99 results match your criteria Speckled Lentiginous Nevus

The first case of Chinese phacomatosis pigmentokeratotica diagnosed by a missense HRAS mosaicism.

J Dermatol 2022 May 13. Epub 2022 May 13.

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

Phacomatosis pigmentokeratotica (PPK) is a rare epidermal nevus (EN) syndrome, featured by co-occurrence of speckled lentiginous nevus (SLN) and nevus sebaceous (NS). The underlying mechanism has not been clarified. Pathogenic mutations in HRAS, KRAS and BRAF gene are recently recognized as the genetic cause of PPK. Read More

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Sudden colour enhancement of congenital speckled lentiginous naevus after partial resection.

Australas J Dermatol 2021 May 2;62(2):e355-e356. Epub 2021 Feb 2.

Department of Dermatology, Faculty of Medicine, Fukuoka University, Fukuoka, Japan.

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Speckled lentiginous nevi in a young African-American woman.

Int J Dermatol 2021 Jan 9;60(1):e32-e33. Epub 2020 Aug 9.

Wayne State University Department of Dermatology, Detroit, MI, USA.

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January 2021

Speckled lentiginous nevus in a patient with Hermansky-Pudlak syndrome type 1.

J Dermatol 2020 Jan 17;47(1):e20-e21. Epub 2019 Oct 17.

Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan.

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January 2020

Papular nevus spilus syndrome: old and new aspects of a mosaic RASopathy.

Eur J Dermatol 2019 Feb;29(1):2-5

Department of Dermatology, Medical Center-University of Freiburg, Hauptstr 7, 79104 Freiburg, Germany.

The co-existence of papular nevus spilus (PNS) and ipsilateral extracutaneous abnormalities involving peripheral nerves of the skin or muscles was originally described as "speckled lentiginous nevus syndrome". To avoid confusion with macular nevus spilus, the condition was recently re-named "papular nevus spilus syndrome". In addition to 14 published cases, we identified six new cases via a search of the worldwide literature. Read More

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February 2019

Dermpath & Clinic: In situ melanoma arising within a speckled lentiginous nevus.

Eur J Dermatol 2018 Dec;28(6):857-859

Department of Dermatology, Venereology and Allergology, University Hospital Würzburg, Josef-Schneider-Str 2, 97080 Würzburg, Germany.

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December 2018

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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Speckled lentiginous nevus: A rare presentation associated with motor neuropathy and muscular atrophy in a child.

Pediatr Dermatol 2018 May 24;35(3):e161-e162. Epub 2018 Mar 24.

Southern California Permanente Medical Group, Pasadena, CA, USA.

Speckled lentiginous nevus syndrome has been described in individuals with a speckled lentiginous nevus with rare associated neurologic deficits. Because speckled lentiginous nevus syndrome almost always affects adults, it is not typically considered when evaluating children. We present the first reported case of speckled lentiginous nevus syndrome presenting in a young child with muscle atrophy and motor deficits affecting muscles along the same distribution as the speckled lentiginous nevus. Read More

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Desmoplastic transformation of a nodular melanoma arising from a speckled lentiginous nevus.

J Dermatol 2018 Aug 21;45(8):e230-e231. Epub 2018 Feb 21.

Department of Plastic and Reconstructive Surgery, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan.

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Phacomatosis pigmentokeratotica: a case of HRAS mosaicism causing rhabdomyosarcoma.

Br J Dermatol 2018 11 9;179(5):1163-1167. Epub 2018 May 9.

Department of Pathology, Hospital Universitario 12 de Octubre, I+12 Research Institute, Universidad Complutense, Madrid, Spain.

A 17-year-old male presented with a large sebaceous naevus (SN) comprising part of his right face and scalp and a speckled lentiginous naevus (SLN) on his left trunk, hip, neck and scalp with a checkerboard pattern. His right oral hemimucosa showed extensive papillomatous lesions, which were contiguous with the upper-lip SN lesions. He also showed extracutaneous manifestations including cardiac, musculoskeletal and ocular alterations. Read More

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November 2018

Speckled lentiginous naevus: understanding the process of development and regression.

Br J Dermatol 2018 06 17;178(6):1447-1448. Epub 2018 Apr 17.

Department of Dermatology, Yamaguchi University Graduate School of Medicine, Yamaguchi, Japan.

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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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February 2018

Vitiligo developing in congenital segmental speckled lentiginous nevus: Another example of immunocompromised cutaneous district due to immunological assault on aberrant melanocytes?

Indian J Dermatol Venereol Leprol 2017 Sep-Oct;83(5):612-614

Department of Dermatology, Katihar Medical College and Hospital, Katihar, Bihar, India.

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November 2018

Phacomatosis Pigmentokeratotica: A Mosaic RASopathy with Malignant Potential.

Pediatr Dermatol 2017 May;34(3):352-355

Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina, Charleston, South Carolina.

Phacomatosis pigmentokeratotica (PPK) is a rare epidermal nevus syndrome characterized by the co-occurrence of a nevus sebaceous arranged along the lines of Blaschko with a speckled lentiginous nevus (SLN). We report a novel KRAS mutation in a patient with a large nevus sebaceous and an SLN who subsequently developed a vaginal botryoid rhabdomyosarcoma, an association not previously reported in the literature. This case expands our knowledge of the genetic basis for phacomatosis, in which mutations in HRAS have been previously described, although this report provides evidence that activating mutations in KRAS or HRAS may cause PPK. Read More

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Nevus Spilus (Speckled Lentiginous Nevus) in the Oral Cavity: Report of a Case and Review of the Literature.

Am J Dermatopathol 2017 Jan;39(1):e8-e12

*Department of Oral and Maxillofacial Pathology, Naval Postgraduate Dental School, Bethesda, MD; and †Department of Pathology, Walter Reed National Medical Military Center, Bethesda, MD.

The congenital melanocytic nevus is a pigmented melanocytic lesion that presents at birth or shortly thereafter. It is commonly described on the skin, usually on the trunk and extremities. Only five intraoral cases of congenital melanocytic nevi have been described in the English literature. Read More

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January 2017

Nevus spilus: is the presence of hair associated with an increased risk for melanoma?

Cutis 2016 Sep;98(3):171-174

Department of Dermatology, University of Mississippi Medical Center, Jackson, USA.

Nevus spilus (NS), also known as speckled lentiginous nevus, is characterized by background café au lait-like lentiginous melanocytic hyperplasia speckled with small, 1- to 3-mm, darker foci. Nevus spilus occurs in 1.3% to 2. Read More

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September 2016

Phacomatosis pigmentokeratotica or the Schimmelpenning-Feuerstein-Mims syndrome?

Clin Case Rep 2016 Jun 4;4(6):564-7. Epub 2016 May 4.

Department of the Medical Genetics of the Russian Medical Academy of Postgraduate Education Barrikadnaya Str. 2/1 Moscow 125993 Russia.

Cutaneous symptoms in some patients with clinical picture of Schimmelpenning-Feuerstein-Mims syndrome can include a speckled lentiginous nevus, also known as nevus spilus. Recent investigations show that somatic heterozygous HRAS mutations are present in the sebaceous and speckled lentiginous nevus tissues of patients with combination of two nevi. Read More

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Speckled lentiginous nevus syndrome.

Int J Dermatol 2016 Nov;55(11):e602-e604

Department of Dermatology, Hospital of the University of Brasilia, Brasilia, Brazil.

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November 2016

Mosaic Neurocutaneous Disorders and Their Causes.

Semin Pediatr Neurol 2015 Dec 12;22(4):207-33. Epub 2015 Nov 12.

Department of Clinical and Experimental Medicine, Section of Pediatrics and Child Neuropsychiatry, University of Catania, Catania, Italy; Department of Biomedical and Biotechnological Sciences, University of Catania, Catania, Italy.

Neurocutaneous disorders are a heterogeneous group of conditions (mainly) affecting the skin [with pigmentary/vascular abnormalities and/or cutaneous tumours] and the central and peripheral nervous system [with congenital abnormalities and/or tumours]. In a number of such disorders, the skin abnormalities can assume a mosaic patterning (usually arranged in archetypical patterns). Alternating segments of affected and unaffected skin or segmentally arranged patterns of abnormal skin often mirror similar phenomena occurring in extra-cutaneous organs/tissues [eg, eye, bone, heart/vessels, lung, kidney and gut]. Read More

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December 2015

Speckled lentiginous nevus syndrome associated with an abnormality of tongue.

J Dermatol 2015 Apr 21;42(4):433-4. Epub 2015 Feb 21.

Department of Dermatology, Peking University People's Hospital, Beijing, China.

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Nodular Melanoma Arising in a Large Segmental Speckled Lentiginous Nevus.

Am J Dermatopathol 2015 Aug;37(8):663-4

*Department of Dermatology, Rambam Medical Center, Haifa, Israel †The Ruth and Bruce Rappaport Faculty of Medicine, Haifa, Israel.

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Combined melanocytic and sweat gland neoplasm: cell subsets harbor an identical HRAS mutation in phacomatosis pigmentokeratotica.

J Cutan Pathol 2014 Aug 9;41(8):663-71. Epub 2014 Jul 9.

Department of Medicine, Dermatology Service, Memorial Sloan-Kettering Cancer Center, New York, NY, USA.

Phacomatosis pigmentokeratotica (PPK) is characterized by the co-existence of epidermal nevi and large segmental speckled lentiginous nevi of the papulosa type. PPK, previously explained as 'twin spot' mosaicism due to the postzygotic crossing-over of two homozygous recessive mutations, has recently been shown to derive from one postzygotic activating RAS mutation. Epidermal nevi, including those in PPK, are known to give rise to neoplasms such as trichoblastoma and basal cell carcinoma. Read More

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Speckled lentiginous nevus syndrome with median nerve paresis: A rare syndrome with a new association.

Indian Dermatol Online J 2013 Jul;4(3):234-5

Department of Dermatology and STD, Lady Hardinge Medical College, Shaheed Bhagat Singh Marg, New Delhi, India.

Speckled lentiginous nevus (SLN) represents a mosaic phenotype which consists of café au lait macule superimposed by melanocytic nevi. Recently, SLN syndrome has been characterized where ipsilateral neurological abnormalities have been reported in association with SLN with papular type of melanocytic nevi only. This case describes the presence of ipsilateral thenar muscle atrophy with median nerve paresis in nevus spilus which had melanocytic nevi of the macular type alone, thus delineating a new association in SLN syndrome, hitherto unreported. Read More

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Congenital melanocytic nevus admixed with speckled lentiginous nevus.

Indian J Dermatol 2013 Mar;58(2):161

Department of Dermatology, School of Medicine, Chungnam National University, Daejeon, Korea. E-mail:

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Phacomatosis pigmentokeratotica is caused by a postzygotic HRAS mutation in a multipotent progenitor cell.

J Invest Dermatol 2013 Aug 21;133(8):1998-2003. Epub 2013 Jan 21.

Department of Dermatology, University of Regensburg, Regensburg, Germany.

Phacomatosis pigmentokeratotica (PPK) is a rare epidermal nevus syndrome characterized by the co-occurrence of a sebaceous nevus and a speckled lentiginous nevus. The coexistence of an epidermal and a melanocytic nevus has been explained by two homozygous recessive mutations, according to the twin spot hypothesis, of which PPK has become a putative paradigm in humans. However, the underlying gene mutations remained unknown. Read More

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Dermacase. Can you identify this condition? Speckled lentiginous nevus.

Can Fam Physician 2012 Dec;58(12):1361, 1364-5

University of British Columbia, Vancouver, Canada.

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December 2012

Phacomatosis pigmentokeratotica without extracutaneous abnormalities: a case study involving a preterm baby.

J Korean Med Sci 2012 Nov 30;27(11):1444-6. Epub 2012 Oct 30.

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

Phacomatosis pigmentokeratotica (PPK) is a rare syndrome defined by the association of an organoid nevus occasionally with sebaceous differentiation, a speckled lentiginous nevus, and other extracutaneous anomalies. A preterm male infant of only 830 g at 27 week gestational age had an organoid nevus showing sebaceous differentiation. Also, he had multiple speckled-lentiginous nevus. Read More

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November 2012