Search

O nevo spilus (NS), também chamado de nevo lentiginoso mosqueado ou nevus sobre nevus, é representado por mancha acastanhada sobre a qual surgem pequenas máculas de tons mais escuros. É mais comum no tronco e em membros inferiores. A prevalência na população geral é de 0,2% a 2,3% e tem caráter benigno. O risco exato para transformação maligna ainda é desconhecido, por isso demanda seguimento clínico-dermatoscópico rotineiro. Apresentamos um caso de melanoma maligno sobre nevo spilus adquirido, no qual foi realizada exérese precoce, sem recidiva, ressaltando a importância do acompanhamento desses pacientes.

Phacomatosis pigmentokeratotica (PPK) is characterized by the co-occurrence of speckled lentiginous nevus (nevus spilus) and an organoid nevus with or without extracutaneous involvement. The extracutaneous manifestations may vary widely with musculoskeletal, neurologic, ocular, and vascular findings. The PPK is also associated with an increased risk of cutaneous or extracutaneous tumors. Therefore, the patients with PPK should be followed up regularly for possible malignant transformation. Here, we report a 5-year-old boy with PPK associated with toe walking due to short Achilles tendon, which was not previously reported, to our knowledge.

Speckled lentiginous nevus syndrome is a rare neurocutaneous phenotype, characterized by a specked lentiginous nevus, appearing as a light brown macule superimposed by multiple melanocytic nevi in the form of macules or papules with uneven distribution, associated with ipsilateral neurological and musculoskeletal abnormalities (i.e., dysesthesia, hyperhidrosis, and musculoskeletal abnormalities) occur. It represents a mosaic phenotype disorder resulting from postzygotic loss of heterozygosity, due to a localized defect in neural crest melanoblasts that originate certain areas of the skin and some ipsilateral neurological and musculoskeletal cells. Environmental and genetic factors may also play a role, but it has not been clarified how these factors behave in this syndrome.

Phacomatosis pigmentokeratotica (PPK) is a rare variant of epidermal nevus syndrome characterized by the combination of a nevus sebaceus and papular speckled lentiginous nevus, usually with associated neurologic, musculoskeletal, and ophthalmologic abnormalities. The nevus sebaceus is arranged along the lines of Blaschko, and the papular speckled lentiginous nevus is typically arranged in a checkerboard pattern. We present a clinically extensive case of cutaneous PPK in a young boy without evidence of extracutaneous abnormalities after more than a decade of follow-up.

Speckled lentiginous nevus syndrome is a recently described condition, characterized by unilateral neurocutaneous manifestations consisting of: speckled lentiginous nevus, hyperhidrosis, muscular weakness and dysesthesia. Skeletal anomalies such as scoliosis, limb asymmetry, and psychiatric manifestations have been also reported. Herein, we report a case of speckled lentiginous nevus syndrome with skeletal anomalies and a pseudo-marfanoid phenotype.

Key Clinical Message 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.

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.

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. A nevus spilus (speckled lentiginous nevus) is a clinical variant of congenital melanocytic nevus. The authors present the case of a 19-year-old male with an intraoral nevus spilus. The anterior mandibular gingiva exhibited multiple speckled, pigmented papules and macules on a thickened, hyperplastic macular background. Microscopic examination revealed characteristic morphologic features of intramucosal nevi extending into the deep portions of the submucosa. Although other authors have reported similar clinical presentation in the oral mucosa, no other case reports were found in the English literature classifying an intraoral congenital nevus as an intraoral nevus spilus. The sixth case of an intraoral congenital melanocytic nevus and the first case subclassified as an intraoral nevus spilus (speckled lentiginous nevus) is reported, with a review of the literature.

Phacomatosis pigmentokeratotica is a rare form of cutaneous mosaicism characterized by epidermal nevus usually nevus sebaceous, papular speckled lentiginous nevus, and extracutaneous anomalies. This type of twin-spot phenomenon (didymosis) is due to postzygotic crossing-over resulting in two homozygous daughter cells, representing the stem cells of the two distinct types of nevi and leading to additional extracutaneous defects. We report a case of this rare syndrome in a 4-year-old male child associated with skeletal anomalies.

Background: Atypical 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

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.

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.

Dermoscopy is a noninvasive technique that allows for the visualization of subsurface colors and structures within pigmented melanocytic skin lesions that are otherwise imperceptible to the naked eye. Dermoscopic findings have been formulated into diagnostic criteria that assist experienced clinicians in differentiating benign and malignant neoplasms. In essence, dermoscopy can help identify cutaneous malignancies, while at the same time minimizing the frequency of unwarranted biopsies of benign cutaneous lesions. In this article, we examine the clinical morphology of melanocytic nevi and melanoma in the pediatric population, and describe the relevant dermoscopic findings and histopathologic correlates that aid in the diagnosis and management of these lesions.

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.

Cutaneous mosaicism may be encountered with different patterns. Type II mosaicism - checkerboard pattern is characterized by flag-like areas with a sharp midline separation. The speckled lentiginous nevus (BLN) and Becker nevus are clinical examples of this pattern. A 38-year-old woman attended the outpatient clinic with a complaint of spots located over the left upper part of her body. These spots had been present from about eight years of age and showed a progressive course. Her mother also had a history of similar lesions. On dermatologic examination a diffuse, flag-like patch including brown macules was present over the left upper anterior trunk and left upper extremity. In this case report a case having BLN localized in type II mozaisizm- checkerboard pattern, which has not been reported previously in the Turkish literature, was presented.

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).

Lentigines are usually flat, pigmented macules on the skin and mucosa. These lesions are characterized by mostly smaller than 0.5 cm, irregular borders and different shades of brown and black. They can be in the form of circumscribed or widespread lesions, and occur either as isolated lesion or as a part of some congenital syndromes. The speckled lentiginous nevus is also named as Nevus Spilus which is a peculiar acquired lentiginous nevus, which can be divided into two distinct clinicopathological variants as macular and papular. On the other hand, centrofacial lentiginosus is a special form of congenital lentiginosus which is inherited as autosomal dominant, and is usually associated with some neurological and psychiatric findings. We report a 43-year old woman with a giant maculo-papular speckled lentiginous nevus associated with a different and non-syndromal isolated centrofacial lentiginosus. To the best of our knowledge this association has not been reported previously.

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. Methods: A review of case reports on SLN published during the years 1970–2004 was performed. Results: This evaluation of cases provided evidence that two different types of SLN exist, in the form of macular versus papular SLN, each one being related to a specific syndrome. Macular SLN is characterized by a tannish-brown background with darker flat speckles. The distribution of speckles is rather even and resembles a polka-dot pattern. Histopathologically, this type of SLN is characterized by what has been called a ‘jentigo’ pattern in the darker speckles and by some nests of melanocytes at the dermoepidermal junction at the tips of the papillae, whereas the background pigmentation shows the microscopical features of a lentigo. Papular SLN is characterized by a light-brown macule superimposed by multiple melanocytic nevi in the form of papules or nodules that show a more uneven distribution reminiscent of a star map. Small dark macules may likewise be present. Histopathologically, the papular component consists of dermal or compound melanocytic nevi. A separation of the two types of SLN is important because our analysis showed that macular SLN is a hallmark of a particular type of phacomatosis pigmentovascularis, whereas papular SLN is typically present in phacomatosis pigmentokeratotica as well as in SLN syndrome. Conclusions: Macular SLN and papular SLN appear to be two distinct cutaneous entities. This dichotomy may turn out to be important when such nevi will be analyzed at the molecular level.

Antecedentes : nevo melanocitico displasico es un diagnostico histopatologico habitual en la mayor parte de los laboratorios de Anatomia Patologica. Sin embargo, parece no existir un consenso general acerca de los criterios objetivos en los que se basa. Material y metodos : los autores han revisado y resumido con la mayor fidelidad posible las descripciones histopatologicas del nevo displasico en los dos trabajos princeps (de Clark et al y de Elder et al), en seis libros recientes de dermatopatologia y en dos libros monograficos sobre neoplasias melanociticas, todos ellos representativos de las diversas opiniones sobre el tema. Resultado :parece haber unanimidad en que el nevo displasico compuesto es un nevo melanocitico compuesto o intradermico rodeado por un «hombro» juntural; en ese «hombro » los nidos son de forma y tamano variables y de distribucion desordenada; sus celulas tienen amplio citoplasma con melanina pulverulenta; la dermis superficial subyacente muestra fibroplasia, infiltrados linfohistiocitarios y neoangiogenesis. Un nevo displasico juntural es un «hombro sin cabeza». El nevo displasico es una lesion benigna. Las discrepancias se plantean en torno a la displasia melanocitica lentiginosa y a la atipia celular. Conclusiones : los autores consideran que el nevo displasico tal como lo describieron Clark et al debe llamarse nevo de Clark; la variante que describieron Elder et al es un nevo lentiginoso. Otros tipos de nevo melanocitico juntural menos frecuentes son el que los autores llaman tipo clasico (con nidos junturales grandes y redondos) y las fases junturales de los nevos de Reed y de Spitz. El nevo de Clark y el nevo lentiginoso son muy frecuentes y no parecen tener mayor propension a la transformacion maligna que los otros tres tipos. La mayor parte de los melanomas aparecidos en pacientes con nevos displasicos surgen de novo, y, a veces, en relacion, logicamente, con los tipos de nevo juntural y nevo compuesto mas frecuentes: el nevo de Clark y el nevo lentiginoso.

Partial unilateral lentiginosis (PUL) is an unusual pigmentary disorder characterized by numerous lentigines grouped within an area of normal skin; the pigmented macules are often in a segmental distribution with a sharp demarcation at the midline. We report the first case of ocular involvement in a patient with this diagnosis. The patient, a 30-year-old Peruvian woman, had multiple brown macules on the left upper face in primarily a V1 and V2 distribution with a sharp demarcation at the midline of the forehead. The lesions first appeared near the hairline when she was 5 years of age, and then began to extend onto the face. She also had a discrete area of brown pigmentation on the left lateral bulbar conjunctiva. Because the patient had been previously diagnosed by several dermatologists as having either a speckled lentiginous nevus or a nevus of Ota, we draw attention to the entity PUL and the possibility of ocular involvement.

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.

We report a case of a congenital nevus sebaceus with numerous brown macules and papules in the left auricular region. In the same lesion, pigmented basal cell carcinomas developed later in life. Additionally, on the left side of the neck, both shoulders and the left arm, features of nevus spilus with melanocytic and blue nevi were present. Such a combination of nevus sebaceus with melanocytic lesions may be explained by the concept of twin spotting and represent another case of phacomatosis pigmentokeratotica.

In non-Caucasians, malignant melanoma most frequently affects the sole of the foot. To improve the prognosis in such patients, accurate diagnosis of early lesions is extremely important, and, to avoid potentially mutilating surgery, it is equally important to identify benign acral nevus. Clemente and colleagues recently proposed a new clinicopathological entity, designated acral lentiginous nevus (ALN) of the plantar skin. The clinical and histopathological characteristics of these nevi enable clinicians to distinguish them from ordinary nevi and melanoma. We report four additional cases of ALN, which can be classified as belonging to the "pseudomelanoma" group.

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.

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. The clinicopathologic features of these patients are the subject of this study. Results Nine patients with a total of 38 SN were identified retrospectively during a 20-year period. Patients ranged in age from 2 to 34 years (mean 22 years). The total number of histologically confirmed SN in each patient ranged from 2 to 13 (mean 5; median 2). These SN were more commonly diagnosed clinically as atypical nevi rather than as SN. The histopathologic findings ranged from those of a classic SN to an atypical Spitzoid neoplasm that was difficult to distinguish from a Spitzoid melanoma. Of the SN examined histologically in this series, the majority (53%) showed no atypical histopathologic features and none had recurred after a re-excision. Limitations The low number of patients with multiple SN is a limitation. Conclusion The presence of multiple SN is a rare phenomenon. The majority display histopathologic characteristics of classic SN or a few atypical features.

A peculiar case of "nevus on nevus" was reported. A 67-year-old man had had a pigmented lesion in the left hypochondrial area since birth. The clinicopathologic findings of the pigmented lesion revealed a combination of speckled lentiginous nevus and patch-type blue nevus. This case of "nevus on nevus" is not described under the term of combined nevus as is current in the literature; it was considered to be a subtype of the type II atypical blue nevus described by Kawamura.

We report a case of segmental lentiginosis (unilateral lentiginosis), that is, asymmetric distribution of lentigines on one side of the body, in a 23-year-old woman. Lesions involved the right side of the face and the cervical region, mostly within the area of division of the trigeminal nerve. Histologic examination disclosed a lentiginous pattern as well as some nests of melanocytes at the dermal-epidermal junction (so-called jentigo pattern). Similar cases have been described in the literature under the term "zosteriform lentiginous nevus," which in our opinion makes for confusion since the same term has also been used to describe cases that fit the diagnostic criteria for speckled lentiginous nevus (nevus spilus).

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.

Speckled lentiginous nevus (SLN) represents a mosaic phenotype which consists of cafe 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.

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. This case can be categorized as a typical example of speckled lentiginous nevus syndrome, a recently recognized phenotype characterized by a speckled lentiginous nevus of the papular type and ipsilateral neurological abnormalities in the form of dysesthesia, muscular weakness or hyperhidrosis. Speckled lentiginous nevus syndrome represents a mosaic phenotype. Most likely it originates from loss of heterozygosity occurring in a heterozygous embryo at an early developmental stage.

We define nevus spilus as a medium-sized, or large, cafe au lait macule speckled with melanocytic nevi. The definition is incorrect and imprecise but it is very entrenched in the common dermatological jargon and it is less cumbersome than others which are nosologically more appropriate. The French literature refers to this entity as “nevus en nevus”.

We report a case of speckled lentiginous nevus with extensive unilateral distribution in a 53-year-old man. The lesions involved the left back, extending from the midline anteriorly to the thorax, arm, and forearm. The background pigmentary patch covered only the back and upper arm. Histologic examination revealed a lentiginous pattern as well as a congenital nevocytic pattern, even in the areas where the background was nontanned skin. This combined clinical pattern in the same patient fits the descriptions for both the usual and unusual speckled lentiginous nevus. Cases with variants of extensive unilateral distribution are infrequent and pathologists should pay particular attention to them because of the possibility of their malignant transformation.

We describe an unusual case of blue nevi and basal cell carcinoma arising within a speckled lentiginous nevus and discuss the relationship between epithelial tumors and nevoid growth of melanocytes.

We describe a 10-year-old girl affected with a speckled lentiginous nevus and an epidermal nevus of the organoid type on corresponding parts of the body. On histopathological examination, the lesions showed epidermal hyperpigmentation and melanocytic hyperplasia on the one hand and verrucous epidermal acanthosis with sebaceous hyperplasia on the other hand. Except for a minor deviation of the spine, the patient had no obvious extracutaneous symptoms. Happle et al. have recently interpreted the rare co-occurrence of these two types of nevi in spatial proximity as an example of twin spotting in human skin and proposed the name 'phacomatosis pigmentokeratotica'. In most cases, additional skeletal or neurological anomalies are found. These are dissimilar from the extracutaneous symptoms of the sebaceous nevus syndrome, from which phacomatosis pigmentokeratotica should be distinguished. Molecular studies are needed to prove the concept of twin spotting and to reveal a link to the extracutaneous manifestations.

Agminate and plaque-type blue nevi are rarely described. We report the occurrence of such a type of blue nevus associated with eccrine changes and a follicular cyst all arising on a macular brown background in a 38-year-old man. The patient presented numerous blue papules and a plaque, overlapping a light tan patch present since birth, on his left thigh. In addition, within the plaque, 3 papules, discharging at intervals a serous fluid, were present. Since the lesion hardened and enlarged, it was surgically excised. Histologic findings revealed a lentigo pattern of the epidermis, corresponding to the light tan macular background and a plaque-type blue nevus, and areas of eccrine ductal proliferations with a ruptured follicular cyst. The association of agminate and plaque-type blue nevus, arising on a light brown patch of lentigo, might represent a variant of speckled lentiginous nevus. Eccrine proliferations may be reactive in nature or represent a more complex hamartomatous lesion. The possibility of malignant transformation and the recent enlargement of the lesion caused by the ruptured follicular cyst convinced us to carry out a wide surgical excision.

From questions on page 171 . The answer is (B), nevus spilus. This lesion is characteristic of a nevus spilus, also known as speckled lentiginous nevus. Typically, nevus spilus presents as a tan-brown patch with superimposed brown nevi that develop over time. It is found in 1.7/1000 newborns, with boys and girls being equally affected. Parents often give a history of noticing the macular component at birth or shortly thereafter, with the gradual appearance of more and more individual nevi within the area. This lesion is …