,13/45）、肌痛（55.56%, 25/45）、皮损（100.00%,45/45）、疲倦（71.11%,32/45）、胃肠道症状（35.55%,16/45）。37例（82.22%）患者躯干四肢出现红斑和斑丘疹。特征性皮损是所有患者出现焦痂,好发于胸部（28.89%,13/45）、腹部（24.44%,11/45）、腋下（11.11%,5/45）和外阴（8.89%,4/45）。结论：恙虫病临床表现多样,皮肤焦痂溃疡性损害是本病的特征性皮肤表现。     

True Tail in a Newborn

Abstract: Human true tall is a rarely reported anomaly that may have a marked psychologic impact on the patient's family and may be associated with other congenital anomalies. A true tail in a newborn girl is reported, and findings from a review of the literature are summarized. The clinical and pathologic differential diagnoses are discussed, as they might affect the management and prognosis of this congenital malformation.