Newborn infant with congenital lentigines as a manifestation of Carney Complex.

Carney Complex (CNC) is a rare syndrome characterised by skin pigmentation, endocrine over activity and myxomas, with the median age of detection being 20 years. We present a case of CNC diagnosed in infancy after being noted to have multiple lentigines over his face, abdomen, back and thighs at birth. We consider the differential diagnoses of similar cutaneous presentations in the well neonate and review the prognosis and suggested surveillance of patients with CNC.

Acropigmentación reticulada de Kitamura y nevus de Ito / Reticulate acropigmentation of Kitamura and nevus of Ito

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Hypermelanoses of the newborn and of the infant.

This article discusses hypermelanoses present at birth or appearing in the first months of life. They may be transient or permanent, localized - as in café-au-lait spots - or segmental, or more rarely, complex or generalized. In most pigmentary diseases, physical examination, including Wood's lamp examination and a detailed history, is usually sufficient. Time of onset, distribution pattern, and associated clinical and sometimes histopathologic findings are helpful in differentiating these disorders. Recently, molecular diagnosis has become available for some rare entities, such as hereditary symmetrical dyschromatoses, but the bulk of nevoid lesions are not understood at the molecular level.

[Recurrent familial cardiac myxomas and lentiginosis. Second recurrence in a 41-year-old female patient]. / Rezidivierende familiäre kardiale Myxome und Lentiginose. Zweites Myxomrezidiv bei einer 41-jährigen Patientin.

The case of a 41-year-old woman with recurrent cardiac myxomas and widespread lentiginosis is reported. The diagnosis of a Carney complex was established 7 and 25 years, respectively, after first manifestation of the cardiac myxomas in both the patient and her brother. This peculiar hereditary disease is commonly associated with multiple neoplasms and an endocrine overactivity, requiring a thorough examination of the patients and their relatives to detect additional typical manifestations.

A giant congenital nevus spilus in an 8-year-old girl.

Congenital nevus spilus is rare, and the lesion is usually small. This report describes an 8-year-old girl with a giant congenital nevus spilus that involved the left side of her abdomen, perineal area, and left upper thigh. The patient also had a Mongolian spot in the lumbosacral area.

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.

Congenital unilateral speckled lentiginous blue nevi with asymmetric spinal muscular atrophy.

We describe a 30-year-old man presenting with congenital widespread unilateral hyperpigmented areas on the right side of the face and limbs that were studded with multiple blue nevi. There was nevus of Ota-like scleral involvement of the right eye. Histologic examination showed common blue nevi, one in association with lentigo. For this unusual type of dermal melanocytosis we propose the term, congenital unilateral speckled lentiginious blue nevi. In addition, the patient had monomelic spinal muscular atrophy of his right shoulder girdle and arm.

Agminated intradermal Spitz nevi arising on an unusual speckled lentiginous nevus with localized lentiginosis: a continuum?

We report an 18-year-old boy with a congenital pigmented lesion measuring 2 x 6 cm on his right thigh. About a third of the lesion was composed of numerous lentiginous macules superimposed on histologically normal and clinically nontan skin; in the remainder of the lesion, several macules and papules with histologic features of junctional and compound nevi were superimposed on clinically normal skin, which had a lentiginous pattern histologically. Some years later, eruptive intradermal Spitz nevi developed at one corner of the lesion. The combined clinical and histological features of the lesion fulfill descriptions for both segmental lentiginosis and an unusual variant of speckled lentiginous nevus. Our case points out the limitations of using strict diagnostic criteria to define speckled lentiginous nevus and offers an opportunity to consider the natural history of the lesion as a continuum from lentigines to melanocytic nevi. Moreover, the presence of eruptive intradermal Spitz nevi arising within the area of speckled lentiginous nevus lacking a distinct tan background, suggests the possibility that the entire area of the lesion per se constitutes an environment where development of nevi is enhanced.

Agminated Spitz nevi occurring within a congenital speckled lentiginous nevus.

A 40-year-old woman had a speckled lentiginous nevus on her thigh since birth. During her first pregnancy, additional papules and nodules appeared within the preexisting hyperpigmented area, histologic examination of which showed features of both junctional and compound Spitz's nevi accompanied by simple lentigolike changes. In this particular case, speckled lentiginous nevus may have constituted a particular environment for the production of multiple Spitz nevi.

Extensive unilateral speckled lentiginous 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.

[White lentiginosis]. / La lentiginose blanche.

INTRODUCTION: A congenital guttate hypomelanosis is an unusual feature not yet mentioned in the dermatologic literature. CASE REPORT: We observed 1982 in a 28 y. female patient numerous guttate lesions, which were flat and pigmented on the light-exposed areas of her limbs, flat or papulokeratotic and depigmented on her trunk. These lesions disclosed a particular histological aspect characterized by a lentiginous hyperplasia of the epidermis, with elongated club-shaped rete ridges, and an unusual loss of pigmentation without disturbance of the keratinization. Further electronmicroscopical and immunohistochemical data were not available. The patient emphasized the congenital occurrence of these lesions, whose fixity could be assessed during a 4 year-follow up time. COMMENTS: The unusual histological aspect allows the differentiation of these depigmented spots and other known similar conditions: macular leucoderma as sequellae of previous inflammatory diseases, hypomelanotic macules associated with genodermatoses, idiopathic guttate hypomelanoses. CONCLUSION: This seems to be a not yet described entity which we propose to denominate "white lentiginosis".

Multiple lentigines syndrome in a Nigerian family.

Three cases of the multiple lentigines syndrome are reported in a Nigerian family, involving a father and two of his children. The major abnormalities in this syndrome include: multiple lentigines, cardiac defects, genitourinary malformations, neurological deficits, growth retardation, cephalofacial dysmorphism and a family history consistent with an autosomal dominant mode of inheritance. We hereby report these cases in whom the main features are multiple lentigines, ocular hypertelorism, electrocardiographic defects and a pattern consistent with an autosomal dominant mode of inheritance.