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    2997 results match your criteria Congenital Nevi

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    CHILD Syndrome Mimicking Verrucous Nevus in a Chinese Patient Responded Well to The Topical Therapy of Compound of Simvastatin and Cholesterol.
    J Eur Acad Dermatol Venereol 2018 Jan 17. Epub 2018 Jan 17.
    Department of Dermatology, Xinhua Hospital Shanghai Jiaotong University School of Medicine, Shanghai, China.
    Background: Congenital hemidysplasia with ichthyosiform erythroderma and limb defects (CHILD) syndrome is a rare X-linked dominant disorder characterized by peculiar cutaneous presentations and ipsilateral skeletal abnormalities. CHILD syndrome is caused by mutations in NSDHL gene, which involves in cholesterol synthesis.

    Objectives: To verify the diagnosis of CHILD syndrome and seek effective pathogenesis-based therapy with little side effects. Read More

    [Phakomatosis pigmentovascularis cesioflammea: a case report].
    Arch Argent Pediatr 2018 Feb;116(1):e121-e124
    Servicio de Dermatología, Hospital Nacional de Pediatría "Prof. Dr. J. P. Garrahan", Ciudad Autónoma de Buenos Aires.
    Phakomatosis pigmentovascularis (PPV) is a syndrome characterized by the association of a vascular nevus with a congenital pigmented lesion (epidermal nevus, nevus spilus, and dermal melanocytosis). There are different types of PPV according to the pigmentary nevus associated with the vascular malformation. Patients may present only the cutaneous condition or have systemic manifestations, among them, trauma, neurological and ophthalmological disorders. Read More

    Widespread dynamic and pleiotropic expression of the melanocortin-1-receptor (MC1R) system is conserved across chick, mouse and human embryonic development.
    Birth Defects Res 2018 Jan 8. Epub 2018 Jan 8.
    GMGF, Aix Marseille University, INSERM, UMR_S910, Marseille, France.
    Background: MC1R, a G-protein coupled receptor with high affinity for alpha-melanocyte stimulating hormone (αMSH), modulates pigment production in melanocytes from many species and is associated with human melanoma risk. MC1R mutations affecting human skin and hair color also have pleiotropic effects on the immune response and analgesia. Variants affecting human pigmentation in utero alter the congenital phenotype of both oculocutaneous albinism and congenital melanocytic naevi, and have a possible effect on birthweight. Read More

    Giant congenital melanocytic nevus with vascular malformation and epidermal cysts associated with a somatic activating mutation in BRAF.
    Pigment Cell Melanoma Res 2018 Jan 5. Epub 2018 Jan 5.
    Dermatological group practice, Lübeck, Germany.
    Giant congenital melanocytic nevi may be symptomatically isolated, or syndromic. Associations with capillary malformations are exceptional, and development of epidermal cysts has not been described. A 71-year old patient with a giant congenital melanocytic nevus of the lower back, buttocks and thighs was asymptomatic except for unexpected hemorrhage during partial surgical excision years before. Read More

    Speckled lentiginous nevus: understanding the process of development and regression.
    Br J Dermatol 2018 Jan 9. Epub 2018 Jan 9.
    Department of Dermatology, Yamaguchi University Graduate School of Medicine, Yamaguchi, Japan.
    Speckled lentiginous nevus (SLN) is characterized by a brownish macule studded with blackish, multiple melanocytic nevi, although the cutaneous manifestations may vary by cases, and change by age. Therefore, there has been disagreement as to whether SLN is a congenital or acquired disease 1, 2). In rare cases of SLN, halo phenomenon may occur around the pre-existing lesion, associated with the coexistence of distant vitiligo 3). Read More

    Using Tissue Expanders as a Choice for the Treatment of Congenital Facial Nevus in the Young Child.
    Eur J Pediatr Surg 2018 Feb 2;28(1):105-108. Epub 2018 Jan 2.
    Division of Vascular Anomalies, Department of Pediatric Surgery, La Paz Children's Hospital, Madrid, Spain.
    Objective:  One of the principal objectives in treatment of facial nevus is to minimize psychological damage and encourage the child's schooling by the best possible cosmetic result. There are several therapeutic techniques: grafts, flaps, dermal regenerator, and tissue expanders.

    Materials And Methods:  We reviewed 10 patients with facial nevus higher than 10 cm treated in the past 8 years. Read More

    Kissing nevus of the penis: a case report and dermatoscopic findings.
    An Bras Dermatol 2017 ;92(5 Suppl 1):95-97
    Department of Dermatology, Faculdade de Medicina da Universidade do Oeste Paulista (Unoeste) - Presidente Prudente - SP, Brazil.
    Divided nevus, also known as kissing nevus, is a rare variant of congenital melanocytic nevi in which there are two adjacent nevi in areas of the body that undergo embryonic cleavage. The original description of this type of lesion was on the eyelid. The location on the penis is even rarer, with only 17 case reports in the literature so far, and only one of them described the dermoscopic findings. Read More

    Congenital longitudinal melanonychia: a case report.
    Acta Dermatovenerol Alp Pannonica Adriat 2017 Dec;26(4):119-120
    Department of Dermatology, University Medical Centre, Maribor, Slovenia.
    Congenital longitudinal melanonychia is rarely seen in Caucasians and poses a diagnostic dilemma. It is characterized by the presence of hyperpigmented brown or black streaks visible in the nail plate. We present the case of 20-month-old boy with congenital longitudinal melanonychia, the diagnostic procedure, and management. Read More

    Genomic analysis of a case of agminated Spitz nevi and congenital-pattern nevi arising in extensive nevus spilus.
    J Cutan Pathol 2018 Feb 17;45(2):180-183. Epub 2017 Dec 17.
    Department of Anatomic Pathology, Moffitt Cancer Center, Tampa, Florida.
    Nevus spilus is a melanocytic neoplasm characterized by a tan macular background punctuated by multiple hyperpigmented macules or papules that represent various types of nevi. These include junctional and compound nevi, Spitz nevi, and rarely blue nevi. We report a unique case of widespread, multiple nevi spili giving rise to agminated Spitz nevi and congenital-pattern compound nevi. Read More

    Unusual Cause of West Syndrome.
    J Pediatr Neurosci 2017 Jul-Sep;12(3):288-290
    Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
    Schimmelpenning-Feuerstein-Mims syndrome is a congenital neurocutaneous disorder, comprising of organoid epidermal nevus with a broad spectrum of multiorgan dysfunction (neurologic, skeletal, cardiovascular, ophthalmic, and urologic) secondary to postzygotic mutation in the early embryonic period. Predominant neurological manifestations include epilepsy, intellectual impairment, and focal deficits. Here, we report a 3-year-old girl who presented with epileptic spasms and had a characteristic linear sebaceous nevus. Read More

    Congenital Non-Neural Granular Cell Tumor Mimicking Nevus Lipomatosus Superficialis.
    Ann Dermatol 2017 Dec 30;29(6):776-778. Epub 2017 Oct 30.
    Department of Dermatology, Hallym University Sacred Heart Hospital, Anyang, Korea.
    A 4-month-old infant presented with asymptomatic soft nodules on his right forearm, which had developed since birth. On the suspicion of nevus lipomatosus superficialis (NLS), biopsy was performed. Histopathologic findings showed monomorphic polygonal cells with abundant granular cytoplasm. Read More

    A case report of the first application of culture epithelial autograft (JACE®) for giant congenital melanocytic nevus after its approval in Japan.
    J Artif Organs 2017 Nov 17. Epub 2017 Nov 17.
    Department of Plastic and Reconstructive Surgery, Kansai Medical University, 2-5-1 Shin-machi, Hirakata, 573-1010, Japan.
    In Japan, the JACE® cultured epidermal autograft (CEA) was approved and covered by public healthcare insurance for use in the treatment of giant congenital melanocytic nevus (GCMN) in 2016. We herein report the results of the application of JACE® after curettage and Q-switched ruby laser therapy. The current patient was the first patient with GCMN to be treated with JACE® since its approval. Read More

    Keratinocyte Sonic Hedgehog Up-regulation Drives the Development of Giant Congenital Nevi via Paracrine Endothelin-1 Secretion.
    J Invest Dermatol 2017 Nov 11. Epub 2017 Nov 11.
    Drug Discovery Group, QIMR Berghofer Medical Research Institute, Herston, Queensland, Australia. Electronic address:
    Giant congenital nevi are associated with clinical complications such as neurocutaneous melanosis and melanoma. Virtually nothing is known about why some individuals develop these lesions. We previously identified the sonic hedgehog (Shh) pathway regulator Cdon as a candidate nevus modifier gene. Read More

    Successful and safe use of Q-switched lasers in the treatment of nevus of Ota in children with phototypes IV-VI.
    Lasers Surg Med 2018 Jan 13;50(1):56-60. Epub 2017 Nov 13.
    Laser & Skin Surgery Center of New York, 317 East 34th Street, New York, New York, 10016.
    Objective: Nevus of Ota represents congenital dermal melanocytosis in a trigeminal distribution, most commonly occurring in Asian individuals and other individuals with skin of color. Evidence suggests early treatment is beneficial. Multiple reports have shown efficacy and safety of Q-switched laser treatment in adults. Read More

    Nevus Comedonicus: Case Report.
    Case Rep Dermatol 2017 Sep-Dec;9(3):184-189. Epub 2017 Oct 4.
    Sector of Dermatology and Post-Graduation Course, University Hospital and School of Medicine, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil.
    Nevus comedonicus is a rare cutaneous anomaly, characterized by the clustering of papules with firm, blackened horny buffers. It in general has a zosteriform pattern and affects both genders equally. In some patients, there is formation of cysts, abscesses, and fistulas, aggravating its psychosocial impact. Read More

    Eccrine-Centric Melanocytic Nevus.
    Am J Dermatopathol 2017 Oct 27. Epub 2017 Oct 27.
    *Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, Miami, FL; †Metropolitan Dermatologic Surgery, Atlanta, GA; ‡Pathology and Laboratory Medicine Service, James A. Haley VA Hospital, Tampa, FL; §Georgia Dermatopathology, Savannah, GA; and ¶Department of Dermatology, University of Florida, Gainesville, FL.
    Benign melanocytic neoplasms present with a diverse array of well-known histopathologic patterns. It is imperative to recognize the benign patterns to render accurate diagnoses. We describe here an interesting and hitherto not described low-power architectural pattern of a benign melanocytic lesion: eccrine-centric melanocytic nevus. Read More

    Melanin pigments in the melanocytic nevus regress spontaneously after inactivation by high hydrostatic pressure.
    PLoS One 2017 1;12(11):e0186958. Epub 2017 Nov 1.
    Department of Biomedical Engineering, National Cerebral and Cardiovascular Center Research Institute, Suita, Osaka, Japan.
    We report a novel treatment for giant congenital melanocytic nevi (GCMN) that involves the reuse of resected nevus tissue after high hydrostatic pressurization (HHP). However, the remaining melanin pigments in the inactivated nevus tissue pose a problem; therefore, we performed a long-term observation of the color change of inactivated nevus tissue after HHP. Pressurized nevus specimens (200 MPa group, n = 9) and non-pressurized nevus tissues (control group, n = 9) were subcutaneously implanted into nude mice (BALB/c-nu) and then harvested 3, 6, and 12 months later. Read More

    Clinicopathologic characteristics of early-onset Becker's nevus in Korean children and adolescents.
    Int J Dermatol 2018 Jan 31;57(1):55-61. Epub 2017 Oct 31.
    Department of Dermatology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
    Background: Becker's nevus (BN) presents as a hairy patch or plaque with or without proliferation of the dermal smooth muscles. BN has been described as acquired as found in a similar entity, congenital smooth muscle hamartoma (CSMH). This study was aimed at evaluating the clinicopathological aspects of BN in Korean cases in differential diagnosis with CSMH. Read More

    THE NON-NEURONAL AND NON-MUSCULAR EFFECTS OF BOTULINUM TOXIN: A Graceful Opportunity for a Deadly Molecule to Treat a Human Disease in the Skin and Beyond.
    Br J Dermatol 2017 Oct 31. Epub 2017 Oct 31.
    Department of Dermatology, University of California, Irvine, CA, USA.
    There is growing evidence that botulinum neurotoxins (BoNTs) exhibit biological effects on various human cell types with a host of associated clinical implications. The BoNT receptors and intracellular targets are not unique for neurotransmission. They have been found in both neuronal and non-neuronal cells, but there are differences in the way BoNT binds to, and acts on neuronal vs. Read More

    Dermoscopy of congenital melanocytic nevi: a ten-year follow-up study and comparative analysis with acquired melanocytic nevi arising in prepubertal age.
    Eur J Dermatol 2017 Oct;27(5):505-510
    Department of Experimental and Clinical Medicine, Institute of Dermatology, University of Udine, Italy.
    Dermoscopic characteristics of congenital melanocytic nevi (CMN) have been reported, however, dermoscopic variation during long-term follow-up and direct comparative analyses with acquired melanocytic nevi (AMN) are poorly documented. To assess dermoscopic changes of CMN (including lesions present at birth or appearing within the first two years of age) after a long-term period and evaluate possible dermoscopic differences with AMN arising during prepubertal age. We re-analysed clinical and dermoscopic features of CMN, investigated ten years earlier. Read More

    Laser treatment of congenital melanocytic naevi: a systematic review.
    Br J Dermatol 2017 Oct 27. Epub 2017 Oct 27.
    Netherlands Institute for Pigment Disorders, Department of Dermatology, University of Amsterdam, the Netherlands.
    Recent studies on congenital melanocytic naevi (CMN) indicate a lower risk of melanoma than has been previously assumed. As a result, the treatment paradigm in CMN has shifted from complete removal to cosmetically acceptable, less invasive treatment options, such as laser treatment. Our objective was to review systematically the efficacy and safety of laser therapy for CMN. Read More

    Acquisition of Somatic NRAS Mutations in Central Nervous System Melanocytes: A Predisposing Risk Factor to Primary Melanoma of the Central Nervous System, a Frequently Forgotten Pitfall in Congenital Nevi.
    Am J Dermatopathol 2017 Oct 17. Epub 2017 Oct 17.
    Departments of *Pathology, and †Dermatology, Hospital Universitario 12 de Octubre, Universidad Complutense, Madrid, Spain.
    Congenital melanocytic nevi (CMN) are benign melanocytic proliferations that are usually present at birth. A somatic mosaicism for an NRAS point mutation is responsible for the several phenotypic abnormalities that may be associated with congenital nevi. We report the case of a 7-year-old boy with a proliferative nodule (PN) arising in a Giant CMN completely excised and with several visceral and intraspinal melanoma metastases with no evidence of primary cutaneous melanoma. Read More

    Serial Tissue Expansion at the Same Site in Pediatric Patients: Is the Subsequent Expansion Faster?
    Arch Plast Surg 2017 Nov 26;44(6):523-529. Epub 2017 Oct 26.
    Department of Plastic and Reconstructive Surgery, Institute of Human-Environment Interface Biology, Seoul National University College of Medicine, Seoul, Korea.
    Background: Serial tissue expansion is performed to remove giant congenital melanocytic nevi. However, there have been no studies comparing the expansion rate between the subsequent and preceding expansions. In this study, we analyzed the rate of expansion in accordance with the number of surgeries, expander location, expander size, and sex. Read More

    Proliferative Melanocytic Nodule on Small Congenital Nevus During Treatment With Etanercept.
    Am J Dermatopathol 2017 Oct 9. Epub 2017 Oct 9.
    *Dermatology, Department of Experimental, Diagnostic and Specialty Medicine, University of Bologna, Bologna, Italy †Department of Pathology, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy.

    Spinal Cavernous Angioma Associated with Klippel-Trenaunay-Weber Syndrome: Case Report and Literature Review.
    World Neurosurg 2018 Jan 17;109:333-337. Epub 2017 Oct 17.
    Department of Neurological Surgery, Nippon Medical School, Tokyo, Japan.
    Background: Klippel-Trenaunay-Weber syndrome (KTWS) is a rare congenital vascular disorder characterized by the classic triad of cutaneous nevi, venous varicosities, and osseous and soft tissue hypertrophy of the affected limb. Various vascular anomalies of the central nervous system have also been described in patients with KTWS. The English language literature to date contains 6 reports of associations between KTWS and spinal cord cavernous angioma (CA), but management of these patients has not been well described. Read More

    Multiple Tissue Expansion for Giant Congenital Melanocytic Nevus.
    Ann Plast Surg 2017 Dec;79(6):e37-e40
    From the *Hospital Infantil Miguel Servet, Zaragoza; and †Hospital La Paz, Madrid, Spain.
    Objective: Tissue expansion is the preferred technique for the treatment of giant congenital melanocytic nevus (GCMN) located on the face, abdomen, and legs. We believe that the use of multiple tissue expanders in the same surgery is a safe and effective technique for the treatment of GCMN.

    Materials And Methods: A total of 14 patients were treated between 2013 and 2016 for GCMN removal with placement in the same surgery of 3 or more tissue expanders. Read More

    Epidermal nevus syndromes: New insights into whorls and swirls.
    Pediatr Dermatol 2018 Jan 16;35(1):21-29. Epub 2017 Oct 16.
    Department of Dermatology, University of California, San Francisco, Santa Rosa, CA, USA.
    Knowledge of the molecular underpinnings of many epidermal nevi and epidermal nevus syndrome has expanded rapidly in recent years. In this review and update on epidermal nevus syndrome, we will cover recent genetic discoveries involving epidermal nevi, including nevus sebaceus, keratinocytic epidermal nevus, nevus comedonicus, congenital hemidysplasia with ichthyosiform nevus and limb defects syndrome, phakomatosis pigmentokeratotica, Becker's nevus, porokeratotic adnexal ostial nevus, inflammatory linear verrucous epidermal nevi, and cutaneous-skeletal hypophosphatemia syndrome. We will discuss how newly defined mutations relate to the biology reflected in the cutaneous patterns seen in these mosaic disorders and how new molecular data has informed our understanding of these diseases and shaped management decisions. Read More

    The advantages of intermediate-tier, inter-optometric referral of low risk pigmented lesions.
    Ophthalmic Physiol Opt 2017 Nov;37(6):661-668
    Centre for Eye Health, Sydney, NSW, Australia.
    Purpose: Pigmented ocular lesions are commonly encountered by eye-care professionals, and range from benign to sight or life-threatening. After identifying a lesion, the primary care professional must establish the likely diagnosis and decide either to reassure, to monitor or to refer. The increasing use of ocular imaging technologies has contributed to an increase in the detection rate of pigmented lesions and a higher number of referrals, which may challenge existing pathways of health-care delivery. Read More

    Multimodal Imaging in Klippel-Trénaunay-Weber Syndrome: Clinical Photography, Computed Tomoangiography, Infrared Thermography, and 99mTc-Phytate Lymphoscintigraphy.
    Clin Nucl Med 2017 Dec;42(12):976-977
    From the *Department of Thoracic and Cardiovascular Surgery and †Nuclear Medicine, Jeju National University Hospital, Jeju National University School of Medicine, Jeju, Korea.
    We report the case of a 19-year-old man who presented with a 12-year history of progressive fatigue, feeling hot, excessive sweating, and numbness in the left arm. He had undergone multimodal imaging and was diagnosed as having Klippel-Trénaunay-Weber syndrome (KTWS). This is a rare congenital disease, defined by combinations of nevus flammeus, venous and lymphatic malformation, and hypertrophy of the affected limbs. Read More

    THE FAST CLINICAL EVOLUTION OF A SPITZ NEVUS: THREE-YEAR FOLLOW-UP OF A CHILD.
    Rev Paul Pediatr 2017 Oct-Dec;35(4):476-479. Epub 2017 Sep 21.
    Universidade Estadual de Campinas, Campinas, SP, Brasil.
    Objective: To report the clinical evolution and handling of a Spitz nevus, from its initial flat feature to becoming an irregular, nodular, reddish lesion.

    Case Description: Female child, phototype II, with a small congenital nevus on the left lower limb and other sustained small nevi. The patient went through annual clinical and dermoscopic evaluations between the ages of three and seven, period during which the nevi located on the left thigh grew rapidly. Read More

    Congenital midline nasal anomalies.
    Proc (Bayl Univ Med Cent) 2017 Oct;30(4):459-460
    Division of Neonatology, Department of Pediatrics (Vora, Kalagiri, Shetty, Raju, Beeram), Department of Pathology (Guillory), and Department of Pediatric Otolaryngology (Powitzky), Baylor Scott & White Health and Texas A&M Health Science Center College of Medicine, Temple, Texas.
    Congenital midline nasal anomalies are rare, with a prevalence of 1 in 20,000 to 40,000 births and with 5% to 7% of them being nasal glioma. Differential diagnoses of nasal anomalies include nasal dermoid cysts, gliomas, encephaloceles, nasal polyps, and some other rare anomalies. Due to current medical technological advancements, most of these anomalies are easily correctable, though delaying management may lead to fatal effects. Read More

    Congenital melanocytic nevus mimicking a turban tumour in an 18-year-old Filipino male.
    BMJ Case Rep 2017 Sep 23;2017. Epub 2017 Sep 23.
    Department of Surgery, Division of Plastic Surgery, University of the Philippines Manila, Philippine General Hospital, Manila, Philippines.
    Congenital melanocytic nevi (CMN) are nevi that are present at birth or arise within the first few weeks of life. They are often found on the trunk, head and neck and extremities. We report herein an unusual presentation of a CMN as a cerebriform tumour presenting as secondary cutis verticis gyrata on the scalp of an 18-year-old Filipino male. Read More

    Congenital Cutaneous Panadnexal Papillomatous Hamartomas in a Calf.
    J Comp Pathol 2017 Aug - Oct;157(2-3):183-187. Epub 2017 Sep 6.
    Department of Veterinary Clinical Sciences, Faculty of Health and Medical Sciences, University of Copenhagen, Dyrlægevej 68, Frederiksberg C, Denmark.
    Several cauliflower-like alopecic masses arose on the head of an otherwise healthy, full-term newborn Jersey × Belgian blue heifer, which was humanely destroyed shortly after birth due to the severity of the skin lesions. Microscopically, the masses were composed of multiple papillary projections displaying well-differentiated sebaceous glands surrounded by a moderate number of well-developed sweat glands, as well as embryonic and fully developed, but dysplastic hair follicles. Thick branching connective tissue stalks supported these adnexal components. Read More

    Pathology and genomics of pediatric melanoma: A critical reexamination and new insights.
    Pediatr Blood Cancer 2018 Feb 12;65(2). Epub 2017 Sep 12.
    Department of Pathology, Institute Curie and Faculty of Medicine, University of Paris Descartes, Paris, France.
    The clinicopathologic features of pediatric melanoma are distinct from those of the adult counterpart. For example, most childhood melanomas exhibit a uniquely favorable biologic behavior, save for those arising in large/giant congenital nevi. Recent studies suggest that the characteristically favorable biologic behavior of childhood melanoma may be related to extreme telomere shortening and dysfunction in the cancer cells. Read More

    Pigmented Trichoblastoma of Nose: An Unusual Occurrence.
    J Clin Diagn Res 2017 Jul 1;11(7):MD09-MD10. Epub 2017 Jul 1.
    Junior Resident, Department of Ear, Nose and Throat, Sri Siddhartha Medical College, Agalakote, Karnataka, India.
    Nevus sebaceus of Jadassohn is a congenital tumour affecting the scalp and face. It is usually presented as a pigmented patch or plaque. It is a complex cutaneous hamartoma which involves pilosebaceous follicle, epidermis and adnexal structures. Read More

    Deep Inferior Epigastric Perforator Free Flaps for Use in Complicated Groin Wound Repair: A Case Report of Severe Groin Scar Contracture and Review of Pedicled and Free Flaps in Groin Wound Repair.
    J Hand Microsurg 2017 Aug 1;9(2):101-106. Epub 2017 Aug 1.
    Department of Microvascular and Reconstructive Surgery, MD Anderson Cancer Center Orlando, Orlando, Florida, United States.
    The use of pedicled and free flaps for tissue transfer and coverage has become a common practice in modern plastic surgery. An area that presents considerable challenge for tissue coverage is the groin. Defects in this area are complicated by issues such as prior surgery; scar contracture; extension of the defect beyond the borders of the groin; radiation damage; high probability of infection; and involvement of vital underlying structures, the genitalia, and perineal and perianal area. Read More

    A case of congenital lipomatous overgrowth, vascular malformations, epidermal nevi, spinal/skeletal anomalies and/or scoliosis syndrome with lipoatrophy as an important clinical manifestation.
    Pediatr Dermatol 2017 Nov 22;34(6):735-736. Epub 2017 Aug 22.
    Division of Paediatric Dermatology, CHU de Québec, Québec, Canada.
    Congenital lipomatous overgrowth, vascular malformations, epidermal nevi, spinal/skeletal anomalies and/or scoliosis syndrome is a PIK3CA-related overgrowth spectrum presenting with congenital, asymmetric, disproportionate overgrowth associated with dysregulated adipose tissue, enlarged bony structures, and mixed primarily truncal vascular malformations. We present this case to raise awareness that very thin body habitus (lipoatrophy) contrasting with areas of overgrowth can be an important clinical feature of this syndrome and, if not recognized, can lead to unnecessary investigations. Read More

    Wilms tumor screening in diffuse capillary malformation with overgrowth and macrocephaly-capillary malformation: A retrospective study.
    J Am Acad Dermatol 2017 Nov 16;77(5):874-878. Epub 2017 Aug 16.
    Department of Dermatology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts; Vascular Anomalies Center, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts. Electronic address:
    Background: CLOVES (congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and skeletal anomalies) syndrome is associated with regional bony and/or soft tissue overgrowth, capillary malformation, and an increased risk for Wilms tumor.

    Objective: To evaluate the frequency of Wilms tumor in patients with 2 similar conditions: diffuse capillary malformation with overgrowth (DCMO) and macrocephaly-capillary malformation (M-CM).

    Methods: Culling our Vascular Anomalies Center database, we retrospectively reviewed patients in whom DCMO and M-CM had been diagnosed and who were evaluated between 1998 and 2016 for possible development of Wilms tumor. Read More

    Transcatheter embolization of persistent embryonic veins in venous malformation syndromes.
    J Vasc Surg Venous Lymphat Disord 2017 Sep;5(5):749-755
    Division of Hematology and Oncology, Department of Pediatrics, Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ; Cancer Institute of New Jersey at the Bristol-Myers Squibb Children's Hospital, Robert Wood Johnson University Hospital, New Brunswick, NJ.
    Persistent embryonic veins represent a major source of venous hypertension and morbidity in venous malformation syndromes, such as Klippel-Trénaunay syndrome and congenital lipomatous overgrowth, vascular malformations, epidermal nevus, and skeletal deformities syndrome. Surgical stripping and phlebectomy are the most commonly reported alternatives to compression therapy for refractory cases. These techniques, although effective in those patients who meet the necessary anatomic criteria, can be associated with bleeding, wound-related complications, and recurrence. Read More

    Combined Melanocytic Nevus, Superficial Congenital and Deep Penetrating Types with Fibroepithelioma of Pinkus, Collision Tumor - A Case Report.
    S D Med 2017 Aug;70(8):363-365
    Sanford Pathology, Sioux Falls, South Dakota.
    We present a case of collision tumor composed of a combined melanocytic nevus with superficial congenital and deep penetrating components and a fibroepithelioma of Pinkus on the left lumbar back of a 21-year-old male. He presented to the dermatologist for evaluation of numerous moles, and the lesion in question was described as a brown variegated papule with slightly irregular shape and irregular borders. This case is being reported as it is very unusual to see a fibroepithelioma of Pinkus in conjunction with a melanocytic lesion. Read More

    [Smooth muscle hamartoma in volar skin].
    Hautarzt 2017 Nov;68(11):916-918
    Klinik für Dermatologie, Venerologie und Allergologie, Universität Leipzig, Philipp-Rosenthal-Str. 23, 04103, Leipzig, Deutschland.
    We report the case of a 12-year-old girl with a smooth muscle hamartoma of the right index finger. Smooth muscle hamartoma (SMH) is a congenital, relatively common disorder typically with predominance of autochthonal arrector pili muscles. An SMH can also rarely originate from smooth muscles of vessels in palmoplantar skin with the absence of pilosebaceous units. Read More

    Single-Stage Reconstruction of Eyebrow Defect Using a V-Y Advancement Pedicle Flap Based on the Orbicularis Oculi Muscle.
    J Craniofac Surg 2017 Sep;28(6):e521-e522
    Department of Plastic and Reconstructive Surgery, The First Bethune Hospital of Jilin University, Changchun, China.
    Eyebrows play an important role in face expression and facial mimics by virtue of muscle contraction. Defects or deformity of the eyebrows result in abnormal facial expressions, and may lead to aesthetic issues for patients. The objective of this study is to report the case of a patient, with a congenital skin pigmented nevus at the right side of the eyebrow treated with direct surgical resection and followed by immediate reconstruction of the eyebrow with a V-Y advancement pedicle flap based on the orbicularis oculi muscle. Read More

    Congenital and infantile malignant melanoma of the scalp: A systematic review.
    Ann Med Surg (Lond) 2017 Sep 19;21:93-95. Epub 2017 Jul 19.
    Section of Neurosurgery, The Aga Khan University Hospital, Karachi, Pakistan.
    Congenital and infantile malignant melanomas are rare and typically carry poor prognosis. The purpose of this article was to review the data on congenital and infantile malignant melanomas of the scalp in order to understand its presentation, diagnosis, management, and outcomes of congenital melanoma of scalp. We searched PubMed, CINAHL and Cochrane databases. Read More

    Giant Congenital Melanocytic Nevus (GCMN) - A New Hope for Targeted Therapy?
    Open Access Maced J Med Sci 2017 Jul 22;5(4):549-550. Epub 2017 Jul 22.
    Department of Dermatology and Venereology, Medical University of Plovdiv, 15A Vasil Aprilov blvd., 4002 Plovdiv, Bulgaria.
    We present a 6-month-old male patient, who was consulted with dermatologist by his parents, because of a pigmented lesion, present since birth, covering almost the all skin of the back and buttocks. A sharply bordered, unequally coloured congenital pigmented nevus, measuring approximately 21 cm in diameter was observed in the whole body skin examination. The lesion was affecting the lower 2/3 of the skin of the back and the top half of the gluteus area, extending to the lateral part of the tors, forward the abdomen and the upper lateral part of the hips, composed by multiple darker-pigmented nests and several lighter areas, with single depigmented zones, hairy surface, irregularly infiltrated on palpation. Read More

    Late Onset Achromatic Melanoma Arising in a Giant Congenital Melanocytic Nevus.
    Open Access Maced J Med Sci 2017 Jul 18;5(4):533-534. Epub 2017 Jul 18.
    Department of Dermatology and Allergology, Academic Teaching Hospital Dresden-Friedrichstadt, Friedrichstrasse 41, 01067, Dresden, Germany.
    A 61-year-old woman, with a lifelong history of a giant congenital melanocytic nevus in the occipital region with secondary development of giant melanoma is presented. Surgical excision was performed, and the histopathological evaluation confirmed the diagnosis of Giant Malignant Melanoma (GMM) with a maximum tumour thickness of 16 mm. Nowadays, there is tremendous uncertainty regarding how giant congenital melanocytic nevi (GCMN) should be treated. Read More

    Clinical and sonographic features of pediatric soft-tissue vascular anomalies part 2: vascular malformations.
    Pediatr Radiol 2017 Aug 4;47(9):1196-1208. Epub 2017 Aug 4.
    Department of Diagnostic Imaging, The Hospital for Sick Children, 555 University Ave., Toronto, ON, M5G 1X8, Canada.
    Vascular malformations are a heterogeneous group of entities, many of which present in the pediatric age group. Sonography plays a major role in the management of children with these vascular anomalies by providing information that helps in diagnosing them, in assessing lesion extent and complications, and in monitoring response to therapy. The interpretation of sonographic findings requires correlation with clinical findings, some of which can be easily obtained at the time of scanning. Read More

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