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    1 OF 34

    An unusual cause of gastrointestinal bleeding in a hemodialysis patient.
    Hemodial Int 2018 Apr 2. Epub 2018 Apr 2.
    AURA Paris Plaisance, Paris, France.
    Iron deficiency caused by gastrointestinal (GI) bleeding is a common cause of anemia in hemodialysis patient. Herein, we report the case of an 89-year-old hemodialysis patient who presented with acute anemia and melena. Endoscopy found gastritis and diverticulosis without active bleeding. Read More

    [Blue rubber bleb naevus syndrome involving the pleura].
    Rev Mal Respir 2018 Mar 28. Epub 2018 Mar 28.
    Service d'oncologie thoracique, maladies de la plèvre et pneumologie interventionnelle, hôpital Nord, chemin des Bourrely, 13015 Marseille, France; Aix-Marseille université, 13284 Marseille cedex 07, France. Electronic address:
    Bean's syndrome ('blue rubber bleb nevus syndrome') is a rare disease characterized by venous malformations involving various organs. Most often these lesions are localized to the skin and the digestive system. Gastro-intestinal bleeding is the most frequent presentation. Read More

    Solitary pigmented lesions in oral mucosa in Latin American children: A case series.
    Pediatr Dermatol 2018 Mar 24. Epub 2018 Mar 24.
    Department of Pathology and Oral Medicine, School of Dentistry, Oral Medicine Clinic, Universidad de Chile, Santiago, Chile.
    Background: A variety of local and systemic processes caused exogenous and endogenous pigmentation of the oral mucosa. Solitary melanotic pigmentation is rare, hence the scarce number of studies in children and adolescents.

    Methods: Clinical and histopathologic features of 10 Latin American children with solitary pigmented lesions of the oral mucosa were reviewed. Read More

    Blue Nevi of the Ocular Surface: Clinical Characteristics, Pathologic Features, and Clinical Course.
    Ophthalmology 2018 Mar 15. Epub 2018 Mar 15.
    Bascom Palmer Eye Institute, University of Miami, Miami, Florida. Electronic address:
    Purpose: Blue nevus is a melanocytic tumor that is commonly found in the skin. Extracutaneous presentations, including the ocular surface, are rare. As such, the purpose of this study was to characterize the clinical features and clinical course of congenital melanocytic tumor (blue nevus) of the conjunctiva. Read More

    Fern-shaped patch as a hallmark of blue rubber bleb nevus syndrome in neonatal venous malformations.
    Eur J Pediatr 2018 Mar 8. Epub 2018 Mar 8.
    Department of Pediatric Surgery, University Hospital La Paz, Madrid, Spain.
    Blue rubber bleb nevus syndrome (BRBNS) is a rare congenital vascular disease associated with important morbidity and non-insignificant risk of mortality in cases of severe gastrointestinal or neurological involvement. Early diagnosis in the neonatal period can be difficult as very often skin lesions appear progressively during childhood having no correlation with gastrointestinal lesion development. The appearance of one large venous malformation (VM) in the neonatal period has suggested a characteristic finding of this syndrome. Read More

    [Bean's syndrome in children: about two cases].
    Pan Afr Med J 2017 4;28:102. Epub 2017 Oct 4.
    Service des Urgences Chirurgicales Pédiatriques, CHU Ibn Sina, Faculté de Médecine Mohammed V, Rabat, Maroc.
    Diffuse angiomatosis or Bean's syndrome is a rare disease characterized by venous malformations mainly involving the skin and the digestive tract which can result in hemorrhage of variable severity. This study reports the case of two children aged 5 and 9 and a half years respectively with diffuse angiomatosis who had been treated in the Department of Emergency Paediatric Surgery over the years. The diagnosis was based on rectal bleeding and/or melenas causing severe anemia requiring regular transfusions in both patients as well as skin angiomas occurrence at the level of the limbs. Read More

    New therapies for vascular anomalies of the GI tract.
    Minerva Pediatr 2018 Feb 23. Epub 2018 Feb 23.
    Harvard Medical School, GI Procedure and Endoscopy Unit, Division of Gastroenterology, Hepatology, and Nutrition, Boston Children's Hospital, Boston, Massachusetts, USA -
    Vascular anomalies are a morphologically and biologically diverse group of vascular channel abnormalities that are often congenital but may evolve or change over time in the developing child. Classification is based on a combination of physical and biological properties and clinical behavior that differentiate primarily between tumors and malformations and includes a few provisionally unclassified lesions. Anomalies of the GI tract may present clinically with GI bleeding, abdominal pain, high-output cardiac failure, and malabsorption. Read More

    Blue light versus red light for photodynamic therapy of basal cell carcinoma in patients with Gorlin syndrome: A bilaterally controlled comparison study.
    Photodiagnosis Photodyn Ther 2018 Feb 19. Epub 2018 Feb 19.
    Department of Quantitative Health Sciences, Cleveland Clinic, Cleveland, OH 44195, United States.
    Background: Photodynamic therapy (PDT) is a non-scarring alternative for treating basal cell carcinoma (BCC) in patients with Basal Cell Nevus Syndrome (BCNS), also known as Gorlin syndrome. In Europe, red light (635 nm) is the predominant source for PDT, whereas in the United States blue light (400 nm) is more widely available. The objective of this study was to conduct a head-to-head comparison of blue light and red light PDT in the same BCNS patients. Read More

    Sirolimus alternative to blood transfusion as a life saver in blue rubber bleb nevus syndrome: A case report.
    Medicine (Baltimore) 2018 Feb;97(8):e9453
    Department of Pediatrics, Chinese PLA General Hospital, Beijing, China.
    Rationale: Blue rubber bleb nevus syndrome (BRBNS) is a rare disease characterized by multiple venous malformations. The gastrointestinal bleeding and secondary iron deficiency anemia are the most common complications. There are currently no effective treatments for BRBNS. Read More

    An institution-wide algorithm for direct-stick embolization of peripheral venous malformations.
    J Vasc Surg Venous Lymphat Disord 2018 May 1;6(3):351-357. Epub 2018 Feb 1.
    Division of Hematology, Department of Medicine, Cancer Institute of New Jersey, Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ.
    Objective: No standardized therapeutic algorithm or embolic agent of choice has yet been identified for management of congenital peripheral venous malformations (VMs). Treatment options and reported outcomes therefore vary widely. Herein, we present an institution-wide algorithm for management of symptomatic congenital peripheral VMs using a single embolotherapeutic modality. Read More

    BRAF, NRAS, and GNAQ Mutations in Conjunctival Melanocytic Nevi.
    Invest Ophthalmol Vis Sci 2018 Jan;59(1):117-121
    Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New York, United States.
    Purpose: To evaluate BRAF, NRAS, and GNAQ mutations in surgical specimens of common and blue conjunctival melanocytic nevi.

    Methods: Surgical specimens from 25 conjunctival melanocytic nevi (23 common and 2 blue) of 25 patients were evaluated. All common nevi were analyzed immunohistochemically for the expression of BRAF V600E or NRAS Q61R. Read More

    Blue nevus with satellitosis: case report and literature review.
    An Bras Dermatol 2017 ;92(5 Suppl 1):30-33
    Private practice - Tubarão (SC), Brazil.
    Blue nevus is a benign melanocytic lesion, typically asymptomatic and of unknown etiology. Many histological subtypes are recognized, the most commons being: common blue nevus, cellular blue nevus, and combined blue nevus. New rare variants have been described in the literature, with emphasis on eruptive blue nevus, plaque, agminate, linear, with satellitosis, disseminated, familial and targetoid. 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

    Keratinocyte Sonic Hedgehog Upregulation Drives the Development of Giant Congenital Nevi via Paracrine Endothelin-1 Secretion.
    J Invest Dermatol 2018 Apr 11;138(4):893-902. 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

    Agminated segmental plaque-type blue nevus associated with hypertrichosis and soft tissue hypertrophy: Report of a case and review of the literature.
    Pediatr Dermatol 2018 Jan 11;35(1):e22-e28. Epub 2017 Oct 11.
    Department of Dermatology, Mayo Clinic, Rochester, MN, USA.
    Blue nevi are common skin neoplasms that typically present as asymptomatic solitary papules, although they may rarely occur in an agminated configuration. We describe a case of agminated blue nevus in a segmental facial distribution associated with soft tissue hypertrophy and hypertrichosis in a 16-year-old boy and present a review of the literature. Although they are generally considered to be benign, concurrent soft tissue changes occurring within an agminated blue nevus should be investigated thoroughly to exclude alternate diagnoses. Read More

    Melanocortin 1 receptor (MC1R) polymorphisms' influence on size and dermoscopic features of nevi.
    Pigment Cell Melanoma Res 2018 Jan 17;31(1):39-50. Epub 2017 Oct 17.
    Dermatology Department, Melanoma Unit, Hospital Clínic, IDIBAPS (Institut d'Investigacions Biomèdiques August Pi i Sunyer), Barcelona, Spain.
    The melanocortin 1 receptor (MC1R) is a highly polymorphic gene. The loss-of-function MC1R variants ("R") have been strongly associated with red hair color phenotype and an increased melanoma risk. We sequenced the MC1R gene in 175 healthy individuals to assess the influence of MC1R on nevus phenotype. Read More

    Surgical Emergencies in Intestinal Venous Malformations.
    Eur J Pediatr Surg 2018 Feb 25;28(1):101-104. Epub 2017 Sep 25.
    Division of Vascular Anomalies, Pediatric Surgery, La Paz Children's Hospital, Madrid, Spain.
    Background:  Venous malformations (VMs) can occur in any part of the body; however, the gastrointestinal tract is a frequent location. These are usually asymptomatic, thus, representing a challenge to diagnosis. Intestinal location of VMs can be associated with severe complications that ultimately require an emergency surgery. 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

    Distinct Patterns and Aetiology of Chromonychia.
    Acta Derm Venereol 2018 Jan;98(1):108-113
    Department of Dermatology, Chonnam National University Medical School, 160 Baekseo-ro, Dong-gu, Gwangju, 61469, Korea.
    Abnormal colouring of the nails may be a sign of underlying systemic or local disorders. This study investigated the prevalence and causes of chromonychia as a whole, as well as of each subtype. Among 163 patients with chromonychia, trauma was the pathogenesis in up to 20. Read More

    Postoperative disseminated intravascular coagulation in a pregnant patient with Blue Rubber Bleb Nevus Syndrome presenting with acute intestinal obstruction: Case report and literature review.
    Int J Surg Case Rep 2017 23;39:235-238. Epub 2017 Aug 23.
    Departament of Surgery, Division of Surgical Clinic III, Hospital das Clinicas of the University of Sao Paulo School of Medicine, Brazil. Electronic address:
    Background: Blue Rubber Bleb Nevus Syndrome (BRBNS) is a rare condition which usually manifests as multiple hemangioma-like skin and gastrointestinal lesions. The latter often present with chronic bleeding. There is no consensus regarding the optimal management of such patients. Read More

    Blue rubber bleb nevus syndrome: our experience and new endoscopic management.
    Medicine (Baltimore) 2017 Aug;96(33):e7792
    aDepartment of Gastroenterology, The First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, Zhejiang Province bDepartment of Nephrology, Jilin City Central Hospital, Jilin, Jilin Province, China.
    The aim of our study is to enhance the awareness of blue rubber bleb nevus syndrome (BRBNS) through the patients in our hospital and introduced a new measure of endoscopic intervention.A retrospective review of 5 patients, who were diagnosed as BRBNS in our hospital from January 2013 to January 2017, was conducted. Data were collected with regard to demographics, clinical presentation, endoscopic and imaging findings, management, and follow-up data. Read More

    Alkaptonuria: A Case Report With Diagnostic Challenge.
    S D Med 2017 Aug;70(8):366-368
    Department of Dermatology, University of South Dakota Sanford School of Medicine.
    Alkaptonuria is a rare autosomal recessive metabolic disorder caused by deficiency of homogentisic acid (HGA) oxidase, the only enzyme capable of catabolizing HGA. Deficiency of this enzyme leads to excess HGA which deposits in the connective tissue. We present a case of a 64-year-old woman who was referred to the dermatology clinic for a full body mole check and skin cancer screening. Read More

    Pigmented Epithelioid Melanocytoma (PEM)/Animal Type Melanoma (ATM): Quest for an Origin. Report of One Unusual Case Indicating Follicular Origin and Another Arising in an Intradermal Nevus.
    Int J Mol Sci 2017 Aug 15;18(8). Epub 2017 Aug 15.
    Departments of Dermatology and Pathology, University of Alabama at Birmingham, Birmingham, AL 35201, USA.
    Pigmented epithelioid melanocytoma (PEM) is a tumor encompassing epithelioid blue nevus of Carney complex (EBN of CNC) and was previously termed animal-type melanoma. Histologically PEMs are heavily pigmented spindled and epithelioid dermal melanocytic tumors with infiltrative borders, however, their origin remains unclear. Stem cells for the epidermis and hair follicle are located in the bulge area of the hair follicle with the potential to differentiate into multiple lineages. Read More

    Blue Nevi and Related Tumors.
    Clin Lab Med 2017 Sep 4;37(3):401-415. Epub 2017 Jul 4.
    Lahey Clinic, 41 Mall Road, Burlington, MA 018056, USA; www.DermatopathologyConsultations.com, C/O Harvard Vanguard Medical Associates Laboratories, 152 2nd Avenue, Needhan, MA 02494, USA. Electronic address:
    The major entities related to blue nevus are common blue nevus, cellular blue nevus, atypical blue nevus, and malignant blue nevus. These lesions share presence of dermal pigmented dendritic melanocytes derived from embryonal precursors to melanocytes, Schwann cells, and glial cells migrating to the skin from the ventral neural crest. Genetically, blue nevi harbor mutations in G-protein-coupled receptor subunits GNAQ and GNA11. Read More

    Cellular Blue Nevus of Perilimbal Conjunctiva: A Case Report and Review of Literature.
    Ann Clin Lab Sci 2017 Aug;47(4):477-480
    Department of Ophthalmology, Tianjin Medical University General Hospital, Tianjin China.
    Cellular blue nevus is an uncommon neoplasm in the conjunctiva. Here we present an unusual case of a cellular blue nevus that clinically resembled conjunctival melanoma. A 29-year-old Chinese male was found to have a giant pigmented lesion of the conjunctiva around the limbal area of right eye from birth. Read More

    Genomic Analysis of Pigmented Epithelioid Melanocytomas Reveals Recurrent Alterations in PRKAR1A, and PRKCA Genes.
    Am J Surg Pathol 2017 Oct;41(10):1333-1346
    Departments of *Pathology §Dermatopathology ∥Dermatology †Clinical Cancer Genomics Laboratory ‡Helen Diller Cancer Center, University of California, San Francisco, CA ¶Department of Pathology, Lahey Clinic, Burlington, MA.
    Pigmented epithelioid melanocytoma (PEM) is a rare cutaneous melanocytic tumor first described as epithelioid blue nevus in patients with the Carney Complex (CC). PEM was among the first established examples of an intermediate class of melanocytic tumors, including atypical Spitz tumors, with frequent metastasis to lymph nodes but only rare extranodal spread. Sporadic and CC-associated PEM are essentially histologically indistinguishable. Read More

    Anatomic mapping and clinicopathologic analysis of benign acral melanocytic neoplasms: A comparison between adults and children.
    J Am Acad Dermatol 2017 Oct 1;77(4):735-745. Epub 2017 Jul 1.
    Department of Dermatology, Chonnam National University Medical School, Gwangju, Korea. Electronic address:
    Background: The clinicopathologic features of benign acral melanocytic neoplasms (BAMNs) remain poorly understood.

    Objective: To define the clinicopathologic features of BAMNs.

    Methods: We analyzed clinical data and mapped BAMNs anatomically. Read More

    Reflectance confocal microscopy analysis of equivocal melanocytic lesions with severe regression.
    Skin Res Technol 2018 Feb 21;24(1):9-15. Epub 2017 May 21.
    Department of Dermatologic Clinic, San Gallicano Dermatological Institute - IRCCS, Rome, Italy.
    Background: The differential diagnosis between regressing nevi and melanoma might be challenging; regressing areas can represent a confounding factor for the diagnosis and the histology still remain mandatory to rule out melanoma. Reflectance confocal microscopy may add valuable information by revealing features suggestive of the nature of the melanocytic proliferation.

    Objective: To assess the impact of confocal microscopy in the management of regressive melanocytic lesions. Read More

    Melanoma Is Skin Deep: A 3D Reconstruction Technique for Computerized Dermoscopic Skin Lesion Classification.
    IEEE J Transl Eng Health Med 2017 16;5:4300117. Epub 2017 Jan 16.
    Research and Development TeamPlanet-I Technologies.
    Melanoma mortality rates are the highest amongst skin cancer patients. Melanoma is life threating when it grows beyond the dermis of the skin. Hence, depth is an important factor to diagnose melanoma. Read More

    [Negative pressure system in the treatment of enterocutaneous fistulas in the pediatric population].
    Cir Pediatr 2016 Oct 10;29(4):166-170. Epub 2016 Oct 10.
    Servicio de Cirugía Pediátrica. Hospital Universitario La Paz. Madrid.
    Aim Of The Study: The management of children with enterocutaneous fistulas (EF) along with large abdominal wall defects secondary to multiple surgical interventions can be difficult and sometimes lead to intestinal failure (IF). The aim of this study is to present the results of negative pressure systems and their properties (edema reduction angiogenesis promotion and granulation tissue formation) in children with enterocutaneous fistulas (EF) and their prognosis.

    Materials And Methods: A retrospective analysis of children with refractory, high output EF treated with NPS between 2008-2014. Read More

    Genomic Assessment of Blitz Nevi Suggests Classification as a Subset of Blue Nevus Rather Than Spitz Nevus: Clinical, Histopathologic, and Molecular Analysis of 18 Cases.
    Am J Dermatopathol 2018 Feb;40(2):118-124
    Dermatology, Feinberg School of Medicine, Northwestern University, Chicago, IL.
    Blitz nevi/tumors are a distinct subset of melanocytic neoplasia which show mixed morphologic features of Spitz and blue nevus. Genomically, most blue nevi have GNAQ or GNA11 mutations while most Spitzoid neoplasms have either an HRAS mutation or translocations involving MET, ROS, BRAF, ALK1, NTRK1, and RET. The criteria used for the assessment of malignancy in blue and Spitzoid lesions are different, and these lesions have different prognostic markers. Read More

    Estimating melanin location in the pigmented skin lesions by hue-saturation-lightness color space values of dermoscopic images.
    J Dermatol 2017 May;44(5):490-498
    Department of Dermatology, Tokyo Women's Medical University Medical Center East, Tokyo, Japan.
    The depth of melanin in the skin can be estimated roughly by observation of the color exhibited on dermoscopy. Currently, there are no objective methods to estimate it. The aim of the present study was to clarify the relationship between the depth of melanin in the skin and the color variation exhibited, and to objectively estimate the 3-D location of melanin in the pigmented skin lesions from dermoscopic images. Read More

    Pigmented epithelioid melanocytoma (animal-type melanoma): An institutional experience.
    J Am Acad Dermatol 2017 Aug 14;77(2):328-332. Epub 2017 Apr 14.
    Department of Dermatology, University of Rochester School of Medicine, Rochester, New York; Department of Pathology, University of Rochester School of Medicine, Rochester, New York.
    Background: Pigmented epithelioid melanocytoma (PEM) is an uncommon, recently described entity with unknown biologic behavior. There is a high rate of regional metastases, but limited evidence of distant metastases or disease-related death.

    Objective: We sought to report our series of patients given a diagnosis of PEM at our institution and provide mutational analysis of genes commonly implicated in melanoma in 2 cases. Read More

    SF3B1 and BAP1 mutations in blue nevus-like melanoma.
    Mod Pathol 2017 Jul 14;30(7):928-939. Epub 2017 Apr 14.
    Department of Dermatology, University Hospital Essen, West German Cancer Center, University Duisburg-Essen and the German Cancer Consortium (DKTK), Essen, Germany.
    Blue nevi are melanocytic tumors originating in the cutaneous dermis. Malignant tumors may arise in association with or resembling blue nevi, so called 'blue nevus-like melanoma', which can metastasize and result in patient death. Identifying which tumors will behave in a clinically aggressive manner can be challenging. Read More

    Phacomatosis Cesioflammea with Cutis Marmorata-like Lesions and Unusual Extracutaneous Abnormalities: Is It a Distinct disorder?
    Indian J Dermatol 2017 Mar-Apr;62(2):207-209
    Department of Dermatology, University Medical Center Freiburg, Freiburg, Germany.
    A 2-month-old boy was presented with widespread lateralized blue macules (nevus cesius), an extensive nevus flammeus, and large patches of cutis marmorata telangiectatica congenita. Moreover, he had macrocephaly, a coarse facial appearance with depressed nasal bridge, retinal abnormalities, septal defects of the heart, and obliteration of the left brachiocephalic vein and major veins of the left arm with pronounced collateralization. The multisystem disorder of this boy cannot be categorized within the present classification of distinct types of phacomatosis pigmentovascularis. Read More

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