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ORIGINAL ARTICLE |
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| Year : 2006 | Volume
: 51
| Issue : 1 | Page : 26-29 |
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| Melanocytic nevi in children: A clinical study |
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M Senthilkumar, Devinder Mohan Thappa
Department of Dermatology & STD, Jawaharlal Institute of Post graduate Medical Education and Research, Pondicherry-605 006, India
Correspondence Address:
M Senthilkumar
Department of Dermatology & STD, Jawaharlal Institute of Post graduate Medical Education and Research, Pondicherry-605 006
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0019-5154.25184
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BACKGROUND: Melanocytic nevi show different presentations in children which have not much studied in India. PURPOSES: To document the incidence and pattern of melanocytic nevi in children. METHODS: A total of 4,256 paediatric cases attending the dermatology OPD, JIPMER during the study period from August 2002 to August 2004 were screened for melanocytic nevi. FINDINGS: Out of these, 41 (0.96%) cases were found to have melanocytic nevi. The mean age of these cases was 1.4 years (range of 3 days to 14 years). Fourteen (34.1%) of them were males and 27 (65.9%) were females with male to female ratio of 1:1.9. Majority of these cases (32 cases, 78%) were in the age group of 0-1 year. Thirty-seven (90.2%) cases had single lesion and 4 (9.8%) cases had multiple lesions. The size of the nevi varied from 1.5 cm to 20 cm in 40 (97.6%) cases and more than 20 cm in 1 (2.4%) case. The most common site of involvement was the back (32 cases, 78%) followed by head and neck (6 cases, 14.6%). Out of the total of 41 cases, 30 (73.2%) were Mongolian spots, 8 (19.5%) were congenital melanocytic nevi, one case (2.4%) each had giant congenital melanocytic nevus, nevus spilus and nevus of Ota. Four cases had other associated cutaneous disorders. These included ashleaf macules in 2 cases, epidermolysis bullosa simplex in 1 case and a solitary case had both cafι-au-lait macule and mixed hemangioma. The systemic associations included seizures (suspected neurocutaneous melanosis) in a solitary case of congenital melanocytic nevi. CONCLUSIONS: The spectrum of melanocytic nevi seen in children in this study partially differs from this of abroad studies. LIMITATION: Unicentre hospital-based study which cannot be generalized.
Keywords: Melanocytic nevi, Mongolian spots, Children
How to cite this article:
Senthilkumar M, Thappa DM. Melanocytic nevi in children: A clinical study. Indian J Dermatol 2006;51:26-9 |
| Introduction | |  |
The term melanocytic nevi include a wide range of conditions having different presentations depending upon their morphologic and biologic behaviours.[1],[2] They have been broadly grouped into five types: (1) Congenital melanocytic naevi, (2) Acquired melanocytic nevi (junctional, compound, intradermal), (3) Special variants (e.g, spitz, nevus spilus, etc), (4) Dysplastic nevi and the dysplastic nevus syndrome and (5) Dermal melanocytic nevi. Congenital 0 melanocytic naevi (CMN) 0 are nevomelanocytic nevi that are present at birth.[3] Some CMN may not be apparent at birth because they lack visible pigment; however they may slowly develop pigment over time and become visible. Those nevi with congenital features in which the history of their presence since birth cannot be verified are termed congenital-naevus-like naevi (CNLN) (e.g. satellite naevi seen with CMN).[3] CMN usually present as round to oval, fairly homogenous, brown, multishaded pigmented lesions with sharply demarcated border often with a mamillated surface and hypertrichosis. On the basis of size (i.e. the greatest diameter), they are categorized as small (<1.5 cm), intermediate/medium (1.5 to 20 cm) and giant (>20 cm) nevi. Between 1 and 6% of infants are born with a CMN and between 2 and 6% of the population have a CNLN.[3],[4]
Congenital nevi are found to be much larger in diameter than acquired nevi. Most congenital nevi are at least 5 mm in diameter even at birth whereas the majority of acquired nevi never grow to more than 5 mm in diameter.[3] The acquired nevi are benign proliferations of melanocytic naevus cells which develop from birth.[5] The proliferation occur initially within the epidermis at the dermoepidermal junction and therefore, early melanocytic naevi are known as junctional nevi, subsequently the nevus cells 'drop down' into the dermis and ultimately show evidence of maturation of the melanocytes within the dermis or differentiation phenotypically towards neural cells. When the nevus cells exclusively exist within the dermis, they are known as dermal cellular nevi. When they are junctional and in dermis, they are called compound naevi.[5] Acquired melanocytic nevi are almost universal in white people and common in Asian / Hispanic skin.[6] Dermal melanocytic nevi are believed to arise from dermal melanocytes that have become arrested in the dermis during fetal life and tissue remodeling and have never reached their normal site in the basal layer.[7] The colour of these lesions is blue because of the optical effects of light reflecting on melanin deep in the dermis.[5] Mongolian spots are dermal melanocytic nevi which present with macular blue-grey pigmentation at birth on the sacral area in normal infants or darker-skinned races.[7] An extensive, blue, patch-like area of dermal melanocytic pigmentation of the sclera and the skin adjacent to the eye due to the presence of dermal melanocytes characterizes nevus of Ota. Unlike the Mongolian spot, it is not usually present at birth, becomes progressively darker in childhood and persists in adult life. Nevus of Ito is also a type of dermal melanocytosis which involves the acromioclavicular region and upper chest and like Ota's nevus is largely confined to the Japanese.[7]
Since, there is lack of studies on incidence and pattern of melanocytic nevi in children particularly in India, this study was undertaken.
| Materials and Methods | | |
In this descriptive study children attending the skin OPD, JIPMER were screened for melanocytic nevi (below the age of 14 years). Children with pigmentary disorders not qualifying for melanocytic nevi were excluded. The clinical findings were recorded in a proforma. The various parameters noted were age, sex, age of onset, reason for attending hospital, family history, duration of nevi, site of involvement, morphology of lesions, number of lesions and associated findings. In addition to the presenting symptoms, Wood's lamp examination, skin biopsy were done wherever necessary. Necessary relevant investigations were carried out for any systemic associations. The results obtained were tabulated and analysed.
| Results | | |
A total of 4,256 pediatric cases attended the dermatology OPD, JIPMER during the study period from August 2002 to August 2004. Out of these, 41 (0.96%) cases were found to have melanocytic nevi. The mean age of these cases was 1.4 years (range of 3 days to 14 years). Fourteen (34.1%) of them were males and 27 (65.9%) were females with male to female ratio of 1:1.9. Majority of these cases (32 cases, 78%) were in the age group of 0-1 year followed by 5 (12.2%) cases in the age group of 1-5 years, 3 (7.3%) cases in the age group of 10-14 years and 1 (2.4%) case in the age group of 6-9 years [Table - 1].
Family history could not be elicited in any of our cases. Out of a total of 41 cases with melanocytic nevi, 37 (90.2%) cases had single lesion and 4 (9.8%) cases had multiple lesions. Thirty three (80.5%) cases were centrally distributed (midline in the back), 5 (12.2%) cases were located on the left side and 3 (7.3%) cases on the right side of the body. The size of the nevi varied from 1.5 cm to 20 cm in 40 (97.6%) cases and more than 20 cm in 1 (2.4%) case. The most common site of involvement was the back (32 cases, 78%) followed by head and neck (6 cases, 14.6%), chest (1 case, 2.4%) and upper limbs (1 case, 2.4%). Multiple sites involvement was noted in one (2.4%) case. Satellite lesions were observed in one (2.4%) case. Thirty nine (95.1%) were stationary, while 2 (4.9%) of them were gradually increasing in size. Thirty (73.2%) cases were blue in colour, 9 (22%) were black and 2 (4.9%) cases were bluish black. Hair growth over the lesion was seen in 4 (9.8%) cases whereas 37 (90.2%) cases had no hair growth.
Out of the total of 41 cases, 30 (73.2%) were Mongolian spots (circumscribed or oval in shape), 8 (19.5%) were congenital melanocytic nevi, one (2.4%) each had giant congenital melanocytic nevi, naevus spilus and nevus of Ota [Table - 2].
Four cases had other associated cutaneous disorders. These included ashleaf macules in 2 cases, epidermolysis bullosa simplex in 1 case and a solitary case had both cafι-au-lait macule and mixed hemangioma. The systemic associations included seizures (suspected neurocutaneous melanosis) in a solitary case of congenital melanocytic nevi. The nevi were complicated by nodularity in 2 cases.
Mongolian spots
In our study of 41 cases of melanocytic nevi, 30 (73.2%) were of Mongolian spots. The mean age of cases with Mongolian spots was 7.9 days with a range of 3 days to 20 days. The age of onset of all these nevi was at birth. Except one case, remaining 29 cases were single. All the lesions of Mongolian spots were blue in colour. The majority of them were circumscribed or oval with the diameter of the lesions varying from 1.5 to 10cms. All 30 (100%) of the cases of Mongolian spots were on the lumbosacral area.
| Discussion | | |
In a study of pediatric dermatoses in a referral centre in South India, out of a total of 24 cases with nevi, 7 (29.2%) cases were found to have melanocytic nevi.[8] Amongst these melanocytic nevi, 3 had acquired melanocytic nevi, 2 had congenital melanocytic naevi and a solitary case each had nevus of Ota and Mongolian spot. In our study, out of a total of 41 (53.2%) cases of melanocytic nevi, 30 (73.2%) were Mongolian spots, 8 (19.5%) were congenital melanocytic naevi and a solitary case (2.4%) each had giant congenital melanocytic nevi, nevus spilus and nevus of Ota. In a study of melanocytic naevi in 211 school children in Queensland, there were 119 (56%) boys and 92 (44%) girls in the final sample and the majority (92%) were 8 to 10 years old. Thus boys had significantly more melanocytic nevi than girls.[9] In our study of 41 children (less than 14 years) of melanocytic nevi, 14 (34.1%) of them were males and 27 (64.9%) were females with male to female ratio of 1:1.9. The mean age of these cases was 1.4 years (range of 3 days to 14 years). Majority of these cases (32 cases, 78%) were in the age group of 0-1 year. Thus in our study in contrast to the above study, the majority of the melanocytic nevi occurred in females and they were much more common in infancy than in older children (majority being Mongolian spots).
It is being observed that between 1% and 6% of infants are born with a congenital melanocytic nevi (CMN) and between 2% and 6% of the population have a Congenital Naevus Like Nevi (CNLN).[3],[4] In our study of 41 cases of melanocytic nevi, 9 (22.0%) cases were found to have congenital melanocytic nevi. Although, there are reported cases of familial clustering of CMN, yet most cases are sporadic.[3] In our study, all 9 cases had no family history of CMN. Thus all our cases had occurred sporadically. In a large study of prevalence of congenital nevus like nevi, CNLN tended to spare the head and neck and upper extremities.[10] But in another study in Eastern Australia, it was found that CNLN were distributed on sun-exposed sites.[11] In our study of 9 cases, the most common site of involvement was head and neck (4 cases), followed by back (3 cases) and one case each had involvement of upper limb and multiple sites. Hence in our study, there was no sparing of head and neck and both sun exposed and non exposed sites were involved.
On the basis of size (i.e. the greatest diameter), congenital melanocytic nevi are categorized as small (<1.5 cm), intermediate / medium (1.5 to 20 cm) and giant (>20 cm) nevi.[5] Most congenital nevi are at least 5 mm in diameter even at birth whereas the majority of acquired naevi never grow to more than 5 mm in diameter. Rarely, giant nevi occur covering large areas of the body. Castilla et al[12] examined 500,000 infants in South America and found only one case at least 10 cm in diameter per 20,495 subjects. In our study, out of 9 cases of CMN, only one case had lesion larger than 20 cm (i.e. giant CMN) and remaining were of 1.5 to 20 cm in diameter. The majority of our cases of CMN were more than 5 mm in diameter. Thus, the size of the nevi in our study is comparable to above studies. Small and medium sized CMN usually grow less rapidly than the infant so that their surface area becomes relatively less. But in a review of CMN, it was stated that except during early infancy when some CMN can grow rapidly, most grow in proportion to the growth of the child.[3] In our study, out of 9 cases of CMN only 2 were progressive and all others were stationary. Thus majority of the CMN in our study had fewer tendencies to spread. These giant CMN may be accompanied in some individuals by many small congenital nevi. Such so called satellite nevi were reported in 74% of one series.[3] In our series also, satellite lesions were observed in a single case of giant CMN. With the passage of time, CMN may get darker; lighter or loose pigmentation and may become more heterogenous.[3] In our series of 9 cases, except one case which is bluish black in colour, all others were black in colour. In small and medium sized nevi, there is frequently a striking increase not only in pigmentation but also in hair growth at puberty. In giant CMN, the hairy component, which occurs in 95% of lesions, tends to become more prominent in late childhood.[7] In our study of 9 cases, 4 cases had hair growth and all remaining had no hypertrichosis. Amongst these 4 cases of CMN, a three-year-old female child with giant CMN had hair growth almost covering all of the lesions. Thus in this baby, hypertrichosis had occurred in much early period of her life. Malignant degeneration of large CMN may be associated with a relatively sudden appearance of dermal or subcutaneous nodules, very dark pigmentation, itching, pain, bleeding or ulceration. [13] In our study, 2 cases were complicated by nodularity suggesting a possibility of melanoma.
Neurocutaneous melanosis is a rare congenital syndrome characterized by the presence of large or multiple CMN and benign or malignant pigment cell tumours of the leptomeninges.[14] In one study of neurocutaneous melanosis, it was found that with large or multiple nevi on the head and neck (94%) or on posterior midline (96%) or a giant CMN (64%) may have an increased risk of leptomeningeal melanosis detectable by MRI-Gd.[14] In our study, a single case of CMN with a size 6 x 5 cm over the left sacral region had seizures and hence suspected to have neurocutaneous melanosis. The cutaneous associations of CMN include vascular nevi, epidermal nevus syndrome, Carney's syndrome, LAMB syndrome, premature ageing syndrome, etc. In our study of 9 CMN cases, the cutaneous associations included ashleaf macules, cafι-au-lait macule and mixed hemangioma.
Mongolian spots are macular blue-grey pigmentation present at birth on the sacral area in normal infants or darker-skinned races.[7] In the series reported from Oklahoma city, Mongolian spots were found in 26% of 830 babies.[15] In our study of 41 cases of melanocytic naevi, 30 (73.2%) were Mongolian spots. Usually the most common site of involvement is the sacrum and lower back. Other rarer sites involved are buttocks, flanks or even shoulder which may be affected in extensive lesions.[7] In our study, all 30 (100%) cases were on the lumbosacral area. Thus, majority of the lesions were on lower back. The Mongolian spots usually appear as blue coloured macules but skin is otherwise normal.[7] In our study also, all cases of Mongolian spots were blue in colour. The lesions of Mongolian spots are usually single but occasionally multiple.[7] In our study, except one case, remaining 29 cases were single. The patches are usually rounded or oval in shape, up to 10 cm or so in diameter. In our study also, the majority were circumscribed or oval with the largest diameter of the lesion varying from 1.5 to 10 cm. The pigmentation in Mongolian spots appears in fetal life, increases in depth for a period after birth and then diminishes. It usually disappears during the first decade but has occasionally persisted into adult life.[7] In our study, the mean age of onset of Mongolian spots was 7.9 days with a range of 3 days to 20 days. Thus, in our study, the Mongolian spots were much more common in neonates which holds true as with other studies. Thus, this study highlights the spectrum of melanocytic nevi seen in children and the nature of complications encountered in them.
| References | | |
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| 6. | Harrison SL, Buettner PG, Machennan R. Body-site distribution of melanocytic nevi in young Australian children. Arch Dermatol 1999; 135: 47-52. |
| 7. | Mackie RM. Melanocytic naevi and malignant melanomas. In: Champion RH, Burton JL, Burns DA, Breathnach SM, eds, Rook / Wilkinson / Ebling Textbook of Dermatology, Vol.2. 6th edn. London: Blackwell Science Limited 1998; 1717-52. |
| 8. | Karthikeyan K, Thappa DM, Jeevankumar B. Pattern of pediatric dermatoses in a referral center in South India. Indian Pediatr 2004; 41: 373-77. [PUBMED] [FULLTEXT] |
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| 11. | Rivers JK, Marhennan R, Kelly JW, Lewis AE, Tate BJ, Harrison S, McCarthy WH. The Eastern Australian childhood nevus study: Prevalence of atypical naevi, congenital nevus - like naevi and other pigmented lesions. J Am Acad Dermatol 1995; 32: 957-63. |
| 12. | Castilla EE, Dutra MDG, Orioti - Parreiras IM. Epidemiology of congenital pigmented naevi. Incidence rates and relative frequencies. Br J Dermatol 1981; 104: 307-15. |
| 13. | Grichnik JM, Rhodes AR, Solier AJ Benign Hyperplasias and Neoplasias of Melanocytes. In: Freedberg IM, Eisen AZ, Wolff K, Austen KF, Goldsmith LA, Katz SI, eds, Fitzpatrick's Dermatology in General Medicine, Vol.1. 6th edn, New York: McGraw Hill 2003; 881 - 905. |
| 14. | Kadonaga JN, Frieden IJ. Neurocutaneous melanosis: Definition and review of literature. J Am Acad Dermatol 1991; 24: 747-55. [PUBMED] |
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Tables
[Table - 1], [Table - 2] |
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