89 results match your criteria Speckled Lentiginous Nevus
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
J Dermatol 2018 Feb 21. Epub 2018 Feb 21.
Department of Plastic and Reconstructive Surgery, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan.
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
Indian J Dermatol Venereol Leprol 2017 Sep-Oct;83(5):612-614
Department of Dermatology, Katihar Medical College and Hospital, Katihar, Bihar, India.
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
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
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
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
Int J Dermatol 2016 Nov;55(11):e602-e604
Department of Dermatology, Hospital of the University of Brasilia, Brasilia, Brazil.
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
J Dermatol 2015 Apr 21;42(4):433-4. Epub 2015 Feb 21.
Department of Dermatology, Peking University People's Hospital, Beijing, China.
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.
Int J Dermatol 2014 May 14;53(5):619-21. Epub 2014 Feb 14.
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
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
Indian J Dermatol 2013 Mar;58(2):161
Department of Dermatology, School of Medicine, Chungnam National University, Daejeon, Korea. E-mail:
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
Can Fam Physician 2012 Dec;58(12):1361, 1364-5
University of British Columbia, Vancouver, Canada.
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
Pediatr Dermatol 2012 Jul-Aug;29(4):546-7; author reply 546
J Am Acad Dermatol 2012 Sep 31;67(3):451-8, 458.e1-2. Epub 2012 Jan 31.
Department of Dermatology, Yale University School of Medicine, New Haven, Connecticut, USA.
Background: Whether agminated, within a speckled lentiginous nevus/nevus spilus, or randomly scattered, multiple Spitz nevi (SN), defined as two or more SN, is a rare entity.
Objective: The aim of this study was to evaluate patients presenting with multiple SN.
Methods: We conducted a search of the database of the Yale Spitzoid Neoplasm Repository from May 1990 to June 2010 to identify patients with multiple SN. Read More
Pediatr Dermatol 2013 Jan-Feb;30(1):100-4. Epub 2012 Feb 3.
Department of Dermatology and Venereology, All India Institute of Medical Sciences, New Delhi, India.
Nevus spilus, also known as speckled lentiginous nevus, is a nevoid disorder characterized by hyperpigmented macules or papules scattered over a background of tan pigmentation. Although nevus spilus is mainly of cosmetic concern, malignant melanoma may rarely develop in the lesions. Although classically not mentioned as a hairy nevus, a few reports in literature mention overlying hypertrichosis in lesions of nevus spilus. Read More
Pediatr Dermatol 2011 Nov-Dec;28(6):749
Pediatr Dermatol 2011 Nov-Dec;28(6):715-9
Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
Epidermal nevus syndrome is the term for the association of an epidermal nevus and extracutaneous anomalies, including neurologic, ophthalmic, and skeletal defects. Epidermal nevus syndromes include different disorders that share the feature of mosaicism. Phacomatosis pigmentokeratotica (PPK) is a distinctive new epidermal nevus syndrome first described in 1996 characterized by the presence of multiple organoid nevi with sebaceous differentiation, a speckled lentiginous nevus, and skeletal and neurologic abnormalities. Read More
Neurol Sci 2012 Apr 2;33(2):477; author reply 479. Epub 2011 Nov 2.
J Am Acad Dermatol 2011 Jun;64(6):1190-3
Pediatr Dermatol 2010 Nov-Dec;27(6):654-6
Department of Dermatology, Hospital Clínico Universitario, Avenida Blasco Ibañez 17, 46010, Valencia, Spain.
Usually speckled lentiginous nevus or nevus spilus is a small solitary lesion consisting of a light tan patch with numerous dark brown macules or papules (or both) within it. It is occasionally associated with complex birth defects such as phacomatosis pigmentovascularis, phacomatosis pigmentokeratotica, or speckled lentiginous nevus syndrome. Uncommon presentations include large segmental lesions that may or may not be systematized and can sometimes be associated with other anomalies. Read More
Pediatr Dermatol 2011 Sep-Oct;28(5):561-7. Epub 2011 Mar 3.
Section of Dermatology, Dartmouth Hitchcock Medical Center, Lebanon, New Hampshire 03756, USA.
Spitz nevi are small dome-shaped nodules that sometimes arise in areas of preexisting hyperpigmentation, such as a speckled lentiginous nevus (nevus spilus), where they present a diagnostic dilemma. We report clinical, histopathological, and molecular findings of two cases of multiple Spitz nevi arising in a speckled lentiginous nevus. We used immunohistochemistry to assess expression of Ki-67, epidermal growth factor receptor, vascular endothelial growth factor, and RelA in two cases of Spitz nevi arising in a speckled lentiginous nevus. Read More
Dermatology 2010 11;221(4):324-5. Epub 2010 Nov 11.
Neurol Sci 2010 Dec 15;31(6):841-4. Epub 2010 Sep 15.
Cerebrovascular Diseases Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.
Phakomatosis refers to several malformation syndromes with simultaneous involvement of the skin, the eye, and the central nervous system by developmental lesions. Speckled lentiginous nevus (SLN), a subtype of congenital melanocytic nevi, is usually an isolate, harmless finding. Here, we report the case of a 52-year-old woman with congenital left laterocervical SLN associated with an ipsilateral intracranial extra-axial cavernous angioma, a yet not described association to date. Read More
Clin Dermatol 2010 May-Jun;28(3):293-302
The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, 560 1st Ave, New York, NY 10016, USA.
Congenital melanocytic nevi (CMN) are evident in 1% to 6% of neonates. In some studies, nevi with clinical, dermatoscopic, and histologic features identical to CMN have had a prevalence of more than 15% in older children and adults, possibly reflecting the "tardive" appearance of nevi programmed from birth. There is ongoing debate about the magnitude of the risk of melanoma and other complications associated with CMN of various sizes and the best approach to management of these lesions. Read More
Arch Dermatol 2010 Feb;146(2):204
Pediatr Dermatol 2009 May-Jun;26(3):298-301
Indiana University School of Medicine, Indianapolis, IN 46202, USA.
Speckled lentiginous nevus syndrome is a recently described neurocutaneous disorder characterized by speckled lentiginous nevus in association with hyperhidrosis, skin dysesthesia, and muscular weakness in an ipsilateral manner. We report a 9-year-old boy with a large speckled lentiginous nevus on his trunk, bilateral upper extremities, and neck associated with hyperhidrosis and nonspecific pain in the distribution of the nevus in addition to prominent upper extremity asymmetry and scoliosis. Similar musculoskeletal abnormalities have been reported in patients with phacomatosis pigmentovascularis and phacomatosis pigmentokeratotica which feature speckled lentiginous nevus; however, this patient demonstrates striking musculoskeletal abnormalities in the absence of coexisting vascular lesions or nevus sebaceous. Read More
Clin Exp Dermatol 2009 Mar 24;34(2):212-5. Epub 2008 Nov 24.
Department of Dermatology and Venerology, Georg August University, Goettingen, Goettingen, Germany.
We report a 14-year-old girl with a large speckled lentiginous naevus (SLN) on her left arm and shoulder. As the occurrence of melanoma within SLN has been described previously, long-term follow-up of atypical lesions by digital dermoscopy was started at the age of 4 years. To date, nine Spitz naevi and four dysplastic compound naevi have been excised due to dynamic changes over time. Read More
Clin Exp Dermatol 2009 Mar 24;34(2):133-5. Epub 2008 Nov 24.
Department of Dermatology, Philipp University of Marburg, Marburg, Germany.
Speckled lentiginous naevus (synonym: naevus spilus) no longer represents one clinical entity, but rather, two different disorders can be distinguished. Naevus spilus maculosus is consistently found in phacomatosis spilorosea, whereas naevus spilus papulosus represents a hallmark of phacomatosis pigmentokeratotica. The macular type is characterized by dark speckles that are completely flat and rather evenly distributed on a light brown background, resembling a polka-dot pattern. Read More
Dermatology 2008 9;217(1):63-5. Epub 2008 Apr 9.
Department of Clinical Molecular Medicine, Division of Dermatology, Kobe University Graduate School of Medicine, Kobe, Japan.
We describe a case with numerous melanocytic nevi in otherwise normal skin. A 5-year-old girl presented with more than 100 small pigment lesions on her left arm, shoulder and upper back without underlying light brown macule. The pigment lesions were first found on her left forearm at 3 months old and gradually increased along with her growth. Read More
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
Cutis 2007 Dec;80(6):465-8
UMDNJ-New Jersey Medical School, Newark, New Jersey 07103, USA.
Nevus spilus (NS), also known as speckled lentiginous nevus (SLN), is a relatively common cutaneous lesion that is characterized by multiple pigmented macules or papules within a pigmented patch. It may be congenital or acquired; however, its etiology remains unknown. NS deserves its own place in the spectrum of classification of important melanocytic nevi; as a lentigo and melanocytic nevus, it has the slight potential to develop into melanoma. Read More
Int J Dermatol 2007 Sep;46(9):967-70
Division of Dermatology, Department of Medicine, King Fahad National Guard Hospital, King Abdulaziz Medical City, Riyadh, Kingdom of Saudi Arabia.
Neurofibromatosis type 1 is an autosomal dominant disease and is considered one of the most commonly inherited diseases in humans. Malignant melanoma has been reported in up to 5% of patients with neurofibromatosis type 1. We report a young Saudi boy with neurofibromatosis type 1 who developed fatal metastatic malignant melanoma arising from giant melanocytic nevi within speckled lentiginous nevus (SLN). Read More
J Cutan Med Surg 2006 Mar-Apr;10(2):85-91
Dermatology Service, Department of Medicine, Memorial Sloan-Kettering Cancer Center, New York, NY 10022, USA.
Background: The classification of many pigmented lesions is not well defined, and an association with melanoma has not been established.
Objective: To better define the spectrum of pigmented lesions (excluding common and dysplastic nevi) and explore links to melanoma.
Methods: Retrospective review of patients with total-body digital photographs at high risk of developing melanoma from the Memorial Sloan-Kettering Cancer Center Dermatology Service. Read More
Int J Dermatol 2006 Nov;45(11):1362-4
Izmir Atatürk Education and Research Hospital Dermatology, Surgery, Pathology, Department, Izmir, Turkey.
Cutaneous melanoma may develop de novo on normal skin or in contiguity with a potential melanocytic precursor. We report a 45-year-old man who presented with a recently developed nodule in a previously stable congenital nevus. Physical examination revealed a 10x18-cm lesion with speckled lentiginous pigmentation and terminal hairs on the lower back. Read More
J Am Acad Dermatol 2006 Aug;55(2 Suppl):S16-20
Ronald O. Perelman Department of Dermatology, New York University School of Medicine, NY 10016, USA.
Phacomatosis pigmentokeratotica (PPK) represents a specific "twin nevus" syndrome in which a speckled lentiginous nevus (SLN) is associated with an organoid nevus with sebaceous differentiation. A boy with a large nevus sebaceus on the left face and upper part of the trunk, a giant segmental SLN extending from the abdomen to the feet bilaterally, and right hemihypertrophy developed an embryonal rhabdomyosarcoma of the right abdominal wall at age 6 months. A variety of musculoskeletal, neurologic, and ocular anomalies have been observed in patients with PPK, reflecting the individual manifestations of both SLN and Schimmelpenning syndromes. Read More
Int J Dermatol 2006 Mar;45(3):314-5
Department of Dermatology, George Washington University, Washington, DC, USA.
A 41-year-old white female with a past medical history of hypothyroidism and alopecia universalis presented on January 24, 2002 with a recently changing mole. She indicated changes in size and color of the superior aspect of a mole that had been present for more than 8 years. She had approximately 20 lifetime peeling sunburns due to being a lifeguard. Read More
Dermatology 2006 ;212(1):53-8
Department of Dermatology, Philipp University of Marburg, Marburg, Germany.
Background: Speckled lentiginous nevus (SLN; synonym: nevus spilus) is a darkly spotted light-brown macule that mostly occurs as an isolated lesion of rather limited dimensions but sometimes may involve large areas of the body. So far, this skin disorder has been considered to represent one clinical entity.
Objective: Because SLN is occasionally associated with complex birth defects such as phacomatosis pigmentovascularis, phacomatosis pigmentokeratotica, or SLN syndrome, we tested our idea that two different types of SLN may exist, each one associated with a particular syndrome. Read More
Br J Dermatol 2005 May;152(5):1039-44
Dermatology Division, Memorial Sloan-Kettering Skin Cancer Center at Suffolk, 800 Veterans Memorial Highway, Hauppauge, NY 11788, USA.
We demonstrate the utility of the Wood's light in a practice that specializes in the evaluation of pigmented lesions. The Wood's light assisted the physician in locating the site of a completely regressed primary cutaneous melanoma, determining the clinical borders of a lentigo maligna melanoma, differentiating between agminated naevi and a naevus spilus and detecting the recurrence of pigmentation after the excision of a dysplastic naevus, and also proved useful in monitoring a large segmental speckled atypical lentiginous naevus for change. Despite the availability of many 'high-tech' imaging and diagnostic devices designed to evaluate skin lesions, the relatively simple Wood's lamp continues to be of great value. Read More
Pediatr Dermatol 2005 Jan-Feb;22(1):44-7
Service of Dermatology, Valencia University General Hospital, Valencia, Spain.
Phacomatosis pigmentokeratotica is a rare syndrome defined by the association of an organoid nevus occasionally with sebaceous differentiation, a speckled lentiginous nevus, and other extracutaneous anomalies. The disorder is a consequence of the so-called twin spot genetic mechanism. We describe the first occurrence involving malignant degeneration of both nevus components, giving rise to three basal cell carcinomas over the sebaceous nevus and a malignant melanoma of the superficial spreading type over the speckled lentiginous nevus. Read More
Dermatology 2004 ;209(4):321-4
Department of Dermatology, Chang Gung University, Chang Gung Memorial Hospital, Kaohsiung, Taiwan.
A 9-year-old Taiwanese girl had an unusual combination of multiple lentigines clustered on her left face, 3 cafe-au-lait spots on her left arm and ipsilateral axillary freckling. Two Lisch nodules of the iris were recognized in the left eye but none in the right eye, which is why we established a diagnosis of segmental neurofibromatosis 1. No family member had any sign of neurofibromatosis 1. Read More
Dermatology 2004 ;209(3):228-9
Department of Dermatology, Georg August University, Göttingen, Germany.
A 42-year-old man had a large speckled lentiginous nevus on the left side of his trunk. The involved area was painful when touched and paresthetic. Moreover, the ipsilateral half of his body showed a pronounced hyperhidrosis. Read More
Hautarzt 2004 Oct;55(10):960-1, 964-8
Klinik für Dermatologie und Allergologie, Klinikum der Philipps-Universität, Marburg.
The concept of cutaneous mosaicism has now been proven by numerous studies. In this way, the idea that all nevi represent mosaics has so far been confirmed. We can distinguish five different patterns of mosaicism in human skin in the form of lines of Blaschko, checkerboard pattern, phylloid pattern, patchy pattern without midline separation, and lateralization. Read More
Eur J Dermatol 2003 Nov-Dec;13(6):534-6
Department of Dermatology and Pathology, Hospital del Niño Jesús, Menéndez Pelayo 65, 28009-Madrid, Spain.
A 15-year-old boy had an unusual combination of giant congenital melanocytic nevus on his back and a large speckled lentiginous nevus arranged in a checkerboard pattern on the dorsal and lateral aspects of his trunk. The two pigmentary nevi showed distinguishing features both clinically and histopathologically. The speckled lentiginous nevus was not noted at birth but became visible during childhood. Read More