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    111 results match your criteria Connective Tissue Nevus

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    [Extensive connective tissue nevus in children].
    Ann Dermatol Venereol 2017 Jul 28. Epub 2017 Jul 28.
    Service de dermatologie, CHU de Caen, avenue de la Côte-de-Nacre, CS 30001, 14033 Caen cedex 9, France.
    Background: Connective tissue nevus (CTN) is a rare condition of the extracellular matrix components that generally presents as papulae of normal skin colour. This condition may be syndromic or sporadic.

    Patients And Methods: We report herein two isolated cases of extensive and infiltrative CTN in children at risk for subsequent joint stiffening. Read More

    Fibroblastic connective tissue nevus: Clinicopathological and immunohistochemical study of 14 cases.
    J Cutan Pathol 2017 Jun 20. Epub 2017 Jun 20.
    Dermopathologie Bodensee, Friedrichshafen, Germany.
    Background: We present herein a series of 14 lesions showing overlapping features with the newly defined benign cutaneous mesenchymal neoplasm labeled as fibroblastic connective tissue nevus (FCTN).

    Methods And Results: Total of 8 patients were male and 5 were female, ranging in age from 1 to 56 years. Lesions appeared as isolated nodules or plaques on the trunk (7 cases), the limbs (4 cases) and the neck (2 cases). Read More

    Congenital Atrophic Plaque: Fibroblastic Connective Tissue Nevus.
    Pediatr Dermatol 2017 Jul 23;34(4):e216-e218. Epub 2017 May 23.
    Department of Pathology, University Hospital of Salamanca, Salamanca, Spain.
    Fibroblastic connective tissue nevus (FCTN) is a rare, benign, dermal mesenchymal hamartoma that affects children. We report a 15-year-old boy with a congenital FCTN and describe the clinical, dermatoscopic, and histopathologic features. Read More

    Clinical Characteristics of Connective Tissue Nevi in Tuberous Sclerosis Complex With Special Emphasis on Shagreen Patches.
    JAMA Dermatol 2017 Jul;153(7):660-665
    Department of Dermatology, Uniformed Services University of the Health Sciences, Bethesda, Maryland.
    Importance: Patients with tuberous sclerosis complex (TSC) frequently develop collagenous connective tissue nevi. The prototypical lesion is a large shagreen patch located on the lower back, but some patients only manifest small collagenomas or have lesions elsewhere on the body. The ability to recognize these variable presentations can be important for the diagnosis of TSC. Read More

    Proteus Syndrome with Arteriovenous Malformation.
    Adv Biomed Res 2017 7;6:27. Epub 2017 Mar 7.
    Department of Dermatology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran.
    Proteus syndrome is a rare sporadic disorder that appears with localized macrosomia, congenital lipomatosis, and slow flow vascular malformations, connective tissue nevus, and epidermal nevus. There are usually some manifestations at birth. The vascular abnormalities that have been reported in Proteus syndrome are capillary and slow flow venous malformation. Read More

    Elastoma: clinical and histopathological aspects of a rare disease.
    An Bras Dermatol 2016 Sep-Oct;91(5 suppl 1):39-41
    Universidade Federal de São Paulo (Unifesp) - São Paulo (SP), Brazil.
    Elastoma is a connective tissue nevus characterized by changes in elastic fibers. It can be congenital or acquired, and is usually diagnosed before puberty. Associated with osteopoikilosis, it is known as Buschke-Ollendorff syndrome. Read More

    Evolution of Fibroblastic Connective Tissue Nevus in an Infant.
    Am J Dermatopathol 2017 Mar;39(3):225-227
    *Department of Pathology, West Virginia University, Morgantown, WV; and Departments of †Plastic Surgery, ‡Dermatology, §Pediatrics, and ¶Pathology, The Penn State Hershey Medical Center, Hershey, PA.
    Fibroblastic connective tissue nevus (FCTN) is a rare and recently described neoplasm of fibroblastic/myofibroblastic lineage. We report a case of a 1-month-old healthy male infant who presented with a dermal plaque on the upper chest since birth. A punch biopsy demonstrated a dermal spindle-cell neoplasm with variable smooth muscle actin positivity and negative staining for CD34, consistent with myofibroma. Read More

    Zosteriform Collagen Nevus in an Infant.
    Acta Dermatovenerol Croat 2016 Jun;24(2):148-9
    Aslı Aksu Çerman, MD, Şişli Hamidiye Etfal Training and Research Hospital, Dermatology Department, Halaskargazi Cad, Etfal S, Istanbul, Turkey;
    Connective tissue nevi (CTN) are dermal hamartomas characterized by an imbalance in the amount and distribution of the normal components of the extracellular dermal matrix, specifically collagen, elastin, and/or proteoglicans. The term "CTN" was first mentioned by Lewandowsky in 1921 (1), although it was not accepted until the review by Gutmann in 1926 (2). Classification of CTN was established by Uitto et al. Read More

    Agminated Fibroblastic Conective Tissue Nevus: A New Clinical Presentation.
    Pediatr Dermatol 2016 Jul 14;33(4):e240-3. Epub 2016 Jun 14.
    Department of Dermatology, Hospital de la Santa Creu I Sant Pau, Barcelona, Spain.
    Connective tissue nevi are benign hamartomatous lesions in which one or several of the components of the dermis (collagen, elastin, glicosaminoglycans) show predominance or depletion. Recently, de Feraudy et al broadened the spectrum of connective tissue nevus, describing fibroblastic connective tissue nevus (FCTN), which is characterized by proliferation of CD34(+) cells of fibroblastic and myofibroblastic lineage. Only solitary papules and nodules have been described. Read More

    [Mucinous nevus of late onset].
    Ann Dermatol Venereol 2016 Aug-Sep;143(8-9):547-53. Epub 2016 May 16.
    Service de dermatologie, hôpital Jean-Bernard, CHU de Poitiers, 2, rue de la Milétrie, 86021 Poitiers cedex, France.
    Introduction: Mucinous nevus is a rare disease characterized by mucin deposits in the dermis, with only 23 cases reported to date. It belongs to the connective tissue nevus family and is characterized by dermal mucin deposits. Two histological subtypes have been described: pure mucinous dermal nevus (connective tissue nevus of the proteoglycan [CTNP]) and combined mucinous nevus (combined CTNP) associating epidermal nevus with dermal mucin deposits. Read More

    The Importance of Collagen Tissue in Papular Elastorrhexis, Eruptive Collagenoma, and Nevus Anelasticus.
    Ann Dermatol 2016 Apr 31;28(2):210-5. Epub 2016 Mar 31.
    Department of Dermatology, Dongguk University Ilsan Hospital, Dongguk University College of Medicine, Goyang, Korea.
    Background: Papular elastorrhexis (PE), eruptive collagenoma (EC), and nevus anelasticus (NA) are described as multiple small papules with decrease, fragmentation, or lack of dermal elastic fibers. These diseases are suggested to be the same entity. The change of collagen fibers in the conditions has not been addressed to date. Read More

    Development of Sinus Tracts within a Connective Tissue Nevus.
    Pediatr Dermatol 2015 Nov-Dec;32(6):e298-9. Epub 2015 Sep 4.
    Loyola University, Chicago Stritch School of Medicine, Chicago, Illinois.
    We report a case of sinus tract development within a connective tissue nevus in a patient with velocardiofacial syndrome and describe our treatment of sinus tracts using surgical deroofing and trichloroacetic acid scarification. Read More

    Fibroblastic connective tissue nevus.
    J Cutan Pathol 2016 Jan 21;43(1):75-9. Epub 2015 Oct 21.
    Department of Pathology, Summa Akron City Hospital, Akron, OH, USA.
    Fibroblastic connective tissue nevus (FCTN) is a newly recognized, benign cutaneous mesenchymal lesion of fibroblasts/myofibroblastic lineage, which expands the classification of connective tissue nevi. We present three cases of FCTN and discuss significant clinical, morphologic and immunophenotypic overlap with dermatomyofibroma. Our cases were from young women, aged 32, 24 and 10, and presented as 1. Read More

    [Analysis of TSC gene mutation in a patient with tuberous sclerosis].
    Zhonghua Yi Xue Yi Chuan Xue Za Zhi 2015 Aug;32(4):506-8
    Department of Dermatology, The Affiliated Hospital of North Sichuan Medical College, Nanchong, Sichuan 637000, P. R. China.
    Objective: To identify pathogenic mutation of the TSC1 and TSC2 genes in a patient with tuberous sclerosis.

    Methods: Peripheral venous blood samples and clinical data of a pregnant woman with tuberous sclerosis and 4 family members (parents, uncle and husband) were collected. Genomic DNA was extracted. Read More

    Fronto-temporal cerebriform connective tissue nevus in Proteus syndrome.
    Indian J Dent Res 2014 Nov-Dec;25(6):828-31
    Director and Consultant Maxillofacial Surgeon, Balaji Dental and Craniofacial Hospital, Chennai, Tamil Nadu, India.
    Proteus syndrome is one of the very rare syndromes with occurrence of cerebriform connective tissue nevus. The aim of the present manuscript was to present a case of Proteus syndrome in an unusual facial location, which to the best of our knowledge, is being reported for the first time. The unusual occurrence further strengthens the mosaical basis of its etiopathogenesis. Read More

    A New Variant of Connective Tissue Nevus with Elastorrhexis and Predilection for the Upper Chest.
    Pediatr Dermatol 2015 Jul-Aug;32(4):518-21. Epub 2014 Dec 28.
    Section of Dermatology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
    Localized changes in cutaneous elastic tissue often manifest with flesh-colored, hypopigmented, or yellow papules, plaques, and nodules. We present five children with clinically similar cobblestone plaques composed of multiple hypopigmented, nonfollicular, pinpoint papules located unilaterally over the upper chest. All lesions first appeared at birth or during early infancy. Read More

    Cerebriform connective tissue nevus of lumbar.
    J Dermatol 2015 Feb 16;42(2):219-20. Epub 2014 Dec 16.
    Department of Dermatology, Wuhan No. 1 Hospital, Tongji Medical College, Huazhong University of Science and Technology (HUST), Wuhan, China.
    Connective tissue nevi represents a kind of hamartoma, and coalescence of the lesions in a cerebriform mode in the lumbar region without Proteus syndrome is rarely seen. Here, we report a 26-year-old woman presenting with nodules and plaques in her left lumbar region of 26 years in duration. Histopathological examination and Masson-trichrome stain showed increased dermal collagen bundles in a haphazard array. Read More

    Zosteriform collagen nevus in a young boy.
    Dermatol Online J 2014 Aug 17;20(8). Epub 2014 Aug 17.
    Okmeydani Training and Research Hospitalq.
    Zosterifom connective tissue nevus is a rare kind of connective tissue nevi composed of collagen, elastin, or glycosaminoglycan, which was first reported by Steiner 1944. Herein, we report a young boy with a collagen nevus that presented in a zosteriform distribution. Read More

    Athlete's nodule in a figure skater: an unusual presentation.
    Am J Dermatopathol 2015 Feb;37(2):e21-5
    Ackerman Academy of Dermatopathology, New York, NY.
    Sports-related acquired connective tissue nevus or "athlete's nodule" has been reported under a variety of different names. The lesion develops in response to activity causing chronic or repetitive low-grade pressure or irritation activity, which is often a component of athletic training. A case of "athlete's nodule" in a figure skater has been reported. Read More

    Ossifying fibroma in Buschke-Ollendorff syndrome.
    J Cutan Pathol 2014 Sep 4;41(9):740-4. Epub 2014 Jul 4.
    Department of Dermatology, University of California, San Francisco, CA, USA.
    Buschke-Ollendorff syndrome represents an autosomal dominant disorder characterized by connective tissue nevi and osteopoikilosis. Cutaneous lesions may contain either predominantly elastic fibers or predominantly collagen fibers or may show both connective tissue components. The disease results from mutations in LEMD3 (MAN1), which lead to enhanced transforming growth factor-β (TGF-β) signaling and resultant changes in fibroblast function. Read More

    A limited form of proteus syndrome with bilateral plantar cerebriform collagenomas and varicose veins secondary to a mosaic AKT1 mutation.
    JAMA Dermatol 2014 Sep;150(9):990-3
    Department of Dermatology, St Helier University Hospital, Surrey, England.
    Importance: Proteus syndrome is an extremely rare disorder of mosaic postnatal overgrowth affecting multiple tissues including bone, soft tissue, and skin. It typically manifests in early childhood with asymmetric and progressive skeletal overgrowth that leads to severe distortion of the skeleton and disability. The genetic basis has recently been identified as a somatic activating mutation in the AKT1 gene, which encodes an enzyme mediating cell proliferation and apoptosis. Read More

    Cylindroma with Stromal Adipose Tissue Metaplasia versus Arising in a Background of Nevus Lipomatosus.
    Case Rep Pathol 2014 16;2014:203298. Epub 2014 Mar 16.
    Department of Pathology and Molecular Medicine, McMaster University, HSC-2N10, 1280 Main Street West, Hamilton, ON, Canada L8S 4K1 ; St. Joseph's Healthcare Hamilton, Charlton Campus, 50 Charlton Avenue East, Hamilton, ON, Canada L8N 4A6.
    Nevus lipomatosus superficialis is a rare type of connective tissue nevus. Cylindroma is a benign skin appendage tumor with a predilection for the scalp of older females. We describe the case of a 56-year-old woman with a scalp lesion demonstrating histopathologic features consistent with benign cylindroma arising within a nevus lipomatosus superficialis. Read More

    Nevus anelasticus: how should such lesions be classified?
    J Cutan Pathol 2014 Jun 26;41(6):519-23. Epub 2014 Mar 26.
    Department of Dermatology, Warren Alpert Medical School of Brown University, Providence, RI, USA.
    Nevus anelasticus represents a rare entity that is most commonly classified as a connective tissue nevus. It typically presents before 20 years of age with asymmetrically distributed white-to-skin-toned or pink-to-red papules or plaques on the trunk and upper extremities. The lesion is defined histopathologically by the absence or degeneration of elastic fibers in the dermis. Read More

    Isolated collagenoma on the scalp: a rare presentation.
    Int J Trichology 2013 Apr;5(2):88-90
    Department of Dermatology and Venereology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh, India.
    Connective tissue nevi of the skin are benign hamartomatous lesions consisting predominantly of one of the components of the extracellular matrix comprising of collagen, elastin or glycosaminoglycans type. Connective tissue nevi may be solitary or multiple, sporadic or inherited. Collagenomas are asymptomatic and usually occurs over upper trunk, arms, back, thighs and soles. Read More

    Isolated corymbose collagenoma responding to intralesional triamcinolone acetonide and hyaluronidase injections.
    Dermatol Ther 2013 Sep-Oct;26(5):419-23. Epub 2013 Mar 28.
    Department of Dermatology, PGIMER, Chandigarh, India.
    Collagenomas are connective tissue nevi with circumscribed hamartomatous proliferation of collagen. Due to their benign nature and lack of any simple medical treatment, they are most often left untreated. We present a case of isolated corymbose collagenoma, a distinct morphological variant not described hitherto that was successfully treated with intralesional injections of combination of triamcinolone acetonide and hyaluronidase. Read More


    Fibroblastic connective tissue nevus: a rare cutaneous lesion analyzed in a series of 25 cases.
    Am J Surg Pathol 2012 Oct;36(10):1509-15
    Department of Pathology, Brigham and Women's Hospital, Boston, MA 02115, USA.
    Fibroblastic connective tissue nevus (FCTN) represents a rare and distinct benign cutaneous mesenchymal lesion of fibroblastic/myofibroblastic lineage, which broadens the spectrum of lesions presently recognized as connective tissue nevus. A series of 25 cases of FCTN has been analyzed to further characterize the clinicopathologic spectrum and immunohistochemical features of this entity. Sixteen patients were female (64%) and 9 were male (36%), with age at presentation ranging from 1. Read More

    Proteus syndrome: three case reports with a review of the literature.
    Fetal Pediatr Pathol 2012 Jun 13;31(3):145-53. Epub 2012 Mar 13.
    Department of Pediatrics and Pathology, Emory University School ofMedicine, 1405 Clifton Road NE, Atlanta, GA 30322, USA.
    Proteus syndrome (PS) is a rare, progressive disorder that manifests as asymmetric, disproportionate overgrowth affecting tissues derived from any germline layer. Cases of PS from 2005-2010 were retrieved from the pathology files at our institution. Two confirmed cases and one possible case of PS were identified. Read More

    Multiple unilateral zosteriform connective tissue nevi on the trunk.
    Ann Dermatol 2011 Oct 31;23(Suppl 2):S243-6. Epub 2011 Oct 31.
    Department of Dermatology, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Korea.
    Connective tissue nevus is not a true tumor, but rather a hamartoma involving various components of connective tissue. It presents as a slow-growing, painless, flesh-colored, or pink nodule or plaque that is evident from childhood. While any region of the body may be affected, there is a predilection for the trunk and extremities. Read More

    Papular elastorrhexis: a case and differential diagnosis.
    Ann Dermatol 2011 Sep 30;23 Suppl 1:S53-6. Epub 2011 Sep 30.
    Department of Dermatology, Dongguk University Ilsan Hospital, College of Medicine, Dongguk University, Goyang, Korea.
    Papular elastorrhexis is a rare cutaneous disorder that occurs predominantly during adolescence. The disorder is characterized by asymptomatic indurated white or flesh papules showing decreased and fragmented elastic fibers in the dermis. We herein report on a 12-year-old girl with multiple asymptomatic white, nonfollicular, firm papules scattered on the extremities and trunk. Read More

    Eruptive collagenomas.
    Dermatol Online J 2010 Nov 15;16(11). Epub 2010 Nov 15.
    Department of Dermatology, New York University, New York, NY, USA.
    A 24-year-old woman presented with hypopigmented papules of the abdomen that had been present for four years without a family history of similar cutaneous findings or associated medical problems. Histopathologic features confirmed the diagnosis of a connective-tissue nevus that was composed of collagen. Eruptive collagenomas are a rare form of acquired collagenomas, which are characterized by the sudden appearance of asymptomatic papules and nodules on the lower trunk and extremities; the lesions are composed of haphazardly arranged collagen fibers. Read More

    Progressive overgrowth of the cerebriform connective tissue nevus in patients with Proteus syndrome.
    J Am Acad Dermatol 2010 Nov 14;63(5):799-804. Epub 2010 Aug 14.
    Department of Dermatology, Uniformed Services University of the Health Sciences, Bethesda, Maryland 20814, USA.
    Background: Proteus syndrome is a rare overgrowth disorder that almost always affects the skin.

    Objective: Our purpose was to evaluate progression of skin lesions in patients with Proteus syndrome.

    Methods: Skin findings were documented in 36 patients with Proteus syndrome. Read More

    Combined epidermal-connective tissue nevus of proteoglycan (a type of mucinous nevus): a case report and literature review.
    J Cutan Pathol 2009 Jul;36(7):808-11
    Department of Dermatology, Chang Gung Memorial Hospital, Chiayi, Taiwan.
    Mucinous nevus is a rare entity with only 11 cases reported previously. It may be divided into two histopathologic types: connective tissue nevus of the proteoglycan (CTNP) and combined epidermal-CTNP. We describe a boy with asymptomatic grouped brown papuloplaques on the lower back since birth. Read More

    A Case of Eruptive Collagenoma on the Left Calf.
    Ann Dermatol 2008 Sep 30;20(3):130-3. Epub 2008 Sep 30.
    Department of Dermatology, College of Medicine, Hallym University, Anyang, Korea.
    Eruptive collagenoma is an acquired connective tissue nevus without family history. It is typically described as numerous small papules or nodules on the trunk and arms with histopathological features of decreased or degenerated elastic fibers. We report a case of a 16-year-old male who presented with multiple asymptomatic 2 to 5 mm sized yellowish grouped papules on the left calf. Read More

    Monozygotic twins discordant for Proteus syndrome.
    Am J Med Genet A 2008 Aug;146A(16):2122-5
    Department of Pediatrics and Pediatric Neurology, Georg August University, Göttingen, Germany.
    Proteus syndrome is a rare, complex disorder predominantly characterized by asymmetric overgrowth of body parts, connective tissue and epidermal nevi, and vascular malformations. General diagnostic criteria comprise mosaic distribution, sporadic occurrence, and progressive course. We report on Proteus syndrome in discordant monozygotic twins. Read More

    Phacomatosis pigmentokeratotica: a follow-up report documenting additional cutaneous and extracutaneous anomalies.
    Pediatr Dermatol 2008 Jan-Feb;25(1):76-80
    Department of Dermatology, Hospital del Niño Jesús, Tucumán, Argentina.
    This is a follow-up report on a boy with phacomatosis pigmentokeratotica. At the age of 10 years he had, in addition to a sebaceous nevus and a speckled lentiginous nevus, multiple lesions of a collagen nevus localized on the chin and in the lumbar area. On the left shoulder, a small telangiectatic spot was present within the area of the speckled lentiginous nevus. Read More

    Isolated plantar collagenoma not associated with Proteus syndrome.
    J Am Acad Dermatol 2008 Mar;58(3):497-9
    Department of Dermatology, Harvard Medical School, Boston, Massachusetts, USA.
    Isolated plantar cerebriform collagenomas are a relatively rare type of connective tissue nevus. They have been suggested to be pathognomonic of Proteus syndrome. However, their presence is now considered to be a major criterion of Proteus syndrome, but the diagnosis of Proteus syndrome also requires the presence of other minor criteria. Read More

    A mild form of Proteus syndrome.
    Pediatr Dermatol 2007 Nov-Dec;24(6):660-2
    Department of Dermatology, Faculty of Medicine, Gazi University, Beşevler, Ankara, Turkey.
    Proteus syndrome is a multisystem disorder and diagnosis has been difficult because of the variability of the syndrome's manifestations. Dermatologists have an important role as cutaneous findings could provide clues for the early diagnosis of the disease. Herein we report a case who had cerebriform connective tissue nevus and lipoma as the sole manifestations of the disease. Read More

    Zosteriform connective tissue nevus in a pediatric patient.
    Pediatr Dermatol 2007 Sep-Oct;24(5):557-8
    Department of Human and Hereditary Pathology, Institute of Dermatology, University of Pavia, Fondazione, IRCCS Policlinico S. Matteo, Pavia, Italy.
    We report an 8-year-old girl affected by hypochromic, asymptomatic, acquired lesions with a paving-stone aspect on the right lumbosacral area and proximal right leg. The results of serum and urine biochemical screening were normal, as was the bone survey A biopsy was performed. The clinical and histologic aspects led to the diagnosis of connective tissue nevus with zosteriform distribution. Read More

    Linear connective tissue nevus.
    Pediatr Dermatol 2007 Jul-Aug;24(4):439-41
    Department of Dermatology, Faculty of Medicine, University of Tokyo, Tokyo, Japan.
    Multiple connective tissue nevi in linear arrangements, which was previously described as zosteriform connective tissue nevus, is a rare variant of connective tissue nevus. We herein report the case of an 8-year-old Japanese boy with this disease. He developed a small mass on the upper region of his right inner ankle at the age of one-half years. Read More

    Familial cutaneous collagenoma: new affected family with prepubertal onset.
    J Dermatol 2007 Jul;34(7):477-81
    Dermatology Clinic, Istanbul Education and Research Hospital, Istanbul, Turkey.
    Two siblings presented with clinical and histopathological findings of familial cutaneous collagenoma which is a rare connective tissue nevus, inherited in an autosomal-dominant pattern. A 13-year-old girl had oval-round, soft, painless papules, 5-10 mm in size and a total of 9-10 on her abdomen and flanks. Skin biopsy demonstrated dense, coarse collagen fibers in the dermis and a decrease in elastic fibers. Read More

    Zosteriform connective tissue nevus: a case report.
    Am J Dermatopathol 2007 Jun;29(3):303-5
    School of Medicine, Flinders University of South Australia, South Australia, Australia.
    Zosteriform connective tissue nevus is a rare form of connective tissue hamartomas, which arises from cells of mesodermal origin. Despite similar clinical appearance of many connective tissue nevi, they can be differentiated histochemically and/or biochemically on the basis of the primary connective tissue element present. There are only 3 reported cases of zosteriform connective tissue nevi in the worldwide literature. Read More

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