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.     

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.     

Generalized congenital epithelioid blue nevi (pigmented epithelioid melanocytomas) in an infant: Report of case and review of the literature

The epithelioid blue nevus (EBN) is a variant of the blue nevus characterized by heavily pigmented epithelioid melanocytes and lightly or nonpigmented spindle cells. It may be associated with Carney complex, a multiple neoplasia syndrome. Congenital cases of EBN not associated with Carney complex are rarely reported. We herein describe an infant who presented with multiple blue‐gray nodules and papules involving the head, trunk, and extremities at birth, the corresponding histopathologic findings, and genetic testing results.     

(17) Histopathology of giant congenital melanocytic naevi: implications for treatment

Giant hairy naevi pose not only cosmetic problems, but also therapeutic difficulties because of the increased risk of malignant melanoma. Prophylactic full thickness excision and grafting have previously been recommended for such lesions, but because of the large size this is not always practical. Following observations that in the neonatal period, naevus cells are superficial (predominantly in the papillary dermis) and only later migrate into reticular dermis, some authors have suggested that dermabrasion or shaving of the naevi must be performed soon after birth, as an alternative therapy. We studied 26 children with giant congenital melanocytic naevi to assess: (i) the histological progression with age; (ii) correlation of clinical and histological appearance; and (iii) results of shaving in the treatment of these naevi.
Initial biopsies revealed that naevus cells often occupy the entire dermis, even in the neonatal period, and not infrequently involve the subcutis. Melanocytes showed little organization into nests.
Follow-up biopsies of all naevi showed no change in histological pattern or progression in depth with age, despite changes in clinical appearance.
Post-shaving biopsies revealed that although the epidermis and sometimes the papillary dermis were removed, all the deeper dermal, subcutaneous and fascial components of the naevi remained.
In conclusion our data suggest that: (i) many congenital melanocytic naevi already show deep dermal involvement in the neonatal period; this does not support the hypothesis that naevus cells migrate into the dermis during infancy; (ii) there is no change in naevus depth with age; (iii) shaving does not remove all the naevus cells, and should only be considered as a cosmetic procedure, Three cases showed regeneration of melanocytes following shaving, similar to what is described as the 'pseudomelanoma' phenomenon.     

The carcinoembryonic antigen (CEA) family (CD66) expressed in melanocytic naevi is not expressed in blue naevuscell naevi in dendritic type

BACKGROUND: Although sporadic reports have regarded the expression of the carcinoembryonic antigen (CEA) family in melanoma, there has been no information about the expression in precursor lesions of melanoma such as melanocytic naevi and blue naevi. METHODS: The expression was immunohistochemically studied in frozen biopsy specimens of 45 acquired and 16 congenital melanocytic naevi and 20 blue naevi, using a panel of monoclonal and polyclonal antibodies that recognize different epitopes of CEA and related molecules. RESULTS: Members of the CEA glycoprotein family were strongly expressed in all of the subtypes of melanocytic naevus. A reduced expression of the CEA glycoproteins with increased dermal depth or acquisition of a spindled morphology of naevus cells was apparent. The expression was not seen in the present blue naevi and normal epidermal melanocytes. CONCLUSIONS: Although the significance of the expression was not clarified, this report has clearly demonstrated that the CEA family is strongly expressed in melanocytic naevi and immunoreactivity is divergent between melanocytic naevi and blue naevi and between dermal naevus cell types, suggesting that the expression may be altered with architectural changes in the melanocyte-lineage cells.     

Epithelioid blue nevus occurring in children with no evidence of Carney complex.

We report two pediatric patients who had biopsies of solitary lesions diagnosed as epithelioid blue nevi. Histologically these lesions had wedge-shaped, heavily pigmented infiltrates extending to the subcutaneous fat. The infiltrate was composed of spindled and polyhedral cells that were nevomelanocytic cells with nuclear pleomorphism. Also noted were pigmented globular cells interpreted as melanophages. These lesions have the same characteristics as those blue nevi occurring in patients with Carney complex. More recently, adult patients have been identified with similar nevi, but without evidence of Carney complex. To our knowledge, pediatric patients with epithelioid blue nevi, but no evidence of Carney complex have not been described previously.     

,,Blaue`` Variante eines Naevus spilus

Summary We report on a 29-year-old female patient with an unusual pigmented lesion of the face. Clinically the lesion looked like a pigment patch of the naevus spilus type, while histological examination revealed the presence of dermal melanocytosis and multiple common blue naevi with a discrete lentiginous component in addition. The melanocytic nature of the infiltrate was ascertained by immunohistochemistry analysis using S 100 protein and HMB 45 antibodies. We interpret this lesion as agminated blue naevi in association with lentigo simplex, an unusual variant of speckled lentiginous naevus.      

色痣和蓝痣的电镜观察

对4例色痣和2例蓝痣进行了电子显微镜观察.发现色痣的痣细胞中充满着许多第皿期和第N期黑素小体.蓝痣的黑素细胞在真皮深部.胞质中的黑素颗粒也相当多.在色痣和蓝痣的痣细胞中可见含黑素的大球形体.可能代表退行现象,也许是溶酶体吞噬了退变的黑素颗粒而形成的.最后讨论了色痣和蓝痣临床色泽差别的原因,蓝痣除黑素细胞部位深、受折光物理因素影响外.细胞中黑幸小体的含量、着色程度也有一定影响.     

Variations in S-100 protein expression in naevocellular naevi may be related to metabolic activity

Significant amounts of S-100 protein were demonstrated in naevus cells by an immunoperoxidase technique. Distribution of S-100 protein was similar in the various types of naevi tested. In most lesions, the superficial A-type naevus cells stained most heavily, while staining was weaker in the deeper B- and C-type naevus cells. The intensity of staining was not, however, homogeneous in the cells of the various layers of the naevus. S-100 protein was especially abundant in giant naevus cells which occurred in the vicinity of sub-epidermal A-type naevocytes. Cells from neurocutaneous melanosis contained extremely large amounts of S-100 protein. Normal melanocytes in the epidermis over dermal naevi contained variable amounts of S-100 protein, but mainly showed weak staining. However, in the basal epidermis of traumatized naevi there were many unusually large and partly dendritic melanocytes. In this part of the epidermis, S-100 protein-positive cells lay close together, and many stained strongly. In earlier work, large amounts of metabolic enzymes were found at the same sites in traumatized naevi and giant cells. These results suggest that a high degree of expression of S-100 protein is due to metabolic activity shown, for example, by giant naevus cells and basal melanocytes of the regenerating epidermis over traumatized naevi.     

Naevus fusco-caeruleus zygomaticus

One hundred and ten cases of an unusual type of naevus, which we have called naevus fusco-caeruleus zygomaticus were studied. The naevus presents as a bilateral speckled discolouration of the skin of the face principally in the zygomatic region. The condition usually does not become apparent until the second decade of life, and is much commoner in females. No association with any other abnormalities was found. Light and electron microscopy of the speckles showed the presence of dermal melanocytes mostly in the upper dermis. A survey was also carried out to determine the prevalence of this naevus in the general population.     

Animal type melanoma: a report of a case with balloon-cell change and sentinel lymph node metastasis

Animal type melanoma is a rare histopathologic variant of melanoma characterized by sheets and nodules of heavily pigmented epithelioid melanocytes that involve the entire thickness of the dermis. This human neoplasm mimics melanocytic neoplasms seen in gray horses and laboratory animals; thus, is termed animal type melanoma. It is quite rare and, with only a few reported cases, its biological behavior is not well understood. We report an example of animal type melanoma on the back of a 27-year-old man. The lesion showed areas of melanoma in situ, which ruled out the possibility of metastatic melanoma. Features of regression were also seen at dermo-epidermal junction and papillary dermis. In some areas, neoplastic melanocytes exhibited a balloon-cell appearance; in others the neoplasm was composed of sheets and fascicles of heavily pigmented epithelioid melanocytes that permeated the entire dermis and extended into the dermal-subcutaneous interface, mimicking a cellular blue nevus. Epithelioid melanocytes in deeper areas showed abundant, heavily pigmented cytoplasm and pleomorphic nuclei with prominent eosinophilic nucleoli and some mitotic figures. The neoplastic cells did not show evidence of maturation in deeper areas of the lesion. In some sections, a nodule of heavily pigmented epithelioid melanocytes was seen far from the main bulk of the lesion, at the dermal-subcutaneous interface, raising the possibility of a satellite lesion. A lymphoscintigraphy showed a sentinel lymph node in the right axilla and a subsequent axillary lymphadenectomy demonstrated that the architecture of the sentinel lymph node was effaced by metastatic melanoma. The patient received adjuvant chemotherapy with inteferon alfa-2b and four months after this treatment the patient is alive and well, without evidence of recurrences or additional metastases.     

颧部褐青色痣黑素细胞特性及来源的研究

目的为了进一步证实颧部褐青色痣的组织病理特点,并初步探讨颧部褐青色痣黑素细胞的来源。方法选取颧部褐青色痣、皮内痣、蓝痣及黑变病标本各10例,所有病理切片标本均进行HE染色和免疫组化法检测CD68、S-100、Melan-A、NSE、C-Kit(CD117)在黑素细胞上的表达。结果①颧部褐青色痣的HE染色:在真皮浅层有散在的含黑素颗粒的梭形细胞分布,其细胞长轴与表皮平行,主要分布于真皮浅层。真皮含黑素颗粒细胞分为散在或弥漫的分布。②免疫组化:颧部褐青色痣、皮内痣和蓝痣各组中银染色、S-100、Melan-A、NSE、CD68免疫组化均为阳性,其中Melan-A染色在颧部褐青色痣和皮内痣组显示有表皮基底层黑素细胞增加;黑变病组仅有CD68阳性。C-kit阳性在颧部褐青色痣组有9例、蓝痣组有8例、皮内痣组有5例。结论颧部褐青色痣的表皮基底黑素细胞增加,真皮内既有黑素细胞,也有噬黑素细胞。其黑素细胞是一种具有痣细胞、神经嵴来源细胞及早期胚胎细胞的特性。     

Blue naevus with satellitosis mimicking malignant melanoma

Blue naevus is an acquired benign melanocytic naevus. It is a firm, sharply defined dark blue to grey-black papule or nodule, which is likely to arise from the arrested dermal melanocytes in the dermis. In the last few years, blue naevus has attracted much attention due to the recognition of new entities and to its confusion with malignant melanoma. We report a 69-year-old man who developed a blue-black nodular lesion with satellitosis on his scalp. Although clinically it was thought to be a malignant melanoma, histopathological investigation and conservative methods such as dermatoscopy and power Doppler ultrasonography did not confirm this diagnosis. Histopathological examination excluded malignant melanoma, as there were no cellular atypia and mitotic activity in either the nodular lesion or the satellitosis. Doppler ultrasonography confirmed the benign nature of the lesion. Dermatoscopic examination showed homogeneous steel-blue pigmentation with individual blue globules, dots and some brown veils, and confirmed the histopathological diagnosis. To the best of our knowledge, our case is the third reported case of a blue naevus with satellitosis mimicking malignant melanoma.     

Sclerosing 'mucinous' blue naevus

Abundant mucin deposition is an unusual finding in melanocytic naevi. We describe the first example, to our knowledge, of a sclerosing blue naevus with an abundant mucinous stroma in two unrelated patients. This uncommon variant of blue naevus should be differentiated from desmoplastic-neurotropic melanoma, in which the presence of mucin stromal deposition is a more typical finding.     

HMB 45 positive balloon cells in combined nevi]

The combined naevus is made up of two components, one resembling a melanocytic naevus, the other a blue naevus. Clinically, these naevi do not give any obvious cause for concern. Histological examination shows that the combined naevus consists of a superficial melanocytic naevus and a deep-seated spindle cell blue naevus. There is a rare variant in which the pigmented spindle cells of the "blue" naevus are replaced by large balloon cells varying in melanin content. These combined naevi, because of the large cells with abundant cytoplasm, closely resemble malignant melanoma. As a further aid to diagnosis we used the monoclonal antibody HMB 45. In our study, the vesicular cells in all seven combined naevi examined reacted strongly with HMB 45. It is suggested that HMB 45 is not always helpful in differentiating between melanoma and naevi.     

Carney complex: In a patient with multiple blue naevi and lentigines, suspect cardiac myxoma

Carney complex is characterized by spotty pigmentation (blue naevi and lentigines), myxomas (cardiac, cutaneous, mammary), endocrine over-activity (Cushing's syndrome, acromegaly), testicular tumours, and schwannomas. We report a male with multiple blue naevi, lentigines, testicular large cell calcifying Sertoli-cell tumour and four cardiac myxomas. The myxomas caused two cerebrovascular accidents and a myocardial infarction. All patients with multiple blue naevi or lentigines should be investigated for the life-threatening association of cardiac myxomas.     

Pathogenic mechanisms in epidermolysis bullosa naevi

Epidermolysis bullosa naevi are large, eruptive melanocytic naevi which frequently arise in areas of former blisters in patients suffering from inherited epidermolysis bullosa. Morphologically, these naevi are similar to malignant melanoma, although so far no malignant transformation has been observed. To investigate the pathogenesis of these moles we documented their clinical evolution and their histopathological and immunocytological characteristics in three patients with epidermolysis bullosa. Clinically, we observed signs of malignant transformation, such as explosive growth and the occurrence of satellite lesions of epidermolysis bullosa naevi. However, malignant melanoma was excluded by histopathological evaluation. In addition, we evaluated the concentrations of various factors known to stimulate melanocyte growth in blister fluid. Human interleukin 8, basic fibroblast growth factor, human hepatocyte growth factor, GM-CSF, leukotriene B4 and prostaglandin E2 revealed concentrations comparable with the levels in inflammatory blisters. We were able to detect individual melanocytes/naevus cells in blister fluid from a blister over an epidermolysis bullosa naevus. The factors detected in the blister fluid might therefore promote the proliferation, migration and melanogenesis of disconnected melanocytes/naevus cells representing the basis of the highly dynamic appearance of epidermolysis bullosa naevi.     

Pigmented spindle cell naevus

We report 22 cases of pigmented spindle cell naevus (PSCN). The usual appearance of these naevi is that of a heavily pigmented papule found mostly on the legs of young patients. Histologically, PSCN was characterized by symmetrical proliferation of spindle-shaped pigmented melanocytes grouped in large junctional nests. Pagetoid spread of single cells in the overlying epidermis was frequently found. In our opinion, PSCN is a distinctive benign acquired melanocytic naevus that in the past has been frequently misdiagnosed as atypical Spitz naevi or malignant melanoma.     

Speckled lentiginous naevus: which of the two disorders do you mean?

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. In contrast, naevus spilus papulosus is defined by dark papules that are of different sizes and rather unevenly distributed, reminiscent of a star map. Histopathologically, the dark spots of naevus spilus maculosus show a 'jentigo' pattern and several nests of melanocytes involving the dermoepidermal junction at the tips of the papillae, whereas most of the dark speckles of naevus spilus papulosus are found to be dermal or compound melanocytic naevi. The propensity to develop Spitz naevi appears to be the same in both types of speckled lentiginous naevus, whereas development of malignant melanoma has been reported far more commonly in naevus spilus maculosus.