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    707 results match your criteria Epidermal Nevus Syndrome

    1 OF 15

    Papillary transitional cell bladder carcinoma and systematized epidermal nevus syndrome.
    Cutis 2017 Jan;99(1):61-64
    Department of Dermatology, State University of New York at Downstate Medical Center, Brooklyn, USA.
    Epidermal nevus syndrome (ENS), also known as Solomon syndrome, is a rare neurocutaneous disorder defined by mosaicism. Epidermal nevus syndrome may be associated with a variety of systemic findings, several of which have been described in the literature, including but not limited to central nervous system abnormalities and internal malignancies. There is a paucity of reports of patients with both epidermal nevi and papillary transitional cell bladder carcinoma in the literature. Read More

    Papular Epidermal Nevus with "Skyline" Basal Cell Layer Syndrome - Natural Course: Case Report and Literature Review.
    Case Rep Dermatol 2017 Jan-Apr;9(1):1-5. Epub 2017 Jan 10.
    Department of Dermatology, University Hospital Basel, Basel, Switzerland; Department of Biomedicine, University Hospital Basel, Basel, Switzerland.
    Papular epidermal nevus with "skyline" basal cell layer (PENS) is a very rare type of keratinocytic nevus and is associated with extracutaneous findings such as neurological symptoms in about 50% of the cases. Therefore, it is also referred to as PENS syndrome. Clinically visible hyperkeratotic papules and plaques already appear at birth or shortly thereafter, while neurological symptoms such as epilepsy and mental retardation manifest themselves during childhood. Read More

    Dermoscopy of Papular Epidermal Nevus with Skyline Basal Cell Layer.
    Pediatr Dermatol 2017 Jan 23. Epub 2017 Jan 23.
    Division of Dermatology "U.O. Multizonale", Outpatient Consultation for Rare Diseases, "S. Chiara" Hospital, Trento, Italy.
    Papular epidermal nevus with skyline basal cell layer (PENS) is a newly described keratinocytic nevus whose dermoscopic characteristics have not been clarified. We used a dermatoscope to investigate the multiple PENSs of a patient with PENS syndrome. All the lesions shared a common dermoscopic homogeneous white pattern surrounded by peripheral, slightly dotted hyperpigmentation. Read More

    First Reported Case of 'Epidermal Nevus Syndrome' with a Triad of Central Nervous System Deformities.
    Cureus 2016 Dec 6;8(12):e916. Epub 2016 Dec 6.
    Internal Medicine, Griffin Hospital.
    Epidermal nevus syndrome (ENS) is a term used to describe the occurrence of an epidermal nevus in association with other extra-cutaneous developmental anomalies, most commonly involving the nervous and musculoskeletal systems. The nevus is classified on the basis of the main component which may be keratinocytic, sebaceous, follicular, apocrine, or eccrine. Most patients who present with ENS is at the time of birth, though some become apparent later in life. Read More

    Becker's Nevus Syndrome in a Pediatric Female Patient.
    Case Rep Pediatr 2016 6;2016:3856518. Epub 2016 Nov 6.
    Pediatric Endocrinology Department, Universidad de Antioquia, Medellin, Colombia.
    Becker's nevus syndrome is part of the epidermal nevus syndromes and has been described with a phenotype that includes Becker's nevus, ipsilateral breast hypoplasia, and variable skeletal malformations. It is more frequent in males than in females (5 : 1) but is more relevant in females. The diagnosis is clinically based and the skin lesion must be present and no other numbered criteria have been established, but with more criteria being present the possibility of the diagnosis is higher. Read More

    Becker Nevus Syndrome of the Lower Body: One Case and Review of the Literature.
    Acta Derm Venereol 2016 Nov 24. Epub 2016 Nov 24.
    Department of Dermatology, Venereology and Allergology, University Hospital Würzburg, Josef-Schneider-Str. 2, DE-97080 Würzburg, Germany.
    Becker naevus syndrome is a rare epidermal naevus syndrome defined by the co-occurrence of a Becker naevus with various cutaneous, muscular and skeletal anomalies. In the majority of cases, abnormalities exclusively consist of ipsilateral hypoplasia of the breast, areola and/or nipple in addition to the naevus. Here, we report on a 42-year-old woman with an extensive Becker naevus reaching from the left buttock to the left calf verified on histological examination. Read More

    Cutaneous Horn of the Eyelid in 13 Cases.
    Ophthal Plast Reconstr Surg 2016 Nov 2. Epub 2016 Nov 2.
    *Ocular Oncology Service and †Department of Ophthalmic Pathology, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania, U.S.A.
    Introduction: Cutaneous horn of the eyelid is uncommon. The authors evaluate the features of benign, premalignant, and malignant tumors at the base of cutaneous horn of the eyelid.

    Objective: To describe the clinical and histopathologic features of cutaneous horn of the eyelid. Read More

    Nevus Sebaceous of Jadassohn With Eight Secondary Tumors of Follicular, Sebaceous, and Sweat Gland Differentiation.
    Am J Dermatopathol 2016 Nov;38(11):861-866
    *Shanghai Skin Disease Hospital, Tongji University, Shanghai, China †Ackerman Academy of Dermatopathology, New York, NY ‡Department of Dermatology, Kasr Alainy Faculty of Medicine, Cairo University, Egypt.

    [Indications of the expansion in pediatric surgery. Experience of 30years and literature review].
    Ann Chir Plast Esthet 2016 Oct 8;61(5):740-749. Epub 2016 Aug 8.
    Service de chirurgie plastique, esthétique et reconstructrice, hôpital Salengro, rue Émile-Laine, 59037 Lille cedex, France. Electronic address:
    The expansion of soft tissue, especially skin, is an old and physiological process to increase the skin reserve allowing excision while coveraging of the resulting loss of substance. Easy in principle, this process is subjected to constraints in children requiring precise planning and rigorous technical procedure. Between 1990 and 2016, we performed 293 expansion protocols with 411 implants in 244 children. Read More

    Combined endovascular and microsurgical treatment of a complex spinal arteriovenous fistula associated with CLOVES syndrome in an adult patient.
    J Clin Neurosci 2016 Dec 8;34:232-234. Epub 2016 Oct 8.
    Department of Neurosurgery, Barrow Neurological Institute, St. Joseph's Hospital and Medical Center, Phoenix, AZ, United States. Electronic address:
    CLOVES (congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and scoliosis/skeletal/spinal anomalies) syndrome is a congenital and almost exclusively pediatric syndrome associated with vascular malformations of the neuroaxis. We report the case of a complex spinal arteriovenous fistula in an adult woman with CLOVES syndrome treated using a multidisciplinary approach with endovascular embolization and microsurgical technique, and review the medical literature on this disease. Read More

    Syringocystadenocarcinoma Papilliferum In Situ, a Variant of Cutaneous Adenocarcinoma In Situ: A Case Report With Literature Review.
    Am J Dermatopathol 2016 Oct;38(10):762-5
    *Department of Dermatology, The Third Xiangya Hospital of Central South University, Changsha, China; †Department of Surgery, and ‡Department of Pathology and Laboratory Medicine and Department of Dermatology, Hofstra Northwell School of Medicine, NY.
    Syringocystadenocarcinoma papilliferum in situ, a variant of cutaneous adenocarcinoma in situ, is extremely rare. Only 9 cases have been published to date with 2 cases demonstrating pagetoid epidermal involvement. In this study, we report a case of syringocystadenocarcinoma papilliferum in situ with pagetoid epidermal involvement arising from a long-standing nevus sebaceus on the scalp of a 60-year-old woman. Read More

    Epidermal Nevus Presenting in a Pediatric Patient With Pallister-Killian Syndrome.
    Skinmed 2016 1;14(3):230-1. Epub 2016 Jun 1.
    Department of Dermatology, University of South Florida College of Medicine, Tampa, FL.
    A six-year-old boy with Pallister-Killian syndrome (PKS) presented to the clinic with extensive lesions on his body (Figure 1). The patient was not born with the lesions but began developing them on the head and neck, extending to his lower extremities, at 2 years of age. These lesions had been evaluated by his primary care physician and were previously treated with desonide and ketoconazole cream with little improvement. Read More

    Cutaneous skeletal hypophosphatemia syndrome: clinical spectrum, natural history, and treatment.
    Osteoporos Int 2016 Dec 6;27(12):3615-3626. Epub 2016 Aug 6.
    Skeletal Clinical Studies Unit, Craniofacial and Skeletal Disease Branch, National ADDRESSES, references BRACKETS, National Institute of Dental and Craniofacial Research, National Institutes of Health, 30 Convent Drive, Room 228, MSC 4320, Bethesda, MD, 20892-4320, USA.
    Cutaneous skeletal hypophosphatemia syndrome (CSHS), caused by somatic RAS mutations, features excess fibroblast growth factor-23 (FGF23) and skeletal dysplasia. Records from 56 individuals were reviewed and demonstrated fractures, scoliosis, and non-congenital hypophosphatemia that in some cases were resolved. Phosphate and calcitriol, but not skin lesion removal, were effective at controlling hypophosphatemia. Read More

    Cutaneous skeletal hypophosphatemia syndrome (CSHS) is a multilineage somatic mosaic RASopathy.
    J Am Acad Dermatol 2016 Aug;75(2):420-7
    Departments of Dermatology, Pathology, and Genetics, Yale University School of Medicine, New Haven, Connecticut. Electronic address:
    Background: We recently demonstrated multilineage somatic mosaicism in cutaneous skeletal hypophosphatemia syndrome (CSHS), which features epidermal or melanocytic nevi, elevated fibroblast growth factor (FGF)-23, and hypophosphatemia, finding identical RAS mutations in affected skin and bone.

    Objective: We sought to: (1) provide an updated overview of CSHS; (2) review its pathobiology; (3) present a new patient with CSHS; and (4) discuss treatment modalities.

    Methods: We searched PubMed for "nevus AND rickets," and "nevus AND hypophosphatemia," identifying cases of nevi with hypophosphatemic rickets or elevated serum FGF-23. Read More

    Coexistent trichilemmoma and trichoblastoma without associated nevus sebaceus.
    J Biol Regul Homeost Agents 2016 Apr-Jun;30(2 Suppl 2):17-20
    Department of Medical 1Department of Pathology, Division of Dermatopathology, University of Virginia Health System, Charlottesville, Virginia.
    Trichilemmoma and trichoblastoma are benign adnexal neoplasms derived from the hair follicle unit. While trichilemmomas are closely associated with the epidermis, trichoblastomas are found within the dermis and subcutaneous tissue. Both tumors have been reported to arise within nevus sebaceus of Jadassohn (NSJ). Read More

    A Second Case of Gobello Nevus Syndrome.
    Case Rep Dermatol 2016 Jan-Apr;8(1):85-90. Epub 2016 Apr 20.
    Dermatology Unit, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, University of Milan, Milan, Italy.
    An uncommon type of epidermal nevus characterized by hyperpigmented hyperkeratotic bands following a Blaschko-linear pattern and generalized follicular hyperkeratosis were observed in a 17-year-old male patient who additionally showed tufted hair folliculitis on the scalp and clinodactyly of the fifth finger of both hands. The combination of epidermal nevus with skeletal abnormalities was first described by Gobello et al. [Dermatology 2000;201:51-55] as a new epidermal nevus syndrome that was named after the first author of this work. Read More

    Four Different Tumors Arising in a Nevus Sebaceous.
    Case Rep Dermatol 2016 Jan-Apr;8(1):75-9. Epub 2016 Apr 20.
    Department of Dermatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan.
    Nevus sebaceous is known by its association with one or more secondary tumors, but more than three multiple tumors arising from a nevus sebaceous is extremely rare. A 67-year-old female presented with a light brown plaque on the back of her head that contained a dome-shaped black node and an erosive lesion. Histopathological examination showed atypical basaloid cells in the black node. Read More

    NAEVUS SEBACEOUS OF JADASSOHN'S OF EYELID.
    J Ayub Med Coll Abbottabad 2015 Oct-Dec;27(4):927-9
    Naevus Sebaceous of Jadassohn is a rarely seen hamartomatous lesion and is a fourth type of sebaceous gland tumour, in which sebaceous glands show nevoid character of growth composed partly or completely of sebaceous glands. A detailed research revealed that very little data is available; no case of this disorder has been reported as an isolated eye lid lesion. Majority of the published cases are associated with systemic involvement. Read More

    A unexpected growth arising within nevus sebaceous of Jadassohn.
    Dermatol Online J 2016 Jan 15;22(1). Epub 2016 Jan 15.
    University Hospital Birmingham.
    The predisposition to epithelial neoplasms in nevus sebaceous is well established; most tumors occur in adults and are benign. Hidradenoma is a relatively rare benign tumor of sweat gland origin that can rarely arise within a nevus sebaceous. We present an interesting case of a hidradenoma and sebaceoma arising within a nevus sebaceous and present a literature review of the 2 conditions. Read More

    Ocular manifestations of genetic skin disorders.
    Clin Dermatol 2016 Mar-Apr;34(2):242-75. Epub 2015 Dec 2.
    The Vision Center, Children's Hospital Los Angeles; Department of Ophthalmology, Keck School of Medicine, University of Southern California, 4650 Sunset Blvd, MS #88, Los Angeles, CA, 90027.
    Genetic skin diseases, or genodermatoses, often have extracutaneous manifestations. Ocular manifestations in particular can have significant clinical implications, like blindness. Other manifestations, such as the corneal opacities that occur in X-linked ichthyosis, are asymptomatic but characteristic of a particular genodermatosis. Read More

    Inflammatory linear verrucous epidermal nevus and regional odontodysplasia: A rare sorority.
    Indian J Dent 2015 Oct-Dec;6(4):203-6
    Department of Oral Medicine and Radiology, Subharti Dental College, Meerut, Uttar Pradesh, India.
    Epidermal nevi are hamartomatous lesion and its association with other developmental defects particularly of the central nervous system, eye and skeletal system are well recognized. We report a rare case of inflammatory linear verrucous epidermal nevus syndrome along with regional odontodysplasia; and to the best of our knowledge this is the second case reported in the literature. Read More

    Nevus Sebaceous and Its Association With Neurologic Involvement.
    Semin Pediatr Neurol 2015 Dec 22;22(4):302-9. Epub 2015 Oct 22.
    University Hospital Policlinico-Vittorio Emanuele, Catania, Italy. Electronic address:
    Several terms are widely used to define cutaneous lesions affecting the epidermis in association with extracutaneous lesions. Recently, based on the wide spectrum of cutaneous epidermal lesions, the various underlying molecular mechanisms and patterns of associated features have led to improved definitions of these disorders. Nevus sebaceous syndrome has been placed under the umbrella term of epidermal nevus syndrome, in which the nevus sebaceous, a congenital hamartomatous lesion of the epidermis, is associated with anomalies involving the brain, eyes, and bones. Read More

    Mosaic Neurocutaneous Disorders and Their Causes.
    Semin Pediatr Neurol 2015 Dec 12;22(4):207-33. Epub 2015 Nov 12.
    Department of Clinical and Experimental Medicine, Section of Pediatrics and Child Neuropsychiatry, University of Catania, Catania, Italy; Department of Biomedical and Biotechnological Sciences, University of Catania, Catania, Italy.
    Neurocutaneous disorders are a heterogeneous group of conditions (mainly) affecting the skin [with pigmentary/vascular abnormalities and/or cutaneous tumours] and the central and peripheral nervous system [with congenital abnormalities and/or tumours]. In a number of such disorders, the skin abnormalities can assume a mosaic patterning (usually arranged in archetypical patterns). Alternating segments of affected and unaffected skin or segmentally arranged patterns of abnormal skin often mirror similar phenomena occurring in extra-cutaneous organs/tissues [eg, eye, bone, heart/vessels, lung, kidney and gut]. Read More

    Basal Cell Carcinoma in Gorlin's Patients: a Matter of Fibroblasts-Led Protumoral Microenvironment?
    PLoS One 2015 22;10(12):e0145369. Epub 2015 Dec 22.
    INSERM U1081-CNRS UMR7284 -UNS, Nice, France.
    Basal cell carcinoma (BCC) is the commonest tumor in human. About 70% sporadic BCCs bear somatic mutations in the PATCHED1 tumor suppressor gene which encodes the receptor for the Sonic Hedgehog morphogen (SHH). PATCHED1 germinal mutations are associated with the dominant Nevoid Basal Cell Carcinoma Syndrome (NBCCS), a major hallmark of which is a high susceptibility to BCCs. Read More

    Basal Cell Carcinoma and Syringocystadenoma Papilliferum Arising in Nevus Sebaceous on Face-A Rare Entity.
    Indian J Dermatol 2015 Nov-Dec;60(6):637
    Department of Pathology, Hind Institute of Medical Sciences, Safedabad, Barabanki, Uttar Pradesh, India.
    Nevus sebaceus of Jadassohn is a congenital cutaneous hamartoma comprising of multiple skin structures. It has the potential to develop into variety of neoplasms of various epidermal adnexal origins. While multiple tumors may occasionally arise, it is unusual to develop two different types of tumor, benign and malignant, to arise simultaneously within a single sebaceus nevus. Read More

    Epidermal nevus syndromes.
    Handb Clin Neurol 2015 ;132:291-316
    Departments of Dermatology and Community and Family Medicine, University of California San Francisco, Santa Rosa, CA, USA. Electronic address:
    The term epidermal nevus syndrome (ENS) has been used to describe the association of epidermal hamartomas and extracutaneous abnormalities. Although many continue to use the term "ENS," it is now understood that this is not one disease, but rather a heterogeneous group with distinct genetic profiles defined by a common cutaneous phenotype: the presence of epidermal and adnexal hamartomas that are associated with other organ system involvement. One commonality is that epidermal nevi often follow the lines of Blaschko and it appears the more widespread the cutaneous manifestations, the greater the risk for extracutaneous manifestations. Read More

    Phenotype/genotype correlations in epidermal nevus syndrome as a neurocristopathy.
    Handb Clin Neurol 2015 ;132:9-25
    Departments of Paediatrics, Pathology (Neuropathology) and Clinical Neurosciences, Calgary, Alberta, Canada.
    Epidermal nevus syndrome (ENS) is a term that encompasses several phenotypes defined by the association of an epidermal nevus with one or more congenital systemic anomalies, mainly ocular, osseous and cerebral. The two most frequent, keratinocytic nevus syndrome and linear sebaceous nevus syndrome, also correspond to the neurological phenotypes. They both exhibit overlapping and distinctive features but same etiology: post-zygotic mosaic mutations in RAS genes. Read More

    [Verrucous variant of porokeratosis of Mibelli as a differential diagnosis of psoriasis vulgaris].
    Hautarzt 2016 Mar;67(3):244-8
    Klinik für Dermatologie, Allergologie und Phlebologie, Klinikum Bremerhaven Reinkenheide, Bremerhaven, Postbrookstr. 103, 27574, Bremerhaven, Deutschland.
    In a 37-year-old man, diagnosis of verrucous porokeratosis could only be made by histological examination. Previously, the skin lesions on the right buttock had been treated by several dermatologists as psoriasis vulgaris. The clinical picture of both dermatoses was characterized by sharply defined, erythematous papules and plaques. Read More

    KRAS G12D mosaic mutation in a Chinese linear nevus sebaceous syndrome infant.
    BMC Med Genet 2015 Oct 31;16:101. Epub 2015 Oct 31.
    The Molecular Genetic Diagnosis Center, Shanghai Key Lab of Birth Defects, Translational Medicine Research Center of Children's Development and Disease, Pediatrics Research Institute, Children's Hospital of Fudan University, Shanghai, 201102, China.
    Background: Linear nevus sebaceous syndrome (LNSS) is a multisystem disorder that includes nevus sebaceous and central nervous system, ocular and skeletal anomalies. We report the first case of a KRAS G12D mosaic mutation in a patient diagnosed with LNSS.

    Case Presentation: A 3-month-old female with a clinical diagnosis of LNSS presented with intermittent epilepsy. Read More

    Secondary neoplasms arising from nevus sebaceus: A retrospective study of 450 cases in Taiwan.
    J Dermatol 2016 Feb 12;43(2):175-80. Epub 2015 Sep 12.
    Department of Dermatology, National Taiwan University Hospital and College of Medicine, Taipei, Taiwan.
    Nevus sebaceus is frequently associated with the development of secondary neoplasms. Incidences of malignant transformation vary among different reports and few data is available regarding Asian populations. We aimed to determine the characteristics of secondary tumors developing from nevus sebaceus in a Taiwanese population and to review the published work. Read More

    Somatic BRAF c.1799T>A p.V600E Mosaicism syndrome characterized by a linear syringocystadenoma papilliferum, anaplastic astrocytoma, and ocular abnormalities.
    Am J Med Genet A 2016 Jan 11;170A(1):189-94. Epub 2015 Sep 11.
    Department of Pediatrics, Tohoku University School of Medicine, Sendai, Miyagi, Japan.
    Genetic mosaicism for somatic mutations of oncogenes is common in genodermatoses, which can be complicated with extra-cutaneous abnormalities. Here we describe an infant with a congenital anaplastic astrocytoma, a linear syringocystadenoma papilliferum, and ocular abnormalities. The BRAF c. Read More

    [Schimmelpenning-Feuerstein-Mims syndrome: a case report].
    Arch Pediatr 2015 Nov 29;22(11):1157-62. Epub 2015 Aug 29.
    Service de dermatologie, CHU Hedi Chaker, 3029 Sfax, Tunisie.
    Schimmelpenning-Feuerstein-Mims syndrome (SFM) is a congenital neurocutaneous disorder characterized by the association of nevus sebaceous with extracutaneous abnormalities. We report a new case of Schimmelpenning-Feuerstein-Mims with aortic coarctation and drug-resistant West syndrome. This case emphasizes the importance of exploring and monitoring patients with nevus sebaceous in order to diagnose associated anomalies. Read More

    A Histological Snapshot of Hypothetical Multistep Progression From Nevus Sebaceus to Invasive Syringocystadenocarcinoma Papilliferum.
    Am J Dermatopathol 2016 Jan;38(1):56-62
    Departments of *Pathology, and †Dermatology, University of Alabama at Birmingham, Birmingham, AL.
    Syringocystadenocarcinoma papilliferum (SCACP) is an extremely rare adnexal neoplasm, believed to arise in a preexisting nevus sebaceus of Jadassohn (NSJ) through a multistep progression process. This hypothetical process involves an NSJ giving rise to syringocystadenoma papilliferum, which then presumably undergoes malignant transformation in rare circumstances to give rise to SCACP in situ, which finally progresses to an invasive SCACP. Of the 30 SCACP cases reported so far, none have documented the process from a birthmark to the final invasive lesion, with histological evidence of each step, in a single tumor. Read More

    Ceruminous adenoma (ceruminoma) arising in a nevus sebaceus of Jadassohn within the external auditory canal of a 3 year-old boy - A case report.
    Int J Pediatr Otorhinolaryngol 2015 Nov 20;79(11):1932-4. Epub 2015 Jul 20.
    Medical University of Warsaw, Department of Pediatric Otolaryngology, Poland.
    Nevus sebaceus of Jadassohn is a congenital yellowish hairless skin lesion, mainly located on the head and neck. A common phenomenon is the coexistence of secondary tumors within the lesion. These are mainly benign tumors, the majority of which are trichoblastoma and syringocystadenoma papilliferum. Read More

    Dermoscopy of tumours arising in naevus sebaceous: a morphological study of 58 cases.
    J Eur Acad Dermatol Venereol 2015 Nov 24;29(11):2231-7. Epub 2015 Aug 24.
    Dermatology Department, Hospital Clinic de Barcelona, Barcelona, Spain.
    Background: Naevus sebaceous is a congenital hamartoma commonly associated with the development of secondary neoplasms. There are sparse data relating to the dermoscopy of tumours arising in naevus sebaceous.

    Objectives: To evaluate the dermoscopic features of a large series of neoplasms arising in naevus sebaceous. Read More

    Klippel-Trenaunay syndrome belongs to the PIK3CA-related overgrowth spectrum (PROS).
    Exp Dermatol 2016 Jan 13;25(1):17-9. Epub 2015 Oct 13.
    Department of Dermatology and Cutaneous Biology, The Sidney Kimmel Medical College at Thomas Jefferson University, Philadelphia, PA, USA.
    Klippel-Trenaunay syndrome (KTS), originally described as a triad of cutaneous capillary malformation, bone and soft-tissue hypertrophy, as well as venous and lymphatic malformations, has been considered by dermatologists as a distinct diagnostic entity. However, cases with KTS have also been reported to have neurological disorders, developmental delay and digital abnormalities, indicating multisystem involvement. Recently, a number of overgrowth syndromes, with overlapping phenotypic features with KTS, have been identified; these include MCAP and CLOVES syndromes as well as fibroadipose hyperplasia. Read More

    Double-Opposing Unilobar Rotation Flaps in the Reconstruction of Moderate-to-Large Defects of the Scalp.
    J Craniofac Surg 2015 Sep;26(6):e523-5
    *University of California, Berkeley, CA.
    Closure of medium-to-large-size defects of the scalp are often associated with unacceptable aesthetic results, wound break down, alopecia, and excessive scarring. The authors present 2 cases of double-opposing unilobar rotation flaps for the reconstruction of large, that is, at least 7 cm diameter, full thickness defects of the scalp. Unlike previously described double flap closures of scalp defects, the double-opposing unilobar rotation flap design are true rotation flaps, which require a Burow triangle excisions and which have a versatility in both width and length of design to accommodate closure of large defects of the scalp. Read More

    Fifty shades of yellow: a review of the xanthodermatoses.
    Int J Dermatol 2015 Oct 30;54(10):1109-23. Epub 2015 Jul 30.
    University of New South Wales, Sydney, New South Wales, Australia.
    The xanthodermatoses consist of a heterogeneous group of cutaneous disorders characterized by the macroscopic yellow hue seen on examination. This hue is attributable to the chemical structure of the accumulating substances within the skin or surrounding tissues. The most common culprits are lipids (cholesterol and triglycerides), elastin, and bilirubin. Read More

    Inactivation of Human Nevus Tissue Using High Hydrostatic Pressure for Autologous Skin Reconstruction: A Novel Treatment for Giant Congenital Melanocytic Nevi.
    Tissue Eng Part C Methods 2015 Nov 21;21(11):1178-87. Epub 2015 Aug 21.
    3 Department of Biomedical Engineering, National Cerebral and Cardiovascular Center Research Institute, Suita, Japan .
    Giant congenital melanocytic nevi are intractable lesions associated with a risk of melanoma. High hydrostatic pressure (HHP) technology is a safe physical method for producing decellularized tissues without chemicals. We have reported that HHP can inactivate cells present in various tissues without damaging the native extracellular matrix (ECM). Read More

    Practical application of new technologies for melanoma diagnosis: Part II. Molecular approaches.
    J Am Acad Dermatol 2015 Jun;72(6):943-58; quiz 959-60
    Department of Dermatology, University of Utah Health Sciences Center, Salt Lake City, Utah; Huntsman Cancer Institute, University of Utah Health Sciences Center, Salt Lake City, Utah. Electronic address:
    The criterion standard for diagnosing cutaneous melanoma continues to be histologic examination. However, classifying some melanocytic lesions by conventional microscopy can be problematic if they exhibit some architectural or morphologic characteristics of both nevus and melanoma. Moreover, histologic appearance does not always predict biologic behavior. Read More

    Keratinocytic epidermal nevus syndrome with Schwann cell proliferation, lipomatous tumour and mosaic KRAS mutation.
    BMC Med Genet 2015 Feb 10;16. Epub 2015 Feb 10.
    Department of Human Genetics, KU Leuven - University of Leuven, Leuven, Belgium.
    Background: Keratinocytic epidermal nevus syndrome (KENS) is a complex disorder not only characterized by the presence of epidermal nevi but also by abnormalities in the internal organ systems. A small number of cases with KENS are molecularly characterized and reported in the literature with somatic activating RAS, FGFR3 and PIK3CA mutations.

    Case Presentation: In this study we present a patient with hyper- and hypopigmented regions, verrucous pigmented skin lesions and a paravertebral conglomerate tumour at the level of the cervical and thoracic spine. Read More

    Systematised epidermal nevus - a case report.
    J Clin Diagn Res 2015 Feb 1;9(2):WD01-2. Epub 2015 Feb 1.
    Consultant Dermatologisty, Chennai, India .
    A 20-year-old female presented with raised skin lesions all over the body since birth. Clinical examination revealed multiple pigmented macules and hyperkeratotic papules seen along Blaschko's lines over the face, neck, trunk and both lower limbs. Histopathological examination revealed hyperkeratosis with moderated acanthosis and uniform pigmentation over basal zone. Read More

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