Lentiginous Nevus Research Articles

Multiple Agminated Acquired Melanocytic Nevi

Dear Editor: 'Agminated' derives from the Latin word 'agmen', meaning an aggregation, and indicates a clustering or circumscribed grouping of lesions localized to a body area. It should be differentiated from other types of segmental distribution without a definite clustering1. Herein, we describe an acquired case of multiple clusters of agminated melanocytic nevi. A 9-year-old girl visited our department with multiple clusters of pigmented moles on her trunk and inguinal area. They began to appear approximately 2 years ago and continuously increased in number. Upon physical examination, multiple circumscribed groups of dark brown colored macules lacking background pigmentation were noted on both sides of the flank and right inguinal area (Fig. 1). The patient was otherwise healthy and had no signs of developmental anomaly. A skin biopsy revealed discrete nests of nevus cells at the dermoepidermal junction, mostly located on the accentuated tips of rete ridges (Fig. 2). A markedly increased amount of melanin pigment was observed in the epidermis with melanophages in the superficial dermis. No nuclear atypia or mitosis was seen among the nevus cells. With the histological features of a junctional nevus, their unique clustered arrangement drew the diagnosis of agminated acquired melanocytic nevi. Three sessions of alexandrite laser treatment was performed with cosmetically acceptable results. Fig. 1 Multiple agminated melanocytic nevi were distributed on the left flank. Fig. 2 Discrete nests of nevus cells at the dermoepidermal junction, mostly located on the accentuated tips of rete ridges (H&E, ×100). Pigmented lesions that are known to occur as agminated include blue nevi, multiple lentigines, Spitz nevi, congenital melanocytic nevi, acquired melanocytic nevi, and nevus spilus. The main differential diagnosis of agminated nevi is nevus spilus without clinically visible background pigmentation2. Nevus spilus, also known as speckled lentiginous nevus, is considered within the spectrum of congenital melanocytic nevi3, due to its usual occurrence during late infancy or early childhood. Dark brown pigmented macules and papules lying on a tan lentiginous patch is the characteristic finding. Histologically, the background area resembles lentigo simplex, whereas the darker spots usually show the features of a lentiginous nevus with lentigo-like areas progressing to junctional and even small compound nevi4. In contrast, agminated nevi usually occur during puberty and lack background pigmentation. In our case, background pigmentation was not clinically visible, and the small amount of normal tissue surrounding the excised nevus did not reveal a lentiginous feature. Since melanoma arising from acquired agminated melanocytic nevi has been described5 and the patient is relatively young, long-term follow-up for any malignant change as well as development of background pigmentation is warranted.

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

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.

Is Speckled Lentiginous Nevus Really Prone to Dysplasia/Neoplasia?

Pediatric DermatologyVolume 29, Issue 4 p. 546-547 Correspondence Is Speckled Lentiginous Nevus Really Prone to Dysplasia/Neoplasia? Daniele Torchia M.D., Daniele Torchia M.D. Department of Dermatology and Cutaneous Surgery, Miller School of Medicine, University of Miami, Miami, Florida e-mail: [email protected]Search for more papers by this authorLawrence A. Schachner M.D., Lawrence A. Schachner M.D. Department of Dermatology and Cutaneous Surgery, Miller School of Medicine, University of Miami, Miami, Florida e-mail: [email protected]Search for more papers by this author Daniele Torchia M.D., Daniele Torchia M.D. Department of Dermatology and Cutaneous Surgery, Miller School of Medicine, University of Miami, Miami, Florida e-mail: [email protected]Search for more papers by this authorLawrence A. Schachner M.D., Lawrence A. Schachner M.D. Department of Dermatology and Cutaneous Surgery, Miller School of Medicine, University of Miami, Miami, Florida e-mail: [email protected]Search for more papers by this author First published: 03 July 2012 https://doi.org/10.1111/j.1525-1470.2011.01621.xCitations: 1Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL No abstract is available for this article.Citing Literature Volume29, Issue4July/August 2012Pages 546-547 RelatedInformation

Response to Comments on “Spitz Nevi Arising in Speckled Lentiginous Nevus: Clinical, Histologic, and Molecular Evaluation of Two Cases”

Letters to the Editor are welcomed for publication (subject to editing). Letters must be signed by all authors, and must not exceed two pages of text including references. Letters should not duplicate material submitted or published in other journals. Prepublication proofs will not be provided.

Atypical Lentiginous Nevus: A Clinical and Histopathologic Study of 14 Cases

BackgroundAtypical lentiginous nevus (of the elderly) is a peculiar form of dysplastic nevus. Clinically, this condition can resemble malignant melanoma and histologically, it has a lentiginous pattern with variable degrees of atypia and an absence of dermal nests. These features may lead to an erroneous diagnosis of lentigo maligna melanoma or lentiginous melanoma. Material and methodsWe reviewed 14 cases of atypical lentiginous nevus diagnosed at the dermatology department of Hospital General de Valencia in Valencia, Spain between December 2007 and March 2009. We studied the clinical and histopathologic features of the lesions after hematoxylin-eosin, Melan-A, and Ki-67 staining and compared our results to data reported in the literature. ResultsFour (28%) of the 14 patients (7 men, 7 women) were under 50 years of age. Clinically, most of the lesions (8/14) resembled atypical nevi and they were all located on the back. Histologically, they all had irregular lentiginous epidermal hyperplasia, with a proliferation of individual melanocytes only in the basal layer of the epidermis and an absence of dermal nests. Focal upward migration of melanocytes into the epidermis was present in just 4 cases. All the lesions had cellular atypia, which was moderate in 85% of cases. The Ki-67 proliferation index was low (<5%) in all the lesions analyzed. ConclusionsAtypical lentiginous nevi, which can be classified as atypical pigmented lesions with a lentiginous pattern, may clinically and histologically resemble melanoma. Our findings support earlier reports that both clinical and histologic findings may suggest a diagnosis of dysplastic nevus. All of the patients in our series are healthy and free of recurrence after 18 months or longer.

Nevus lentiginoso atípico. Estudio clínico-patológico de 14 casos

BackgroundAtypical lentiginous nevus (of the elderly) is a peculiar form of dysplastic nevus. Clinically, this condition can resemble malignant melanoma and histologically, it has a lentiginous pattern with variable degrees of atypia and an absence of dermal nests. These features may lead to an erroneous diagnosis of lentigo maligna melanoma or lentiginous melanoma. Material and methodsWe reviewed 14 cases of atypical lentiginous nevus diagnosed at the dermatology department of Hospital General de Valencia in Valencia, Spain between December 2007 and March 2009. We studied the clinical and histopathologic features of the lesions after hematoxylin-eosin, Melan-A, and Ki-67 staining and compared our results to data reported in the literature. ResultsFour (28%) of the 14 patients (7 men, 7 women) were under 50 years of age. Clinically, most of the lesions (8/14) resembled atypical nevi and they were all located on the back. Histologically, they all had irregular lentiginous epidermal hyperplasia, with a proliferation of individual melanocytes only in the basal layer of the epidermis and an absence of dermal nests. Focal upward migration of melanocytes into the epidermis was present in just 4 cases. All the lesions had cellular atypia, which was moderate in 85% of cases. The Ki-67 proliferation index was low (<5%) in all the lesions analyzed. ConclusionsAtypical lentiginous nevi, which can be classified as atypical pigmented lesions with a lentiginous pattern, may clinically and histologically resemble melanoma. Our findings support earlier reports that both clinical and histologic findings may suggest a diagnosis of dysplastic nevus. All of the patients in our series are healthy and free of recurrence after 18 months or longer.

Hairy Nevus Spilus: A Case Series

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. We hereby report four cases of nevus spilus without malignant change with overlying terminal hairs that arose mainly from the background pigmented area.

Phacomatosis Pigmentokeratotica without Extracutaneous Abnormalities: A Case Study Involving a Preterm Baby

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. Correlating the observed clinical presentation with the histopathological findings, the diagnosis of PPK was established. There have been less than 10 cases of PPK without extracutaneous manifestation. We present an uncommon case of a preterm patient with PPK who had no extracutaneous abnormalities.

Speckled lentiginous nevus: sometimes, but not always, part of a syndrome

We completely agree with the objections raised by Doctors Torchia and Schachner (doi:10.1007/s10072-011-0736-8). Indeed, the issue we intended to raise with our report was: Do undescribed non-random associations of SLN-internal vascular malformations exist? We widely discussed the possibility that the association was completely accidental in our patient. However, here, we move on a quite unexplored ground. Accordingly, we forwarded our cutting-edge provocation to provide clinicians with PPV/SLN patients or the alike with some written, even low-grade evidence to propose whenever they wish to study these occasional patients in depth. ‘‘Occasional’’, because if these patients were thousands, the issue would have already been worked out—what are you going to do with your next systematized SLN patient without evident clinical neurological involvement (with special regard to expensive NMR)?

Speckled Lentiginous Nevus Syndrome: Central Nervous System Abnormalities as a Critical Diagnostic Feature

Speckled Lentiginous Nevus Syndrome: Central Nervous System Abnormalities as a Critical Diagnostic Feature

Phacomatosis Pigmentokeratotica: A Further Case without Extracutaneous Anomalies and Review of the Condition

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. Only a handful of cases of PPK without extracutaneous manifestations have been reported. We report here an individual with PPK with only cutaneous signs and confirm this distinctive syndrome has two subtypes according to the presence or absence of extracutaneous involvement.

Speckled lentiginous nevus: sometimes, but not always, part of a syndrome

Speckled lentiginous nevus: sometimes, but not always, part of a syndrome

Melanoma(s) arising in large segmental speckled lentiginous nevi: A case series

Melanoma(s) arising in large segmental speckled lentiginous nevi: A case series

Spitz Nevi Arising in Speckled Lentiginous Nevus: Clinical, Histologic, and Molecular Evaluation of Two Cases

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. We observed rare staining for the proliferative marker Ki-67, but positive staining for the growth and antiapoptotic factors epidermal growth factor receptor, vascular endothelial growth factor, and RelA. Characterization of the molecular phenotype of Spitz nevi arising in speckled lentiginous nevi may provide a useful adjunct to long-term monitoring in this rare but difficult clinical presentation.

Lentiginous Melanoma

Atypical lentiginous melanocytic lesions, particularly in older individuals, continue to pose a diagnostic challenge. Such lesions often show features intermediate between lentiginous nevus and melanoma in situ. We have recently defined within this group of lesions a histologic pattern of lentiginous melanoma, a slowly progressing variant of melanoma typically found on the trunk and proximal extremities of middle-aged and older individuals. To review the clinical and histologic features of lentiginous melanoma and its histologic differential diagnosis. Review of pertinent published literature and work in our laboratory. Lentiginous melanoma defines a subset of slowly progressing melanoma occurring in middle-aged and older patients. It is histologically characterized by a broad atypical lentiginous growth pattern of moderately atypical melanocytes showing focal nesting and pagetoid spread without significant dermal fibroplasia or alteration of the rete ridges. Lentiginous melanoma shows significant overlap in clinical and histologic features with atypical lentiginous nevus (of the elderly). Relationship between these entities requires further investigations. Given the risk of progression to invasive melanoma, all lesions showing features of lentiginous melanoma should be treated with adequately wide excision.

Melanocytic Lesions: Current State of Knowledge—Part II

Melanocytic Lesions: Current State of Knowledge—Part II

Widespread Nevus Spilus Associated with Torsion Dystonia: A Case Report

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. We hereby report a 6-year-old Caucasian patient with systematized nevus spilus associated with torsion dystonia, a combination not published thus far.

Intracranial cavernoma and speckled lentiginous nevus: extending the spectrum of phakomatoses?

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. After revision of the literature, we suggest that both these lesions could be correlated in the setting of an atypical, yet unclassifiable form of phakomatosis, such as phakomatosis pigmentovascularis or SLN syndrome. We also propose that patients with bizarre, geometrical, pigmented or vascular cervicocranial skin lesions should undergo a thorough neurologic and ophthalmologic evaluation.

Speckled Lentiginous Nevi: No Longer One Single Disorder

I read with interest the arguments presented by Barysch and Dummer1 on how to distinguish speckled lentiginous nevi from segmental lentiginosis. I agree that such criteria are important because these disorders are sometimes confused. However, I would like to make the following annotations.

The Spectrum of Widespread Hyperpigmentations From SLN to SUL

Single efflorescences of both speckled lentiginous nevus (SLN) and segmental unilateral lentiginosis (SUL) look similar and may be easily confused. Herein, we detail a case of a segmental SLN to draw attention to the distinction.