A Combination of Speckled Lentiginous Nevus with Patch-type Blue Nevus

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.     

Speckled lentiginous nevus

Speckled lentiginous nevus is, we feel, a distinctive nevocytic disorder and a clinical variety of nevus-cell nevus. The speckled areas show varying histological patterns that range from nevus incipiens to junctional and compound nevi. The background shows histological features of lentigo simplex. It is our contention that speckled lentiginous nevus should be separated from nevus spilus and nevus spilus tardus (Becker's), which we consider to be variants of epidermal nevus.     

Plaque-type blue nevus combined with lentigo (nevus spilus)

Three cases of plaque-type blue nevus accompanied with lentigo-like changes were reported. The combination of blue nevus and lentigo (nevus spilus) is a new type of "combined nevus", but it belongs to the second type of Kawamura's atypical blue nevus. This combination is unlikely to be rare, not only in plaque-type, but also in common type blue nevus, and to be comparable with the findings of such dermal melanocytosis as nevus of Ota. Histologically, cellular blue nevus was also observed in areas of Case 1.     

Woolly Hair Nevus with an Ipsilateral Associated Epidermal Nevus and Additional Findings of a White Sponge Nevus

Abstract:  We report a case of a 16-year-old male with a woolly hair nevus, an associated ipsilateral epidermal nevus who also had a white plaque on his tongue, clinically diagnosed as a white sponge nevus. The concurrent findings of a white sponge nevus, a woolly hair nevus, and an ipsilateral epidermal nevus, to our knowledge, have not been previously reported.     

Eponyms, morphology, and pathogenesis of some less mentioned types of melanocytic nevi

Some types of melanocytic nevi are seldom mentioned in the literature and are therefore less well known. In the current study, we focus on the concepts of eponyms, synonyms, clinical presentation, and morphology of the following types: Meyerson nevus, cockarde (or cockade) nevus, Hori nevus, Sun nevus, Hidanos nevus, Duperrat nevus, Spark nevus, nevus spilus, eclipse nevus, Kerl nevus, and Kopf nevus.     

Angiomatoid Spitz Nevus

Spitz nevus is a variant of melanocytic nevus which is histopathologically defined as large spindle and/or epithelioid cells. Angiomatoid Spitz nevus is a rare histologic variant of desmoplastic Spitz nevus characterized by prominent vasculature. We present a case of angiomatoid Spitz nevus, celluar type, that has not been reported before. We provide another example to show the remarkable diversity of Spitz nevus.     

Hypopigmented Reed nevus

Reed nevus, also named pigmented spindle cell nevus, is a peculiar melanocytic nevus, now regarded as a variant of Spitz nevus by the majority of authors. It is characterized by spindle-shaped melanocytes disposed in nests located in epidermis and papillary dermis. It is usually heavily pigmented, and many melanophages may also be present. Hypopigmented Reed nevus shows all the typical features of conventional pigmented spindle cell nevus, but it does not contain abundant melanin. This variant of Reed nevus is poorly described in literature, so we report five cases of hypopigmented Reed nevus and discuss its clinical and histopathological features.     

Nevus cells observed in the sebaceous glands

Of sebaceous gland-containing specimens of melanocytic nevi, a total of 79 specimens from 70 cases of nevocellular nevus (consisting of 10 cases of compound nevus and 60 of intradermal nevus), four cases of juvenile melanoma and three of blue nevus were examined light-microscopically in detail for the presence or absence of nevus cells or dermal melanocytes in the sebaceous glands. As a result, clusters of cells that appeared to be nevus cells were found in the sebaceous glands of specimens from a total of three cases, that is, two cases of nevocellular nevus i.e. compound nevus, and one of the cases of juvenile melanoma. Two cases were infantile and one case was the age of puberty. The origin of the nevus cells found in the sebaceous glands is discussed.     

晕痣和痣细胞痣bcl-2蛋白的表达

为了研究细胞凋亡在晕痣发生中的作用,我们对8例晕痣和8例痣细胞痣患者皮损进行了bcl-2原癌基因产物(B-cell lymphoma/leukemia 2 gene product,以下简称bcl-2)的免疫组化观察.结果表明8例晕痣均见有bcl-2的阳性表达,8例痣细胞痣仅有3例表达bcl-2,而且染色明显较弱,大部分组织呈阴性表达.由于两种痣bcl-2表达明显不同,作者认为晕痣组织内bcl-2的过量表达与晕痣发生的自身免疫机制有关,可能是痣组织内对痣细胞的高凋亡率的一种自动平衡反应.     

Developmental aspects of nevus spilus: review of the literature apropos of 7 cases

The nevus on nevus is a dischromic lesion with a double component made of a pigmented, pale-brown coloured spot, most often congenital punctuated by macular or discretely papular darker elements, with a usually later setting-up and conventionally characterized by an absence of evolutivity. A series of seven cases is reported of whom three present a very peculiar evolution: A 37 years old man is taking a medical advice for a nevus on a congenital nevus on the right buttock on which appeared later on a blue-coloured, lightly sensitive nodule which clinically calls to mind the diagnosis of a blue nevus: the surgical exeresis is refused by the patient. A little girl, born in 1972, has since her birth a nevus on nevus of her right fore-arm; in 1975 and 1976 appeared successively on this lesion three nodules evocative of Spitz melanoma (fig. 4), which are surgically removed; histologic examination confirms the clinical diagnosis (fig. 5). A woman, aged 38, presents a big nevus on congenital zosteriform nevus of the right lower limb; since 1982, one of the dark elements situated on the lower third of the leg is progressively spreading and becoming polychromic (fig. 6); the clinical suspicious of superficial spreading malignant melanoma is confirmed by the histologic examination. In the literature, the terminology aiming to call this kind of nevic pale brown spot recovered by darker macules is not quite clear, as it was already emphasized by Stewart in 1978 (27). Indeed, numerous different denomination are found in it: "nevus spilus", nevus on nevus", "spotty nevus" "speckled lentiginous nevus", "speckled nevus "spilus".(ABSTRACT TRUNCATED AT 250 WORDS)     

Epithelioid blue nevus: neoplasm Sui generis or variation on a theme?

The epithelioid blue nevus has recently been associated with the Carney complex, which is characterized by myxomas, spotty skin pigmentation, endocrine overactivity, and schwannomas. Using the general criteria proposed by Carney and Ferreiro, similar lesions were identified in 33 patients with no evidence of the Carney complex. Those lesions presented on the face, trunk and extremities of 15 males and 18 females. The mean age was 35 years, much older than those in the Carney complex (mean 16.3 years). Clinical diagnoses included malignant blue nevus, atypical nevus, melanoma, congenital nevus, and dermatofibroma. The lesions were symmetric, predominantly dermal melanocytic proliferations arranged as short fascicles, small nests, and single cells. Large polygonal and epithelioid melanocytes with moderate pleomorphism, and occasional nuclear pseudoinclusions were admixed with heavily pigmented dendritic and spindled melanocytes and melanophages. Rare mitotic figures were seen in some cases. The neoplasms showed a morphologic spectrum that encompassed a group of combined blue nevi with epithelioid melanocytes and other Spitz's nevus characteristics. These epithelioid combined nevi (ECN) fell into three phenotypes with morphologies that most closely paralleled those pictured by Carney and Ferreiro in the Carney complex: the classic or Carney complex pattern (ECN-CC), those that showed overlap with deep penetrating nevus (ECN-DPN), and those that have many dermal Spitz's nevus features, [BLue + SpITZ's nevus; (ECN-BLITZ)I. In six cases, there was such an admixture of features that it was difficult to ascribe them to one of the groups. Nine lesions had associated banal congenital nevus. Follow-up that averaged over 2.5 years (31 months) (range 6-162 months) showed no evidence of malignancy or recurrent disease after excision. Epithelioid combined nevus is a type of combined nevus with blue nevus and Spitz's nevus features, which may or may not be associated with the Carney complex. It shows morphologic overlap with the epithelioid blue nevus described by Carney (ECN-CC), deep penetrating nevus (ECN-DPN), and blue nevus with intradermal Spitz's (desmoplastic) nevus (ECN-BLITZ). Epithelioid combined nevus is thought to be a fitting nosologic designation for all of these lesions.     

Epidermal nevus syndromes: clinical findings in 35 patients

Of the patients with epidermal nevi, 10-18% may have disorders of the eye, nervous, and musculoskeletal systems. A predisposition to malignant neoplasms in ectodermal and mesodermal structures may also be found. There are six different epidermal nevus syndromes described so far: Proteus, congenital hemidysplasia with ichthyosiform nevus and limb defect syndrome, phakomatosis pigmentokeratotica, sebaceous nevus, Becker nevus, and nevus comedonicus. Thirty-five patients with epidermal nevus syndrome seen at the National Institute of Pediatrics in Mexico City during a 31-year period are described. This syndrome represented 7.9% of 443 patients with epidermal nevi; its relative frequency was 1 case per 11,928 pediatric patients and 1 case per 1080 dermatologic patients. Nine epidermal nevus syndrome patients (26%) had Proteus syndrome. Sebaceous nevus syndrome was found in six patients (17%), while the nevus comedonicus syndrome was found in three (8%). Two patients were diagnosed with phakomatosis pigmentokeratotica and one patient with congenital hemidysplasia with ichthyosiform nevus and limb defect syndrome. This is the first report of phakomatosis pigmentokeratotica and congenital hemidysplasia with ichthyosiform nevus and limb defect syndrome in Mexican patients. One patient had an inflammatory linear verrucous epidermal nevus with systemic involvement. Thirteen patients (37%) had keratinocytic nevi with systemic involvement. We propose the keratinocytic nevus syndrome to be defined as the association of a keratinocytic nevus with neuronal migration and/or musculoskeletal disorders in addition to a higher risk for mesodermal neoplasms.     

Desmoplastic hypopigmented hairless nevus: a variant with progressive depigmentation, induration, and overgrowth

Large congenital melanocytic nevus rarely presents itself without hairs, with hardened skin and progressive depigmentation. We report a girl who presented with a large congenital melanocytic nevus in the left cheek. Over the years, the nevus became pruriginous, light brown, bumpy, and hard. Histology revealed nevus cells interspersed with dense fibrosclerotic collagen bundles. There are few reported cases of large congenital melanocytic nevus with this evolution, so-called desmoplastic hypopigmented hairless nevus.     

Plaque-type blue nevus of the oral cavity

BACKGROUND: The blue nevus of the oral cavity is a rare lesion with important differential diagnoses. The plaque-type blue nevus is an uncommon variant of the blue nevus. Because of its particular clinical appearance, it can easily be confused with satellite metastases from malignant melanoma. The diagnosis usually requires a biopsy. OBJECTIVES: To describe the clinical and histological features of a plaque-type blue nevus of the buccal mucosa in a 20-year-old white woman, to review all intraoral blue nevi and all plaque-type blue nevi reported in the literature so far and to compare the criteria of blue nevi and nevus of Ota. RESULTS: An intraoral blue nevus was described for the first time in 1959. Since then around 70 further cases have been documented. Our case is the first report of a plaque-type blue nevus of the oral cavity. CONCLUSIONS: The exceptional widespread intraoral blue nevus described herein can clinically be confused with an intraoral malignant melanoma, and it has a very similar clinical appearance as the intraoral part of nevus of Ota. Apart from the clinical resemblance, there is also some degree of histological overlap of the dermal melanocytoses. Transitional states between blue nevus and nevus of Ota may occur clinically and histologically.     

Linear Cowden nevus: a new distinct epidermal nevus

Within the group of epidermal nevi, a so far nameless disorder is described under the term "linear Cowden nevus". This non-organoid epidermal nevus is caused by loss of heterozygosity, occurring at an early developmental stage in an embryo with a germline PTEN mutation, giving rise to Cowden disease. Hence, linear Cowden nevus can be categorized as a characteristic feature of type 2 segmental Cowden disease. Until now, several authors had mistaken this epidermal nevus as a manifestation of Proteus syndrome. The concept of linear Cowden nevus implies that Proteus syndrome is by no means caused by PTEN mutations. As a clinical difference, linear Cowden nevus is markedly papillomatous and thick, whereas linear Proteus nevus tends to be rather flat. Moreover, the spectrum of possibly associated cutaneous or extracutaneous anomalies differs in the two types of nevi. In conclusion, linear Cowden nevus, that may also be called "linear PTEN nevus", represents a distinct clinicogenetic entity.     

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

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.     

Malignant melanoma and acquired dermal melanocytosis on congenital nevus spilus

We reported a case of malignant melanoma and acquired dermal melanocytosis that appeared on congenital nevus spilus; this is the first report from Japan. An 85-year-old woman had had a nevus spilus on the right lower leg since birth. A black-brown nodule developed on the nevus three years before treatment. Blue-gray patches were found within the nevus on inspection. Histopathological analysis of these lesions revealed superficial spreading melanoma and acquired dermal melanocytosis, respectively. There have been 19 previous case reports of malignant melanoma on nevus spilus, and there have only been 4 cases of dermal melanocytosis (plaque-type blue nevus) on nevus spilus. We reviewed the reported cases in the literature and discussed the risk factors of nevus spilus.     

Nevus roseus: a distinct vascular birthmark

The new term nevus roseus is proposed to denote a lateralized telangiectatic birthmark with a light-red or pale-pink color, unlike the dark hue of nevus flammeus. It appears to be a distinct entity rather than just a color variant of nevus flammeus. Remarkably, nevus flammeus is a characteristic component of phacomatosis pigmentovascularis type II ("phacomatosis cesioflammea"), whereas nevus roseus represents a distinguishing feature of phacomatosis pigmentovascularis type III ("phacomatosis spilorosea"). In analogy to "port-wine stain" that is used as a synonym for nevus flammeus, nevus roseus could also be called "rosé-wine stain". This lateralized vascular birthmark should be distinguished from the salmon patch that always involves the midline of the body. Nevus roseus belongs to the rather broad category of telangiectatic nevi, as well as to the even larger group of "capillary malformations". For obvious reasons these terms are not suitable to designate any specific type of vascular birthmark such as nevus roseus.     

Epithelioid blue nevus: a rare variant of blue nevus not always associated with the Carney complex

Epithelioid blue nevus is a rare variant of blue nevus that has been recently described in patients with Carney complex. Some of the patients with Carney complex have multiple epithelioid blue nevi and a familial history of similar lesions is often recorded. Epithelioid blue nevus consists of an intradermal melanocytic nevus composed of polygonal epithelioid cells laden with melanin. Neoplastic cells show no maturation at the base of the lesion and, in contrast with the usual stromal changes in blue nevi, epithelioid blue nevus exhibits no fibrosis of the dermis. We have studied three cases of epithelioid blue nevus in three patients with no evidence of Carney complex. The lesions were solitary and there was no family history of similar lesions. Therefore, epithelioid blue nevus is a distinctive variant of blue nevus that may also appear as a sporadic lesion and is not always associated with Carney complex.     

Phacomatosis melanorosea without extracutaneous features: an unusual type of phacomatosis pigmentovascularis

Phacomatosis pigmentovascularis (PPV) represents a group of different types of didymosis (twin spotting) characterized by the coexistence of a large pigmentary nevus such as a mongolian spot, café-au-lait macule or macular nevus spilus, and an extensive telangiectatic nevus, such as nevus flammeus or nevus roseus. We describe a third case of phacomatosis melanorosea and discuss the denotation of this neologism.