Background: There is a dearth of information regarding the occurrence of neurocutaneous melanocytosis (NCM) in a large cohort of persons with large congenital melanocytic nevi (LMCN) or multiple congenital melanocytic...Background: There is a dearth of information regarding the occurrence of neurocutaneous melanocytosis (NCM) in a large cohort of persons with large congenital melanocytic nevi (LMCN) or multiple congenital melanocytic nevi (MCMN). Objective: The purpose of this article is to report occurrence of NCM and other complications in 1008 persons having LCMN or MCMN. Methods: Evaluation of information obtained from a database of persons with LCMN or MCMN voluntarily submitted by the affected persons to a nevus support group, the Nevus Network. Results: Of those with truncal LCMN, 6.8% developed significant complications, 4.8% developed symptomatic NCM, and 2.3% died from either benign or malignant NCM or cutaneous melanoma. Of the 4.8% of persons with a truncal nevus who developed symptomatic NCM, 34% died. Of those with head or extremity LCMN, 0.8% developed symptomatic NCM, and, to date, none have died from any cause. Of the small number with MCMN without a giant nevus, 71% developed symptomatic NCM, and 41% died of it. Limitations: Attending physician confirmation of submitted information was unavailable. Conclusions: LCMN of the trunk were associated with a relatively low occurrence of medical complications and death in our group, considering the large nevomelanocytic burden present. If symptomatic NCM developed in those with truncal nevi, the occurrence of death rose to a third. LCMN of the head or extremity were associated with minimal medical complications and no deaths. In contrast, most of the rare persons (N = 17) with MCMN developed symptomatic NCM, and more than a third died.展开更多
The balloon-cell nevus was first described over 100 years ago. Since then, balloon-cell changes of melanocytes have been noted in numerous tumors, including melanoma, blue nevus, and Spitz nevus. Whether these changes...The balloon-cell nevus was first described over 100 years ago. Since then, balloon-cell changes of melanocytes have been noted in numerous tumors, including melanoma, blue nevus, and Spitz nevus. Whether these changes reflect cellular deterioration or proliferative changes is a matter of debate. We report a case in which balloon-cell changes were found within proliferative nodules occurring in a large congenital melanocytic nevus.展开更多
Background: Recently, we identified and described dermoscopic aspects, present with a higher frequency in congenital melanocytic lesions with respect to acquired naevi. We also classified small- and medium-sized conge...Background: Recently, we identified and described dermoscopic aspects, present with a higher frequency in congenital melanocytic lesions with respect to acquired naevi. We also classified small- and medium-sized congenital naevi (CN) into nine subtypes according to their macroscopic and dermoscopic aspects. Objectives: Because the recognition of dermoscopic features may be instrument dependent, in this study, we wanted to check whether dermoscopic patterns specific for CN can be identified in digital images acquired by means of different instruments. We also wanted to check the validity of our previously proposed classification and assess possible age- and site-dependent variations of dermoscopic patterns and naevus subtypes. Patients/methods: Images corresponding to 384 small- or medium-sized CN were collected in eight different centres employing four different instruments. Lesion images were evaluated and checked for the presence of specific dermoscopic criteria, classified, and compared with a database of 350 acquired naevi. Results: Specific and unspecific dermoscopic features were identifiable in images acquired by means of all four instrument types The mean number of identified features per lesion did not vary according to the instrument employed for the acquisition of the images; however, it was lower for lesions recorded employing low magnifications. The previously proposed classification was easily applied to the whole image database. The variegated naevus type was identified as a highly specific clinical/dermoscopic pattern. Dermoscopic features varied according to age and location. The globular type prevailed in subjects under 11 years of age and on the trunk, whereas the majority of reticular lesions were located on the limbs. Conclusions: Because definite clinical and histological criteria for the diagnosis of the congenital nature of naevi are lacking, the use of dermoscopy can be of great help in identifying those lesions where the presence of specific dermoscopic features makes the diagnosis of CN more likely. Moreover, dermoscopy can be useful both for the classification of lesions already identified as congenital according to definite clinical and anamnestic data and for a possible correlation of naevus phenotype and dermoscopic patterns to the risk of developing a malignant melanoma in prospective studies.展开更多
Background: Based on the dermoscopic classification of acquired melanocytic naevi, six different dermoscopic types can be distinguished by morphology (globular, globular-reticular, globular-homogeneous, reticular, ret...Background: Based on the dermoscopic classification of acquired melanocytic naevi, six different dermoscopic types can be distinguished by morphology (globular, globular-reticular, globular-homogeneous, reticular, reticular-homogeneous, homogeneous) and by pigment distribution (uniform, central hyperpigmentation, central hypopi- gmentation, peripheral hyperpigmentation, peripheral hypopigmentation, multifocal hyper/ hypopigmentation). It has been suggested that most individuals harbour one predominant dermoscopic type among their naevi. Objectives: To evaluate whether the age of the patient influences the predominant naevus pattern observed in individuals with multiple acquired melanocytic naevi. Methods: Individuals were recruited from the pigmented skin lesion clinic in Graz between July 2000 and February 2001. Individuals with at least 10 melanocytic naevi were selected consecutively until a total of 10 individuals in each of five age groups was obtained. Age groups were: 0-15 years, 16-30 years, 31-45 years, 46-60 years and > 60 years. Digitized images of acquired melanocytic naevi, defined as benign melanocytic proliferations having a diameter of at least 5 mm with a macular component and which were not apparent within the first year of life, were evaluated by dermoscopic criteria. The associations of dermoscopic features as a function of patient age were analysed. We calculated absolute numbers and frequencies, given as percentages, as well as predominance of the dermoscopic types of naevi in the different age groups. Results: Analysis of 1268 naevi revealed that the globular pattern predominated in the youngest age group. By contrast, the reticular and/or homogeneous patterns were increasingly exhibited in naevi from older individuals (older than 15 years). Uniform pigmentation was most common in melanocytic naevi in the youngest age group, while central hyperpigmentation was predominantly seen in the group of individuals aged 16-30 years. Conclusions: The predominance of dermoscopic types of melanocytic naevi varies according to the individual’s age. Awareness of the age-related dermoscopic predominance of melanocytic naevi might allow more accurate recognition of dermoscopic patterns of melanocytic skin lesions that are unusual with respect to the individual’s age. This observation may help in the early recognition of some ‘banal’appearing melanomas. Furthermore, the observations made in this study raise interesting questions regarding naevus evolution.展开更多
Background: Giant congenital melanocytic naevi (GCMN)-convey a 14-fold increased melanoma risk. In contrast,medium congenital melanocytic naevi (MCMN) are rarely associated with malignant transformation. Management of...Background: Giant congenital melanocytic naevi (GCMN)-convey a 14-fold increased melanoma risk. In contrast,medium congenital melanocytic naevi (MCMN) are rarely associated with malignant transformation. Management of patients with GCMN is challenging and there is no consensus on the most appropriate strategy for treating these patients. Objectives: To provide a rationale for performing curettage of GCMN in the neonatal period in order to reduce the risk of malignant transformation to melanoma. Methods: Twenty-six infants with GCMN who underwent biopsies before excisional surgery (n = 7) or curettage (n = 19) during the past 14 years (Academic Hospital, Vrije Universiteit Brussel) and 10 MCMN patients who underwent excision biopsies (Radboud University Nijmegen Medical Centre) were included in this study. Using these biopsies, we performed genetic and detailed immunohistochemical evaluations of changes that are associated with malignant transformation. Variables of interest included melanoma-associated BRAF mutations, proliferative activity, vascularity, cellular context and extracellular matrix architecture. Results: GCMN and MCMN did not show oncogenic BRAF mutation and displayed similar features with respect to the amount of nonmelanocytic cells within the naevus and matrix architecture. Naevus cells in the superficial component of the GCMN, however, were more proliferative, and this component was more vascular compared with its deep component and with MCMN. In this study, none of the 19 newborn patients who underwent curettage developed a melanoma within a mean follow-up time of 7 years. Conclusions: The data presented here support the idea that curettage of GCMN in neonates has the potential for lowering the risk of developing cutaneous melanoma by not only obtaining an important numerical reduction of naevus cells but also removing the ‘active’melanocytes.展开更多
Background: Melanocytic nevi with eccentric foci of hyperpigmentation (“ Bolognia sign” ) can be considered as a melanoma-simulating type of acquired melanocytic nevus. We report on the morphologic changes of this t...Background: Melanocytic nevi with eccentric foci of hyperpigmentation (“ Bolognia sign” ) can be considered as a melanoma-simulating type of acquired melanocytic nevus. We report on the morphologic changes of this type of melanocytic nevus over a 39-month period of dermoscopic follow-up. Observations: A 5-year-old girl had a 4-mm brown papule with a peripheral blue-black area on her right upper arm. The eccentric focus of the hyperpigmentation corresponded dermoscopically to a blue-gray area of pigmentation associated with irregular brown-black globules or dots and partially with a superficial black network. After 39 months, a globular type of acquired melanocytic nevus was detectable, which clinically and dermoscopically appeared to be completely benign. A nearly identical situation was observed in 5 other melanocytic nevi, underlining the involution of the pigmented foci in these nevi. The histopathologic diagnoses of 2 lesions were consistent with a compound type of acquired melanocytic nevus with eccentric foci of hyperpigmentation. Conclusions: Dermoscopy allows identification of a morphologic pathway of modifications, probably typical for this type of melanocytic nevus in children, and therefore enables avoidance of surgical excision with attendant hypertrophic scarring in children. Conversely, in adults, when dermoscopic follow-up of melanocytic nevi reveals eccentric foci of hyperpigmentation, surgical excision of the lesion is indicated.展开更多
文摘Background: There is a dearth of information regarding the occurrence of neurocutaneous melanocytosis (NCM) in a large cohort of persons with large congenital melanocytic nevi (LMCN) or multiple congenital melanocytic nevi (MCMN). Objective: The purpose of this article is to report occurrence of NCM and other complications in 1008 persons having LCMN or MCMN. Methods: Evaluation of information obtained from a database of persons with LCMN or MCMN voluntarily submitted by the affected persons to a nevus support group, the Nevus Network. Results: Of those with truncal LCMN, 6.8% developed significant complications, 4.8% developed symptomatic NCM, and 2.3% died from either benign or malignant NCM or cutaneous melanoma. Of the 4.8% of persons with a truncal nevus who developed symptomatic NCM, 34% died. Of those with head or extremity LCMN, 0.8% developed symptomatic NCM, and, to date, none have died from any cause. Of the small number with MCMN without a giant nevus, 71% developed symptomatic NCM, and 41% died of it. Limitations: Attending physician confirmation of submitted information was unavailable. Conclusions: LCMN of the trunk were associated with a relatively low occurrence of medical complications and death in our group, considering the large nevomelanocytic burden present. If symptomatic NCM developed in those with truncal nevi, the occurrence of death rose to a third. LCMN of the head or extremity were associated with minimal medical complications and no deaths. In contrast, most of the rare persons (N = 17) with MCMN developed symptomatic NCM, and more than a third died.
文摘The balloon-cell nevus was first described over 100 years ago. Since then, balloon-cell changes of melanocytes have been noted in numerous tumors, including melanoma, blue nevus, and Spitz nevus. Whether these changes reflect cellular deterioration or proliferative changes is a matter of debate. We report a case in which balloon-cell changes were found within proliferative nodules occurring in a large congenital melanocytic nevus.
文摘Background: Recently, we identified and described dermoscopic aspects, present with a higher frequency in congenital melanocytic lesions with respect to acquired naevi. We also classified small- and medium-sized congenital naevi (CN) into nine subtypes according to their macroscopic and dermoscopic aspects. Objectives: Because the recognition of dermoscopic features may be instrument dependent, in this study, we wanted to check whether dermoscopic patterns specific for CN can be identified in digital images acquired by means of different instruments. We also wanted to check the validity of our previously proposed classification and assess possible age- and site-dependent variations of dermoscopic patterns and naevus subtypes. Patients/methods: Images corresponding to 384 small- or medium-sized CN were collected in eight different centres employing four different instruments. Lesion images were evaluated and checked for the presence of specific dermoscopic criteria, classified, and compared with a database of 350 acquired naevi. Results: Specific and unspecific dermoscopic features were identifiable in images acquired by means of all four instrument types The mean number of identified features per lesion did not vary according to the instrument employed for the acquisition of the images; however, it was lower for lesions recorded employing low magnifications. The previously proposed classification was easily applied to the whole image database. The variegated naevus type was identified as a highly specific clinical/dermoscopic pattern. Dermoscopic features varied according to age and location. The globular type prevailed in subjects under 11 years of age and on the trunk, whereas the majority of reticular lesions were located on the limbs. Conclusions: Because definite clinical and histological criteria for the diagnosis of the congenital nature of naevi are lacking, the use of dermoscopy can be of great help in identifying those lesions where the presence of specific dermoscopic features makes the diagnosis of CN more likely. Moreover, dermoscopy can be useful both for the classification of lesions already identified as congenital according to definite clinical and anamnestic data and for a possible correlation of naevus phenotype and dermoscopic patterns to the risk of developing a malignant melanoma in prospective studies.
文摘Background: Based on the dermoscopic classification of acquired melanocytic naevi, six different dermoscopic types can be distinguished by morphology (globular, globular-reticular, globular-homogeneous, reticular, reticular-homogeneous, homogeneous) and by pigment distribution (uniform, central hyperpigmentation, central hypopi- gmentation, peripheral hyperpigmentation, peripheral hypopigmentation, multifocal hyper/ hypopigmentation). It has been suggested that most individuals harbour one predominant dermoscopic type among their naevi. Objectives: To evaluate whether the age of the patient influences the predominant naevus pattern observed in individuals with multiple acquired melanocytic naevi. Methods: Individuals were recruited from the pigmented skin lesion clinic in Graz between July 2000 and February 2001. Individuals with at least 10 melanocytic naevi were selected consecutively until a total of 10 individuals in each of five age groups was obtained. Age groups were: 0-15 years, 16-30 years, 31-45 years, 46-60 years and > 60 years. Digitized images of acquired melanocytic naevi, defined as benign melanocytic proliferations having a diameter of at least 5 mm with a macular component and which were not apparent within the first year of life, were evaluated by dermoscopic criteria. The associations of dermoscopic features as a function of patient age were analysed. We calculated absolute numbers and frequencies, given as percentages, as well as predominance of the dermoscopic types of naevi in the different age groups. Results: Analysis of 1268 naevi revealed that the globular pattern predominated in the youngest age group. By contrast, the reticular and/or homogeneous patterns were increasingly exhibited in naevi from older individuals (older than 15 years). Uniform pigmentation was most common in melanocytic naevi in the youngest age group, while central hyperpigmentation was predominantly seen in the group of individuals aged 16-30 years. Conclusions: The predominance of dermoscopic types of melanocytic naevi varies according to the individual’s age. Awareness of the age-related dermoscopic predominance of melanocytic naevi might allow more accurate recognition of dermoscopic patterns of melanocytic skin lesions that are unusual with respect to the individual’s age. This observation may help in the early recognition of some ‘banal’appearing melanomas. Furthermore, the observations made in this study raise interesting questions regarding naevus evolution.
文摘Background: Giant congenital melanocytic naevi (GCMN)-convey a 14-fold increased melanoma risk. In contrast,medium congenital melanocytic naevi (MCMN) are rarely associated with malignant transformation. Management of patients with GCMN is challenging and there is no consensus on the most appropriate strategy for treating these patients. Objectives: To provide a rationale for performing curettage of GCMN in the neonatal period in order to reduce the risk of malignant transformation to melanoma. Methods: Twenty-six infants with GCMN who underwent biopsies before excisional surgery (n = 7) or curettage (n = 19) during the past 14 years (Academic Hospital, Vrije Universiteit Brussel) and 10 MCMN patients who underwent excision biopsies (Radboud University Nijmegen Medical Centre) were included in this study. Using these biopsies, we performed genetic and detailed immunohistochemical evaluations of changes that are associated with malignant transformation. Variables of interest included melanoma-associated BRAF mutations, proliferative activity, vascularity, cellular context and extracellular matrix architecture. Results: GCMN and MCMN did not show oncogenic BRAF mutation and displayed similar features with respect to the amount of nonmelanocytic cells within the naevus and matrix architecture. Naevus cells in the superficial component of the GCMN, however, were more proliferative, and this component was more vascular compared with its deep component and with MCMN. In this study, none of the 19 newborn patients who underwent curettage developed a melanoma within a mean follow-up time of 7 years. Conclusions: The data presented here support the idea that curettage of GCMN in neonates has the potential for lowering the risk of developing cutaneous melanoma by not only obtaining an important numerical reduction of naevus cells but also removing the ‘active’melanocytes.
文摘Background: Melanocytic nevi with eccentric foci of hyperpigmentation (“ Bolognia sign” ) can be considered as a melanoma-simulating type of acquired melanocytic nevus. We report on the morphologic changes of this type of melanocytic nevus over a 39-month period of dermoscopic follow-up. Observations: A 5-year-old girl had a 4-mm brown papule with a peripheral blue-black area on her right upper arm. The eccentric focus of the hyperpigmentation corresponded dermoscopically to a blue-gray area of pigmentation associated with irregular brown-black globules or dots and partially with a superficial black network. After 39 months, a globular type of acquired melanocytic nevus was detectable, which clinically and dermoscopically appeared to be completely benign. A nearly identical situation was observed in 5 other melanocytic nevi, underlining the involution of the pigmented foci in these nevi. The histopathologic diagnoses of 2 lesions were consistent with a compound type of acquired melanocytic nevus with eccentric foci of hyperpigmentation. Conclusions: Dermoscopy allows identification of a morphologic pathway of modifications, probably typical for this type of melanocytic nevus in children, and therefore enables avoidance of surgical excision with attendant hypertrophic scarring in children. Conversely, in adults, when dermoscopic follow-up of melanocytic nevi reveals eccentric foci of hyperpigmentation, surgical excision of the lesion is indicated.
