793 results match your criteria Epidermal Nevus Syndrome


Verruciform xanthoma overlying inflammatory linear verrucous epidermal nevus and in broad segmental distribution.

BMJ Case Rep 2018 Oct 17;2018. Epub 2018 Oct 17.

Department of Pathology, All India Institute of Medical Sciences, New Delhi, India.

A 17-year-old woman presented with moderately itchy, non-progressive, linearly arranged verrucous plaques over dorsum of left foot since early childhood. Two years ago, she developed slowly increasing, verrucous exophytic growth in posterior most aspect of linear verrucous plaque. One year ago, she also developed multiple, linearly arranged, fleshy plaques with surface crusting over lateral aspect of right leg extending to thigh. Read More

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http://dx.doi.org/10.1136/bcr-2018-225964DOI Listing
October 2018
3 Reads

Inflammatory linear verrucous epidermal nevus responsive to 308-nm excimer laser treatment.

Cutis 2018 Aug;102(2):111-114

Advanced Dermatology Associates LTD; Lehigh Valley Health Network, Allentown, Pennsylvania, USA.

Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare cutaneous disease that presents as linear psoriasiform plaques with associated prominent pruritus. The lesions commonly pre-sent on the legs with onset during childhood. Inflammatory linear verrucous epidermal nevus typically is refractory to treatment. Read More

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August 2018
5 Reads

Dermatoscopy of Common Lesions in Pediatric Dermatology.

Dermatol Clin 2018 Oct 16;36(4):463-472. Epub 2018 Aug 16.

Dermatology Clinic, University of Catania, Via S. Sofia 78, Catania 95123, Italy.

The use of dermatoscopy to assist in the diagnosis of a variety of proliferative, pigmentary, inflammatory, infectious, congenital, and genetic cutaneous and skin appendage disorders is constantly increasing, as it is effective, affordable, noninvasive, and quick to perform. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S07338635183108
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http://dx.doi.org/10.1016/j.det.2018.05.012DOI Listing
October 2018
16 Reads
1.434 Impact Factor

Dermoscopy of Adnexal Tumors.

Dermatol Clin 2018 Oct 16;36(4):397-412. Epub 2018 Aug 16.

Dermatology Department, Hospital General Universitario de Alicante, ISABIAL, C/Maestro Alonso 109, 03010 Alicante, Spain.

Cutaneous adnexal tumors include lesions with apocrine, eccrine, follicular, sebaceous, and mixed differentiation. Most are benign and sporadic, although malignant forms are occasionally observed and some cases develop in the setting of inherited syndromes. These tumors often cause immense diagnostic difficulty. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S07338635183107
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http://dx.doi.org/10.1016/j.det.2018.05.007DOI Listing
October 2018
15 Reads

Choroid Osteoma in Schimmelpenning-Feuerstein-Mims Syndrome.

Indian Pediatr 2018 07;55(7):601-602

Department of Ophthalmology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, India.

Background: Schimmelpenning syndrome is a multisystem disorder.

Case Characteristics: A term female neonate with sebaceous nevi of the face had choroid osteoma of the right eye.

Observation: At one month of age, the infant was observed to have choroidal neovascularization that was successfully treated with laser photo-coagulation and anti-VEGF. Read More

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July 2018
17 Reads
1.014 Impact Factor

Epidermal Nevi and Related Syndromes - Part 2: Nevi Derived from Adnexal Structures.

Actas Dermosifiliogr 2018 Oct 2;109(8):687-698. Epub 2018 Jul 2.

Servicio de Dermatología, Hospital de Manacor, Manacor, Islas Baleares, España.

Epidermal nevi are hamartomatous lesions derived from the epidermis and/or adnexal structures of the skin; they have traditionally been classified according to their morphology. New variants have been described in recent years and advances in genetics have contributed to better characterization of these lesions and an improved understanding of their relationship with certain extracutaneous manifestations. In the second part of this review article, we will look at nevi derived from the adnexal structures of the skin and associated syndromes. Read More

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http://dx.doi.org/10.1016/j.ad.2018.05.004DOI Listing
October 2018
11 Reads

Atypical Nevi in a Patient After Toxic Epidermal Necrolysis.

Acta Dermatovenerol Croat 2018 Jun;26(2):183-185

Ružica Jurakić Tončić, MD, Department of Dermatovenereology, University Hospital Centre Zagreb, University of Zagreb School of Medicine, Šalata 4, 10000 Zagreb, Croatia;

Dear Editor, There are few literature data about nevi in patients with a history of toxic epidermal necrolysis (TEN) and little recommendations for follow-up and risks of melanoma (MM). Eruptive melanocytic nevi (EMN) is a rare phenomenon that has been associated with bullous disorders, immunosuppression, and immunodeficiency, but in some cases can occur without precipitating factors (1). The etiology is largely unknown, but there is evidence that immunosuppression might play a crucial role in nevogenesis, probably due to the inability of the immune system to inhibit melanocytic (MC) proliferation (2,3). Read More

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June 2018
14 Reads

Inflammatory linear verrucous epidermal nevus associated with erosive monoarthritis.

Pediatr Dermatol 2018 Sep 4;35(5):e291-e293. Epub 2018 Jul 4.

Department of Dermatology, Hospital del Mar, Barcelona, Spain.

Inflammatory linear verrucous epidermal nevus and linear psoriasis are different entities with clinical and histopathologic similarities. Isolated reports of inflammatory linear verrucous epidermal nevus with concomitant psoriasis or a positive family history of psoriasis have been described, and the possibility that inflammatory linear verrucous epidermal nevus may be a mosaic form of cutaneous psoriasis has been postulated. We report a 17-year-old boy with a congenital, linear, erythematous, keratotic plaque on the dorsum of the fifth finger of the left hand with ipsilateral nail dystrophy. Read More

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http://dx.doi.org/10.1111/pde.13592DOI Listing
September 2018
16 Reads

Overexpression of Androgen, Oestrogen and Progesterone Receptors in Skin Lesions of Becker's Naevus.

Acta Derm Venereol 2018 Oct;98(9):867-872

Department of Dermatology, Affiliated Hospital of Guangdong Medical University, Zhanjiang, Guangdong, China.

Becker's naevus is androgen-dependent. The aim of this study was to investigate whether oestrogen and progesterone receptors are involved in this disorder. Immunohistochemistry showed that epidermal expression of androgen receptors, oestrogen receptors (α, β) and progesterone receptors was higher in skin lesions of Becker's naevus than in perilesional and control skin. Read More

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http://dx.doi.org/10.2340/00015555-2986DOI Listing
October 2018
3 Reads

Pulmonary thromboembolic events in patients with congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and spinal/skeletal abnormalities and Klippel-Trénaunay syndrome.

J Vasc Surg Venous Lymphat Disord 2018 07;6(4):511-516

Division of Vascular and Interventional Radiology, Vascular Anomalies Center, Boston Children's Hospital and Harvard Medical School, Boston, Mass. Electronic address:

Objective: Patients with Klippel-Trénaunay syndrome (KTS) and congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and spinal/skeletal abnormalities (CLOVES) syndrome have central phlebectasia and enlarged persistent embryonic veins that are often incompetent and prone to thromboembolism. The purpose of the study was to determine the presence of phlebectasia and the incidence of symptomatic pulmonary embolism (PE).

Methods: A retrospective review was conducted of patients referred to the Vascular Anomalies Center at our institution during a 21-year period who were diagnosed with KTS and CLOVES syndrome. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S2213333X183012
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http://dx.doi.org/10.1016/j.jvsv.2018.01.015DOI Listing
July 2018
12 Reads

Targeted therapy in patients with PIK3CA-related overgrowth syndrome.

Nature 2018 06 13;558(7711):540-546. Epub 2018 Jun 13.

INSERM U1151, Institut Necker Enfants Malades, Paris, France.

CLOVES syndrome (congenital lipomatous overgrowth, vascular malformations, epidermal naevi, scoliosis/skeletal and spinal syndrome) is a genetic disorder that results from somatic, mosaic gain-of-function mutations of the PIK3CA gene, and belongs to the spectrum of PIK3CA-related overgrowth syndromes (PROS). This rare condition has no specific treatment and a poor survival rate. Here, we describe a postnatal mouse model of PROS/CLOVES that partially recapitulates the human disease, and demonstrate the efficacy of BYL719, an inhibitor of PIK3CA, in preventing and improving organ dysfunction. Read More

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http://dx.doi.org/10.1038/s41586-018-0217-9DOI Listing
June 2018
30 Reads
1 Citation
42.351 Impact Factor

A call to study orphan diseases.

Authors:
Faizan Alawi

Oral Surg Oral Med Oral Pathol Oral Radiol 2018 08 22;126(2):95-97. Epub 2018 May 22.

Associate Professor of Pathology, University of Pennsylvania, School of Dental Medicine, 240 South 40th Street, Room 328B, Philadelphia, PA 19104, USA.

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https://linkinghub.elsevier.com/retrieve/pii/S22124403183093
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http://dx.doi.org/10.1016/j.oooo.2018.05.003DOI Listing
August 2018
2 Reads

Extensive Nevus Comedonicus, Complicated with Recurrent Abscesses, Successfully Treated with Surgical Resurfacing.

J Cutan Aesthet Surg 2018 Jan-Mar;11(1):33-37

Department of Plastic Surgery, St Johns' Medical College and Hospital, Bengaluru, Karnataka, India.

Nevus comedonicus is a rare epidermal abnormality of the pilosebaceous unit, which is congenital in most patients but may also appear early in childhood. It may be localized or have an extensive involvement, the latter showing a unilateral predominance with only a few cases presenting bilaterally. Extensive nevus comedonicus can be associated with musculoskeletal defects, eye and neurological involvement, which constitutes nevus comedonicus syndrome. Read More

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http://www.jcasonline.com/text.asp?2018/11/1/33/229976
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http://dx.doi.org/10.4103/JCAS.JCAS_122_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5921448PMC
May 2018
20 Reads

Burosumab: First Global Approval.

Authors:
Yvette N Lamb

Drugs 2018 Apr;78(6):707-714

Springer, Private Bag 65901, Mairangi Bay, Auckland, 0754, New Zealand.

Burosumab (Crysvita; Kyowa Hakko Kirin Co., Ltd. and Ultragenyx Pharmaceutical Inc. Read More

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http://link.springer.com/10.1007/s40265-018-0905-7
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http://dx.doi.org/10.1007/s40265-018-0905-7DOI Listing
April 2018
14 Reads

Reconstruction of a buttock defect after excision of inflammatory linear verrucous epidermal nevus.

Dermatol Ther 2018 May 6;31(3):e12597. Epub 2018 Apr 6.

Institute of Dermatology, Jiangsu Key Laboratory of Molecular Biology for Skin Diseases and STIs, Chinese Academy of Medical Science & Peking Union Medical College, Nanjing, China.

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http://dx.doi.org/10.1111/dth.12597DOI Listing
May 2018
5 Reads

Basaloid Follicular Hamartoma: A Case Report and a Novel Cosmetic Treatment.

J Clin Aesthet Dermatol 2018 Mar 1;11(3):39-41. Epub 2018 Mar 1.

Dr. Segars is with NSU-COM/Largo Medical Center Dermatology Residency in Largo, Florida.

Basaloid follicular hamartoma (BFH) is a rare, benign neoplasm of the hair follicle, characterized by multiple brown papules involving the face, scalp, and trunk. It is described by multiple clinical forms, and can present as localized or generalized. Diagnosis is made histologically via biopsy, which is important in order to distinguish BFH from basal cell carcinoma (BCC) or other malignant epithelial neoplasms. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5868784PMC
March 2018
5 Reads

Familial papular epidermal nevus with "skyline" basal cell layer.

Pediatr Dermatol 2018 May 25;35(3):e191-e192. Epub 2018 Mar 25.

Department of Dermatology, Complejo Asistencial Universitario de Salamanca, Salamanca, Spain.

Papular epidermal nevus with "skyline" basal cell layer is a variant of keratinocytic nevus that usually occurs sporadically but may affect different family members. We report on the fourth family with papular epidermal nevus with "skyline" basal cell layer affecting a 3-month-old girl and her father. Read More

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http://dx.doi.org/10.1111/pde.13449DOI Listing
May 2018
8 Reads

Syringocystadenoma papilliferum and trichoblastoma arising in the nevus sebaceous.

Indian J Pathol Microbiol 2018 Jan-Mar;61(1):106-108

Department of Dermatology and Venerology, China-Japan Friendship Hospital, Beijing, China.

Nevus sebaceous (NS) is a clinically common benign tumor and has a high potential to develop into a great diversity of neoplasms of epidermal and adnexal origins. However, it is a rare phenomenon of the coexistence in a single NS with two or more skin tumors. We report a case of a 58-year-old woman with two kinds of neoplastic proliferation including syringocystadenoma papilliferum and trichoblastoma arising in NS on the scalp. Read More

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http://dx.doi.org/10.4103/IJPM.IJPM_613_16DOI Listing
November 2018
1 Read

Biodynamic excisional skin tension lines for surgical excisions: untangling the science.

Authors:
S P Paul

Ann R Coll Surg Engl 2018 Apr 15;100(4):330-337. Epub 2018 Mar 15.

University of Queensland, School of Medicine , Brisbane, Queensland , Australia.

Objective There remains confusion between Langer's lines and wrinkle lines with respect to the optimal orientation of elliptical excisions on the trunk. This study sought to determine the directions of wound closure that would result in least wound tension after skin lesion excisions. Materials and methods Some 1181 consecutive skin lesion excisions were investigated (age range 13-95 years, median 64 years) using a tensiometer to determine directions of least wound tension. Read More

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http://dx.doi.org/10.1308/rcsann.2018.0038DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5958865PMC
April 2018
22 Reads

Yellow-orange hairless plaque on the scalp.

Authors:
Ahdi Amer

Cutis 2018 Jan;101(1):E10-E12

Wayne State University School of Medicine, Detroit, Michigan, USA.

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January 2018
3 Reads

Phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha (PIK3CA)-related overgrowth spectrum: A brief report.

Pediatr Dermatol 2018 May 1;35(3):e186-e188. Epub 2018 Mar 1.

Department of Human Genetics, Katholieke Universiteit Leuven, Leuven, Belgium.

A patient with extensive multisystem overgrowth caused by a somatic gain of function PIK3CA-mutation is described. This case is an example of the clinical diversity of the PIK3CA-Related Overgrowth Spectrum (PROS) as the patient had overlapping features of Congenital Lipomatous Overgrowth Vascular malformations Epidermal nevi and Skeletal abnormalities (CLOVES) syndrome and Megalencephaly-Capillary malformation Polymicrogyria (MCAP) syndrome and underlines the utility of this umbrella term. Read More

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http://dx.doi.org/10.1111/pde.13441DOI Listing
May 2018
12 Reads

Familial papular epidermal nevus with "skyline" basal cell layer and multiple pilomatricomas: A new association?

Pediatr Dermatol 2018 May 28;35(3):e147-e150. Epub 2018 Feb 28.

Division of Dermatology, "U.O. Multizonale," S. Chiara Hospital, Outpatient Consultation for Rare Diseases, Trento, Italy.

Papular epidermal nevus with "skyline" basal cell layer is a newly described keratinocytic nevus. Recently, papular epidermal nevus with "skyline" basal cell layer has been reported in association with extracutaneous involvement, and the term papular epidermal nevus with "skyline" basal cell layer syndrome is used to indicate a neurocutaneous syndrome characterized by the presence of papular epidermal nevus with "skyline" basal cell layer and different neurologic symptoms that seem to improve during infancy and adolescence. Multiple pilomatricomas have been reported in association with various syndromes. Read More

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http://dx.doi.org/10.1111/pde.13428DOI Listing
May 2018
6 Reads

Management of naevus sebaceous: a national survey of UK dermatologists and plastic surgeons.

Clin Exp Dermatol 2018 Jul 20;43(5):589-591. Epub 2018 Feb 20.

Dermatology Department, Churchill Hospital, Oxford University Hospitals NHS Foundation Trust, Oxford, UK.

Naevus sebaceous (NS) is a congenital cutaneous hamartoma, which typically occurs on the head and neck. Historically, the treatment of choice was excision in infancy because of the potential for malignant transformation; however, recent studies suggest that this risk is < 1% and unlikely in childhood. We sent a questionnaire to UK dermatologists and plastic surgeons to investigate current management practice of NS. Read More

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http://dx.doi.org/10.1111/ced.13422DOI Listing
July 2018
2 Reads

Syndromic sebaceous nevus: current findings.

Int J Dermatol 2018 May 16;57(5):599-604. Epub 2018 Feb 16.

Service of Pediatric Orthopedic Plastic Surgery, Hôpital Lapeyronie, Montpellier, France.

Background: Sebaceous nevus is a congenital malformation of the skin that usually occurs on the scalp or face. Syndromic forms do rarely exist with associated cerebral and ocular malformations. The skin lesions are pale at birth and become irregular by puberty. Read More

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http://dx.doi.org/10.1111/ijd.13942DOI Listing
May 2018
9 Reads

An institution-wide algorithm for direct-stick embolization of peripheral venous malformations.

J Vasc Surg Venous Lymphat Disord 2018 05 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

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http://dx.doi.org/10.1016/j.jvsv.2017.12.011DOI Listing
May 2018
33 Reads

Somatic KRAS mutation in an infant with linear nevus sebaceous syndrome associated with lymphatic malformations: A case report and literature review.

Medicine (Baltimore) 2017 Nov;96(47):e8016

Department of Pediatric Neurology, the Children's Hospital, School of Medicine, Zhejiang University, Hangzhou, Zhejiang, China.

Rationale: Linear nevus sebaceous syndrome (LNSS) is a rare neurocutaneous syndrome, characterized by nevus sebaceous,central nervous system (CNS), ocular and skeletal abnormalities. The present study describes KRAS somatic mosaic mutation in a case of LNSS with lymphatic malformations (LMs).

Patient Concerns: A 4-month-old female with a clinical diagnosis of LNSS presented with infantile spasms, mental retardation, skull dysplasia, ocular abnormalities, congenital atrial septal defect, and LMs. Read More

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http://dx.doi.org/10.1097/MD.0000000000008016DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5708909PMC
November 2017
7 Reads

Rare desmoplastic trichilemmoma associated with sebaceous nevus.

An Bras Dermatol 2017 Nov-Dec;92(6):836-837

Private Clinic - São Paulo (SP), Brazil.

Nevus sebaceous of Jadassohn is a congenital hamartoma that usually affects the scalp and face. Several benign or malignant neoplasias may develop in the lesion and the most common are trichoblastoma, syringocystadenoma papilliferum, and basal cell carcinoma. Trichilemmoma is a benign solid tumor originating from external sheath cells of pilosebaceous follicles. Read More

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http://www.scielo.br/scielo.php?script=sci_arttext&pid=S
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http://dx.doi.org/10.1590/abd1806-4841.20176540DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5786400PMC
April 2018
8 Reads

CHILD syndrome mimicking verrucous nevus in a Chinese patient responded well to the topical therapy of compound of simvastatin and cholesterol.

J Eur Acad Dermatol Venereol 2018 Jul 1;32(7):1209-1213. Epub 2018 Feb 1.

Department of Dermatology, Xinhua Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China.

Background: Congenital hemidysplasia with ichthyosiform erythroderma and limb defects (CHILD) syndrome is a rare X-linked dominant disorder characterized by peculiar cutaneous presentations and ipsilateral skeletal abnormalities. CHILD syndrome is caused by mutations in NSDHL gene, which involves in cholesterol synthesis.

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

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http://dx.doi.org/10.1111/jdv.14788DOI Listing
July 2018
2 Reads

[Phakomatosis pigmentovascularis cesioflammea: a case report].

Arch Argent Pediatr 2018 Feb;116(1):e121-e124

Servicio de Dermatología, Hospital Nacional de Pediatría "Prof. Dr. J. P. Garrahan", Ciudad Autónoma de Buenos Aires.

Phakomatosis pigmentovascularis (PPV) is a syndrome characterized by the association of a vascular nevus with a congenital pigmented lesion (epidermal nevus, nevus spilus, and dermal melanocytosis). There are different types of PPV according to the pigmentary nevus associated with the vascular malformation. Patients may present only the cutaneous condition or have systemic manifestations, among them, trauma, neurological and ophthalmological disorders. Read More

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http://dx.doi.org/10.5546/aap.2018.e121DOI Listing
February 2018
7 Reads

Eruptive melanocytic nevi in a patient with toxic epidermal necrolysis-like cutaneous lupus.

Lupus 2018 06 4;27(7):1220-1222. Epub 2018 Jan 4.

1 Unidad de Gestión Clínica de Dermatología, 16824 Hospital Universitario Puerta del Mar , Cádiz, Spain.

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http://dx.doi.org/10.1177/0961203317751064DOI Listing
June 2018
1 Read

Picosecond 532-nm neodymium-doped yttrium aluminum garnet laser-a promising modality for the management of verrucous epidermal nevi.

Lasers Med Sci 2018 Apr 3;33(3):597-601. Epub 2018 Jan 3.

Photodermatoses Clinic, Laser Unit, Dermatology Department, Rabin Medical Center, Petach Tikva, Israel.

The verrucous epidermal nevus (VEN) is the most common type of epidermal nevi. As lesions can be disfiguring, treatment is often sought. Many therapeutic approaches have been reported, with variable efficacy and safety. Read More

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http://dx.doi.org/10.1007/s10103-017-2427-zDOI Listing
April 2018
23 Reads

Intratarsal keratinous eyelid cysts in Gorlin syndrome: A review and reappraisal.

Surv Ophthalmol 2018 Sep - Oct;63(5):711-718. Epub 2017 Dec 27.

Department of Ophthalmology, Harvard Medical School, Boston, Massachusetts, USA; Ophthalmic Plastic and Reconstructive Surgery Service, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, USA.

A 38-year-old woman presented with multiple bilateral recurrent eyelid cysts. Her medical history was notable for Gorlin (nevoid basal cell carcinoma) syndrome. Histopathologic and immunohistochemical examinations revealed that the lesions were intratarsal keratinous cysts. Read More

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http://dx.doi.org/10.1016/j.survophthal.2017.12.007DOI Listing
September 2018
11 Reads

Wooly hair nevus.

An Bras Dermatol 2017 ;92(5 Suppl 1):163-165

Department of Dermatology at Hospital Naval Marcílio Dias (HNMD) - Rio de Janeiro (RJ), Brazil.

Woolly hair nevus is a rare condition characterized by a structural anomaly of the hair, restricted to certain areas of the scalp. The hair becomes coiled and slightly hypopigmented. The term woolly hair refers to changes that affect all the scalp and has a hereditary character. Read More

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http://dx.doi.org/10.1590/abd1806-4841.20175289DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5726711PMC
March 2018
4 Reads

Unusual Cause of West Syndrome.

J Pediatr Neurosci 2017 Jul-Sep;12(3):288-290

Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Schimmelpenning-Feuerstein-Mims syndrome is a congenital neurocutaneous disorder, comprising of organoid epidermal nevus with a broad spectrum of multiorgan dysfunction (neurologic, skeletal, cardiovascular, ophthalmic, and urologic) secondary to postzygotic mutation in the early embryonic period. Predominant neurological manifestations include epilepsy, intellectual impairment, and focal deficits. Here, we report a 3-year-old girl who presented with epileptic spasms and had a characteristic linear sebaceous nevus. Read More

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http://dx.doi.org/10.4103/jpn.JPN_24_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5696673PMC
December 2017
5 Reads

Nevus Sebaceous at an Unusual Location: A Rare Presentation.

Chin Med J (Engl) 2017 Dec;130(23):2897-2898

Department of Pathology, Tianjin Academy of Traditional Chinese Medicine Affiliated Hospital, Tianjin 300120, China.

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http://dx.doi.org/10.4103/0366-6999.219147DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5717878PMC
December 2017
4 Reads

Acne arising on a facial Becker nevus following the lines of Blaschko.

An Bras Dermatol 2017 Sep-Oct;92(5):740-742

Department of Dermatology of the Escola Paulista de Medicina at Universidade Federal de São Paulo (UNIFESP) - São Paulo (SP), Brazil.

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http://dx.doi.org/10.1590/abd1806-4841.20176588DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5674718PMC
September 2018
13 Reads

Is Ki-67, keratin 16, involucrin, and filaggrin immunostaining sufficient to diagnose inflammatory linear verrucous epidermal nevus? A report of eight cases and a comparison with psoriasis vulgaris.

An Bras Dermatol 2017 Sep-Oct;92(5):682-685

Department of Dermatology, Affiliated Hospital of Guangdong Medical University, Zhanjiang, Guangdong, China.

Inflammatory linear verrucous epidermal nevus and linear psoriasis are sometimes hard to differentiate clinically and pathologically. Although immunohistochemical expression of keratin 10 (K10), K16, Ki-67, and involucrin may be useful for differentiating both entities, these results have been reported in only a few cases. We collected data from 8 patients with inflammatory linear verrucous epidermal nevus, 11 with psoriasis vulgaris, and 8 healthy controls and evaluated immunohistochemical expression of Ki-67, K16, involucrin, and filaggrin among them. Read More

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http://dx.doi.org/10.1590/abd1806-4841.20176263DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5674702PMC
February 2018
3 Reads

A case report of the first application of culture epithelial autograft (JACE) for giant congenital melanocytic nevus after its approval in Japan.

J Artif Organs 2018 Jun 17;21(2):261-264. Epub 2017 Nov 17.

Department of Plastic and Reconstructive Surgery, Kansai Medical University, 2-5-1 Shin-machi, Hirakata, 573-1010, Japan.

In Japan, the JACE cultured epidermal autograft (CEA) was approved and covered by public healthcare insurance for use in the treatment of giant congenital melanocytic nevus (GCMN) in 2016. We herein report the results of the application of JACE after curettage and Q-switched ruby laser therapy. The current patient was the first patient with GCMN to be treated with JACE since its approval. Read More

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http://dx.doi.org/10.1007/s10047-017-1007-0DOI Listing
June 2018
17 Reads

Detection of somatic mutations in secondary tumors associated with nevus sebaceus by targeted next generation sequencing. Comment on Kitamura et al.

Int J Dermatol 2018 01 31;57(1):120-122. Epub 2017 Oct 31.

Department of Pathology, Immunology, and Laboratory Medicine, University of Florida College of Medicine, Gainesville, FL, USA.

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http://dx.doi.org/10.1111/ijd.13758DOI Listing
January 2018
9 Reads

Epidermal nevus syndromes: New insights into whorls and swirls.

Pediatr Dermatol 2018 Jan 16;35(1):21-29. Epub 2017 Oct 16.

Department of Dermatology, University of California, San Francisco, Santa Rosa, CA, USA.

Knowledge of the molecular underpinnings of many epidermal nevi and epidermal nevus syndrome has expanded rapidly in recent years. In this review and update on epidermal nevus syndrome, we will cover recent genetic discoveries involving epidermal nevi, including nevus sebaceus, keratinocytic epidermal nevus, nevus comedonicus, congenital hemidysplasia with ichthyosiform nevus and limb defects syndrome, phakomatosis pigmentokeratotica, Becker's nevus, porokeratotic adnexal ostial nevus, inflammatory linear verrucous epidermal nevi, and cutaneous-skeletal hypophosphatemia syndrome. We will discuss how newly defined mutations relate to the biology reflected in the cutaneous patterns seen in these mosaic disorders and how new molecular data has informed our understanding of these diseases and shaped management decisions. Read More

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http://dx.doi.org/10.1111/pde.13273DOI Listing
January 2018
23 Reads

A Case of Phacomatosis Pigmentokeratotica Associated With Multiple Basal Cell Carcinomas.

Am J Dermatopathol 2018 Feb;40(2):131-135

Department of Dermatology, College of Medicine, Kyung Hee University, Seoul, Korea.

Phacomatosis pigmentokeratotica is a recently identified disease characterized by the coexistence of an epidermal nevus with sebaceous differentiation arranged along Blaschko lines and a speckled lentiginous nevus showing a checkerboard pattern, mostly in association with various extracutaneous defects. A 52-year-old man presented with asymptomatic ulcerative plaque on his left side of perioral area. A 2 × 2-cm-sized fleshy colored ulceration on his left perioral area was consistent with basal cell carcinoma with nevus sebaceous. Read More

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http://dx.doi.org/10.1097/DAD.0000000000001012DOI Listing
February 2018
8 Reads

Pigmented Trichoblastoma of Nose: An Unusual Occurrence.

J Clin Diagn Res 2017 Jul 1;11(7):MD09-MD10. Epub 2017 Jul 1.

Junior Resident, Department of Ear, Nose and Throat, Sri Siddhartha Medical College, Agalakote, Karnataka, India.

Nevus sebaceus of Jadassohn is a congenital tumour affecting the scalp and face. It is usually presented as a pigmented patch or plaque. It is a complex cutaneous hamartoma which involves pilosebaceous follicle, epidermis and adnexal structures. Read More

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http://dx.doi.org/10.7860/JCDR/2017/25798.10321DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5583918PMC
July 2017
9 Reads

A case of congenital lipomatous overgrowth, vascular malformations, epidermal nevi, spinal/skeletal anomalies and/or scoliosis syndrome with lipoatrophy as an important clinical manifestation.

Pediatr Dermatol 2017 Nov 22;34(6):735-736. Epub 2017 Aug 22.

Division of Paediatric Dermatology, CHU de Québec, Québec, Canada.

Congenital lipomatous overgrowth, vascular malformations, epidermal nevi, spinal/skeletal anomalies and/or scoliosis syndrome is a PIK3CA-related overgrowth spectrum presenting with congenital, asymmetric, disproportionate overgrowth associated with dysregulated adipose tissue, enlarged bony structures, and mixed primarily truncal vascular malformations. We present this case to raise awareness that very thin body habitus (lipoatrophy) contrasting with areas of overgrowth can be an important clinical feature of this syndrome and, if not recognized, can lead to unnecessary investigations. Read More

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http://dx.doi.org/10.1111/pde.13256DOI Listing
November 2017
11 Reads

Wilms tumor screening in diffuse capillary malformation with overgrowth and macrocephaly-capillary malformation: A retrospective study.

J Am Acad Dermatol 2017 Nov 16;77(5):874-878. Epub 2017 Aug 16.

Department of Dermatology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts; Vascular Anomalies Center, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts. Electronic address:

Background: CLOVES (congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and skeletal anomalies) syndrome is associated with regional bony and/or soft tissue overgrowth, capillary malformation, and an increased risk for Wilms tumor.

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

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

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http://dx.doi.org/10.1016/j.jaad.2017.06.014DOI Listing
November 2017
23 Reads

Transcatheter embolization of persistent embryonic veins in venous malformation syndromes.

J Vasc Surg Venous Lymphat Disord 2017 09;5(5):749-755

Division of Hematology and Oncology, Department of Pediatrics, Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ; Cancer Institute of New Jersey at the Bristol-Myers Squibb Children's Hospital, Robert Wood Johnson University Hospital, New Brunswick, NJ.

Persistent embryonic veins represent a major source of venous hypertension and morbidity in venous malformation syndromes, such as Klippel-Trénaunay syndrome and congenital lipomatous overgrowth, vascular malformations, epidermal nevus, and skeletal deformities syndrome. Surgical stripping and phlebectomy are the most commonly reported alternatives to compression therapy for refractory cases. These techniques, although effective in those patients who meet the necessary anatomic criteria, can be associated with bleeding, wound-related complications, and recurrence. Read More

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http://dx.doi.org/10.1016/j.jvsv.2017.05.016DOI Listing
September 2017
18 Reads

Giant Cutaneous Horn Arising in an Epidermal Nevus.

Pediatr Dermatol 2017 Sep 7;34(5):e290-e291. Epub 2017 Aug 7.

MedStar Washington Hospital Center, MedStar Georgetown University Hospital and Children's National Health System, Washington, DC.

Cutaneous horns, which rarely occur in children, can overlie a large array of pathologic lesions that range from benign to malignant. When a cutaneous horn is encountered, it is not the horn itself that is of relevance, it is the nature of the underlying disease that dictates overall prognosis and management. In this report we present a case of a cutaneous horn arising within a linear verrucous epidermal nevus and briefly review cutaneous horns, with an emphasis on their clinical implications and differential diagnosis. Read More

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http://dx.doi.org/10.1111/pde.13221DOI Listing
September 2017
11 Reads

Multiple Basal Cell Carcinoma Arising in a Verrucous Epidermal Naevus: Clinical, Histological and Therapeutic Observations.

Acta Derm Venereol 2018 01;98(1):132-133

Department of Dermatology, University of Parma, via Gramsci 14, IT-43100 Parma, Italy.

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http://dx.doi.org/10.2340/00015555-2753DOI Listing
January 2018
11 Reads

CLOVE Syndrome.

Indian J Pediatr 2018 01 28;85(1):79-80. Epub 2017 Jul 28.

Pediatric Neurology and Neurodevelopment Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

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http://dx.doi.org/10.1007/s12098-017-2416-zDOI Listing
January 2018
12 Reads
0.920 Impact Factor