124 results match your criteria Connective Tissue Nevus


Cutaneous mucinosis of infancy: report of a rare case and review of the literature.

Dermatol Online J 2018 Oct 15;24(10). Epub 2018 Oct 15.

Geisel School of Medicine at Dartmouth, Hanover, New Hampshire.

Cutaneous mucinosis of infancy (CMI) is a rare dermatologic condition, first reported in 1980 and currently classified within the complex group of papular mucinoses. We report a case of CMI and review the prior 13 cases in the literature. The patient was a 5-year-old girl who presented with asymptomatic dermal papules and plaques on her leg and back with no overlying color change. Read More

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October 2018
1 Read

Novel KHDRBS1-NTRK3 rearrangement in a congenital pediatric CD34-positive skin tumor: a case report.

Virchows Arch 2019 Jan 6;474(1):111-115. Epub 2018 Sep 6.

Department of Dermatology, Unit of Pediatric Dermatology - CHRU Tours, Tours, France.

Cutaneous spindle-cell neoplasms in adults as well as children represent a frequent dilemma for pathologists. Along this neoplasm spectrum, the differential diagnosis with CD34-positive proliferations can be challenging, particularly concerning neoplasms of fibrohistiocytic and fibroblastic lineages. In children, cutaneous and superficial soft-tissue neoplasms with CD34-positive spindle cells are associated with benign to intermediate malignancy potential and include lipofibromatosis, plaque-like CD34-positive dermal fibroma, fibroblastic connective tissue nevus, and congenital dermatofibrosarcoma protuberans. Read More

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http://dx.doi.org/10.1007/s00428-018-2415-0DOI Listing
January 2019
14 Reads
2.651 Impact Factor

Mucinous Nevus.

Ann Dermatol 2018 Aug 28;30(4):465-467. Epub 2018 Jun 28.

Department of Dermatology, Ewha Womans University College of Medicine, Seoul, Korea.

Mucinous nevus is an uncommon entity classified as either a cutaneous mucinosis or a connective tissue nevus. The condition presents as grouped papules and coalescent plaques growing in a unilateral or zosteriform manner. The key histopathological feature is a band-like deposition of mucin in the superficial dermis. Read More

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http://dx.doi.org/10.5021/ad.2018.30.4.465DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6029955PMC
August 2018
1 Read

Progress of an isolated collagenoma during pregnancy.

Australas J Dermatol 2018 Jul 24. Epub 2018 Jul 24.

Department of Pathology, Bahçeşehir University Faculty of Medicine, Istanbul, Turkey.

Collagenomas are connective tissue naevi composed predominantly of collagen. Isolated collagenomas are usually localized to a single body region, acquired, and of rare occurrence. We describe a patient with an isolated collagenoma that showed an increase in size during pregnancy and regressed afterwards. Read More

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http://dx.doi.org/10.1111/ajd.12876DOI Listing
July 2018
4 Reads

Congenital fibroblastic connective tissue nevi: Unusual and misleading presentations in three infantile cases.

Pediatr Dermatol 2018 Sep 19;35(5):644-650. Epub 2018 Jul 19.

Services de Chirurgie Maxillo-Faciale et Plastique, Assistance Publique-Hôpitaux de Paris, Hôpital Universitaire Necker Enfants-Malades, Université Sorbonne Paris Cité, Université Paris Descartes, Paris, France.

Background: Fibroblastic connective tissue nevi (FCTN) are benign skin conditions characterized by bland spindle cells infiltrating the reticular dermis and the upper subcutis with preservation of adnexal structures. A subset of FCTN expresses CD34, which may cause difficulties in the differential diagnosis, in particular with dermatofibrosarcoma (DFSP). We aim to study clinical and histological main features of congenital FCTN to better understand their heterogeneity. Read More

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http://dx.doi.org/10.1111/pde.13571DOI Listing
September 2018
4 Reads

Acquired elastoma in a subungual location.

Dermatol Online J 2017 Sep 15;23(9). Epub 2017 Sep 15.

Department of Dermatology, SUNY Downstate Medical Center, Brooklyn, New York.

Elastomas are connective tissue nevi or hamartomas. They may occur in isolation or can be associated with familial syndromes such as Buschke-Ollendorff syndrome. Elastomas typically present in childhood as small ivory papules or firm skin-colored nodules that can coalesce into larger yellow plaques. Read More

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September 2017
3 Reads

Satisfactory treatment of a large connective tissue nevus with intralesional steroid injection.

Dermatol Pract Concept 2018 Jan 31;8(1):12-14. Epub 2018 Jan 31.

Molecular Dermatology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.

Collagenoma is a type of connective tissue nevi, a rare hamartomatous malformation characterized by the predominant proliferation of normal collagen fibers and normal, decreased, or increased elastic fibers. Collagenomas present as multiple or solitary, hereditary or sporadic, asymptomatic, skin-colored papules, nodules, and plaques with variable sizes, and are usually located on the trunk, arm, and back. Here, we report on a 14-year-old boy who presented with an isolated giant collagenoma of the frontal area that dramatically responded to intralesional triamcinolone acetonide. Read More

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http://dx.doi.org/10.5826/dpc.0801a03DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5808365PMC
January 2018
6 Reads

Fibrous cephalic plaques in tuberous sclerosis complex.

J Am Acad Dermatol 2018 04 16;78(4):717-724. Epub 2017 Dec 16.

Department of Dermatology, Uniformed Services University of the Health Sciences, Bethesda, Maryland. Electronic address:

Background: Fibrous cephalic plaques (FCPs) stereotypically develop on the forehead of patients with tuberous sclerosis complex (TSC). They constitute a major feature for TSC diagnosis and may present before other TSC-related cutaneous hamartomas.

Objective: To describe the clinical characteristics of FCPs in TSC. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S01909622173285
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http://dx.doi.org/10.1016/j.jaad.2017.12.027DOI Listing
April 2018
16 Reads
4.450 Impact Factor

Pathogenetic insights from quantification of the cerebriform connective tissue nevus in Proteus syndrome.

J Am Acad Dermatol 2018 04 16;78(4):725-732. Epub 2017 Oct 16.

Department of Dermatology, Uniformed Services University of the Health Sciences, Bethesda, Maryland. Electronic address:

Background: The plantar cerebriform connective tissue nevus (CCTN) is the most common and problematic cutaneous manifestation of Proteus syndrome.

Objective: To gain insights into CCTN pathogenesis and natural history.

Methods: The size and location of plantar CCTN was measured on 152 images from 22 individuals with Proteus syndrome by 2 independent, blinded reviewers. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S01909622173254
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http://dx.doi.org/10.1016/j.jaad.2017.10.018DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5857242PMC
April 2018
8 Reads

Fibroblastic connective tissue nevus: the role of histopathological and molecular techniques in differential diagnosis.

Eur J Dermatol 2017 10;27(5):547-548

Department of Medicine of Sensory and Motor Organs, Division of Dermatology, Faculty of Medicine, Tottori University, 86 Nishi-Cho, Yonago, Tottori 683-8503, Japan.

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http://dx.doi.org/10.1684/ejd.2017.3093DOI Listing
October 2017
7 Reads

[Extensive connective tissue nevus in children].

Ann Dermatol Venereol 2017 Nov 29;144(11):700-704. Epub 2017 Jul 29.

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

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http://dx.doi.org/10.1016/j.annder.2017.06.006DOI Listing
November 2017
6 Reads

Fibroblastic connective tissue nevus: Clinicopathological and immunohistochemical study of 14 cases.

J Cutan Pathol 2017 Oct 19;44(10):827-834. Epub 2017 Jul 19.

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

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http://dx.doi.org/10.1111/cup.12993DOI Listing
October 2017
10 Reads

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

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http://dx.doi.org/10.1111/pde.13155DOI Listing
July 2017
8 Reads

Clinical Characteristics of Connective Tissue Nevi in Tuberous Sclerosis Complex With Special Emphasis on Shagreen Patches.

JAMA Dermatol 2017 07;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

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http://dx.doi.org/10.1001/jamadermatol.2017.0298DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5817464PMC
July 2017
14 Reads

Connective Tissue Nevi: A Review of the Literature.

Am J Dermatopathol 2017 May;39(5):325-341

*Medical Student MS3, Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, Miami, FL; †Resident in Dermatology (PGY2), University of Puerto Rico-Medical Sciences Campus, San Juan, Puerto Rico; ‡Researcher at Genetics and Molecular Medicine Laboratory, Department of Medicine, Universidad del Norte, Barranquilla, Atlántico, Colombia; §Department of Dermatology and ‖Faculty Dermatology and Dermatopathology, University of Pittsburgh Medical Center, Pittsburgh, PA; and ¶Professor of Dermatology and Dermatopathology, Director of Dermatopathology, University of Miami Miller School of Medicine, Miami, FL.

Connective tissue nevi (CTN) are hamartomas of the dermis, with the 3 main components being collagen, elastin, and proteoglycans. Each subtype can present as a solitary lesion or multiple lesions. They could present as part of systemic diseases or inherited disorders. Read More

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http://dx.doi.org/10.1097/DAD.0000000000000638DOI Listing
May 2017
1 Read

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

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http://dx.doi.org/10.4103/2277-9175.201684DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5360000PMC
March 2017
10 Reads

Collagenoma in a Patient With Down Syndrome: A Case Report and Review of the Literature.

Am J Dermatopathol 2018 May;40(5):355-357

Departments of Radiology, and.

A 26-year-old woman with Down syndrome presented with a 4-cm-sized palpable mass in the sacrococcygeal region. Histologic evaluation of the specimen revealed densely packed collagen fibers in the dermis, which is consistent with a collagenoma. Masson trichrome staining showed dense fibrosis, and elastic Van Gieson staining revealed a significant decrease in elastic tissue. Read More

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http://dx.doi.org/10.1097/DAD.0000000000000873DOI Listing
May 2018
3 Reads

Eruptive collagenoma in a child.

Dermatol Online J 2017 Jan 15;23(1). Epub 2017 Jan 15.

Dermatovenereology Department, Hospital Garcia de Orta, Almada, Portugal.

Eruptive collagenoma is a rare entity, with unknownetiology, considered to be a type of connective tissuenevus composed of collagen. It is usually reported inyoung adults occurring predominantly on the trunkand extremities. Systemic findings and family historyof a similar condition are not typically associated andthe prognosis is excellent. Read More

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January 2017
5 Reads

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

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http://dx.doi.org/10.1590/abd1806-4841.20164541DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5324988PMC
July 2017
13 Reads

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

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http://dx.doi.org/10.1097/DAD.0000000000000726DOI Listing
March 2017
7 Reads

Recurrent cerebriform connective tissue nevus on the foot of a patient with Proteus syndrome.

Cutis 2016 Oct;98(4):E16-E19

Institute of Dermatology, Chinese Academy of Medical Sciences, Peking Union Medical College, Nanjing, China.

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October 2016
17 Reads

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

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June 2016
10 Reads

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

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http://dx.doi.org/10.1111/pde.12896DOI Listing
July 2016
8 Reads

[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

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http://dx.doi.org/10.1016/j.annder.2016.03.017DOI Listing
June 2017
4 Reads

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

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http://dx.doi.org/10.5021/ad.2016.28.2.210DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4828385PMC
April 2016
13 Reads

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

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http://dx.doi.org/10.1111/pde.12671DOI Listing
September 2016
3 Reads

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

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http://dx.doi.org/10.1111/cup.12605DOI Listing
January 2016
5 Reads

[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

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http://doi.med.wanfangdata.com.cn/10.3760/cma.j.issn.1003-94
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http://dx.doi.org/10.3760/cma.j.issn.1003-9406.2015.04.012DOI Listing
August 2015
8 Reads

Fronto-temporal cerebriform connective tissue nevus in Proteus syndrome.

Authors:
S M Balaji

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

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http://dx.doi.org/10.4103/0970-9290.152215DOI Listing
October 2015
1 Read

Proposal of the new name "eruptive papular collageno-elastopathy" to unify the two indistinguishable entities, eruptive collagenoma and papular elastorrhexis.

J Dermatol 2015 Apr 21;42(4):440-1. Epub 2015 Feb 21.

Department of Dermatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.

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http://dx.doi.org/10.1111/1346-8138.12807DOI Listing
April 2015
3 Reads

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

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http://dx.doi.org/10.1111/pde.12456DOI Listing
April 2016
4 Reads

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

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http://dx.doi.org/10.1111/1346-8138.12735DOI Listing
February 2015
6 Reads

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

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August 2014
4 Reads

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

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http://dx.doi.org/10.1097/DAD.0000000000000163DOI Listing
February 2015
5 Reads

Linear connective tissue nevus along Blaschko's lines: literature review and case report.

Int J Dermatol 2015 25;54(5):e166-8. Epub 2014 Jun 25.

Department of Pathology, Faculty of Medicine, Universidade Estadual Paulista (UNESP) Botucatu, São Paulo, Brazil.

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http://dx.doi.org/10.1111/ijd.12602DOI Listing
January 2016
5 Reads

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

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http://dx.doi.org/10.1111/cup.12365DOI Listing
September 2014
8 Reads

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

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http://dx.doi.org/10.1001/jamadermatol.2013.10368DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4281031PMC
September 2014
22 Reads

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

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http://dx.doi.org/10.1155/2014/203298DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3976770PMC
April 2014
2 Reads

A 5-year-old with connective tissue nevi: Buschke-Ollendorff syndrome.

J Pediatr 2014 Jul 14;165(1):206. Epub 2014 Apr 14.

Pediatric Dermatology, Mayo Clinic, Rochester, Minnesota.

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http://dx.doi.org/10.1016/j.jpeds.2014.03.003DOI Listing
July 2014
4 Reads

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

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http://dx.doi.org/10.1111/cup.12311DOI Listing
June 2014
5 Reads

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

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http://dx.doi.org/10.4103/0974-7753.122969DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3877481PMC
April 2013
24 Reads
2 Citations

Linear collagen naevus: an unusual connective tissue disorder.

Acta Derm Venereol 2014 May;94(3):361-2

Department of Dermatology, Freiburg University Medical Center, 79104 Freiburg, Germany.

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http://dx.doi.org/10.2340/00015555-1747DOI Listing
May 2014
3 Reads

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

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http://dx.doi.org/10.1111/dth.12016DOI Listing
June 2014
10 Reads
2 Citations
1.480 Impact Factor

Proteus syndrome review: molecular, clinical, and pathologic features.

Authors:
M Michael Cohen

Clin Genet 2014 Feb 23;85(2):111-9. Epub 2013 Oct 23.

Department of Pediatrics, Faculty of Medicine, Dalhousie University, Halifax, Nova Scotia, Canada.

Proteus syndrome is caused by an activating AKT1 mutation (c.49G>A, p.Glu17Lys). Read More

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http://dx.doi.org/10.1111/cge.12266DOI Listing
February 2014
6 Reads

AKT1 gene mutation levels are correlated with the type of dermatologic lesions in patients with Proteus syndrome.

J Invest Dermatol 2014 Feb 24;134(2):543-546. Epub 2013 Jul 24.

National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland, USA.

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http://dx.doi.org/10.1038/jid.2013.312DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3868633PMC
February 2014
25 Reads
7 Citations
7.220 Impact Factor

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

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http://dx.doi.org/10.1097/PAS.0b013e31825e63bfDOI Listing
October 2012
4 Reads

[Eruptive collagenomas: a description of 2 cases].

An Pediatr (Barc) 2012 Sep 10;77(3):212-4. Epub 2012 May 10.

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https://linkinghub.elsevier.com/retrieve/pii/S16954033110056
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http://dx.doi.org/10.1016/j.anpedi.2011.11.012DOI Listing
September 2012
3 Reads

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

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http://dx.doi.org/10.3109/15513815.2012.656830DOI Listing
June 2012
8 Reads

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

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http://dx.doi.org/10.5021/ad.2011.23.S2.S243DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3229076PMC
October 2011
3 Reads