Clinical Profile of Children with Pigmentary Disorders Accepted: 08-12-2019
Total Page:16
File Type:pdf, Size:1020Kb
International Journal of Dermatology, Venereology and Leprosy Sciences. 2020; 3(1): 08-13 E-ISSN: 2664-942X P-ISSN: 2664-9411 Original Article www.dermatologypaper.com/ Derma 2020; 3(1): 08-13 Received: 06-11-2019 Clinical profile of children with pigmentary disorders Accepted: 08-12-2019 Dr. Sori Tukaram Dr. Sori Tukaram, Dr. Dyavannavar Veeresh V, Dr. TJ Jaisankar and Assistant Professor, Department of Skin & STD, Dr. Thappa DM GIMS, Gadag, Karnataka, India DOI: https://doi.org/10.33545/26649411.2020.v3.i1a.31 Dr. Dyavannavar Veeresh V Abstract Associate Professor, Pigmentary disorders are believed to be the commonest group of dermatoses in pediatric age group [6]. Department of Skin & STD, but, there is a dearth of adequate data regarding the frequency and pattern of different types of GIMS, Gadag, Karnataka, India pigmentary disorders in children. Any deviation from the normal pattern of pigmentation results in significant concerns in the affected individual. Even, relatively minor pathologic pigmentary changes Dr. TJ Jaisankar can cause children to become pariahs in their community. This study was a descriptive study spanning Professor, Department of Skin over a period of 23 months. Institute ethics committee clearance was obtained. All children attending & STD, GIMS, JIPMER, the Dermatology out Patient Department (OPD) (6 days in a week) were screened for any cutaneous Pondicherry, India pigmentary lesions. Children (up to 14 years of age) with pigmentary disorders were included in the study after getting informed consent from the parents/ guardians. Out of 167 children, 53 (31.7%) had Dr. Thappa DM hyperpigmentation lesions only, whereas 108 (64.7%) had hypopigmentary lesions only. Six children Professor, Department of Skin had presence of both hyper- and hypopigmentary lesions. Five children (2.9%) had overlap of unrelated & STD, GIMS, JIPMER, hyper- and hypopigmentary lesions. Only one child (dyschromatosis universalis hereditaria) had ‘true’ Pondicherry, India combination of hyper- and hypopigmentary lesions, the presence of both of were together were required for the diagnosis. The pigmentary disorders in our study had characteristic features in most children, while in some the features noted were rather unique. Keywords: Clinical profile, children, pigmentary disorders Introduction Physiologically, human skin presents a unique color. Skin color varies from individual to individual, and in an individual, variation in the degree of pigmentation occurs in various regions of the body. Normal skin color is dependent on haemoglobin (in the oxygenated and reduced state), carotenoids and melanin pigment. Melanin is the major determinant of skin color, and racial and ethnic differences in skin color are related to the number, size, shape, distribution of melanin-laden organelles called melanosomes. Melanocyte is the sole site of melanin synthesis. Eumelanin and pheomelanin are the two major types of melanin; they impart brown-black and yellow-red color, respectively. Melanin synthesis can be induced by a number of factors; hence skin pigmentation is classified as constitutive and facultative. The constitutive skin color refers to the “baseline” genetically determined color in the absence of sun exposure and other influences. The facultative (inducible) skin color is due to pigmentary darkening after secondary melanin production which can result from sun exposure and endocrine causes. Skin pigmentation is the body’s main defence against [1] ultraviolet rays of sunlight . Skin color is an important visible sociocultural characteristic of an individual. Hence any deviation from the normal pattern of pigmentation results in significant concerns in the affected individual. Even, relatively minor pathologic pigmentary changes can cause children to become pariahs in their community. Pigmentary disturbances include decrease or absence or increase in pigmentation which could be either epidermal or dermal. Pigmentary disorders Corresponding Author: are a group of dermatoses characterized by alteration of the normal pattern of pigmentation Dr. Dyavannavar Veeresh V in a localized (circumscribed), or generalized distribution, and may be congenital or acquired Associate Professor, in origin [2]. Pigmentary disorders may be hypopigmented (hypomelanotic) or Department of Skin & STD, GIMS, Gadag, Karnataka, hyperpigmented (hypermelanotic) or both hypo- and hyperpigmented. Many of the India pigmentary disturbances are present at birth (congenital melanocytic nevus, Mongolian ~ 8 ~ International Journal of Dermatology, Venereology and Leprosy Sciences www.dermatologypaper.com spots, hypomelanotic macules of tuberous sclerosis children with hyperpigmentation lesions was counted as 58, complex, cutaneous mosaicism), and parents’ of such and that with hypopigmentary lesions as 113. children usually become very much concerned about the Dyschromatosis universalis hereditaria is considered pigmentary disturbances and seek medical advice regarding separately. the future course of the disorder [2, 3]. Pigmentary disorders are believed to be the commonest Age group of dermatoses in pediatric age group. But, there is a The mean age of 167 children in the study was 7.1 years, dearth of adequate data regarding the frequency and pattern ranging from 3 days to 14 years with a median of 8 years of different types of pigmentary disorders in children [4]. (SD-4.57, SEM-0.35). The mean age of children with Hence, this study is being undertaken to find out the pattern hyperpigmentation disorders was 6.89 years, and that in of pigmentary disorders in children. hypopigmentary disorders was 7.2 years. Methodology Gender This study was a descriptive study spanning over a period of There were 93 boys (55.7%) and 74 girls (44.3%) in our 23 months. Institute ethics committee clearance was study, with a male to female ratio of 1.25:1. The mean age obtained. All children attending the Dermatology out Patient of boys was 7.84 years, with a range of 3 days to 14 years Department (OPD) (6 days in a week) were screened for any and median of 8 years (SD-4.64, SEM-0.48). The mean age cutaneous pigmentary lesions. Children (up to 14 years of of the girls was 6.35 years, with a range of 45 days to 14 age) with pigmentary disorders were included in the study years and median of 6.5 years (SD-4.37, SEM-0.50). Out of after getting informed consent from the parents/ guardians. 93 boys, 31 (33.3%) had hyperpigmentation disorders and Children more than 14 years of age, and when 60 (64.5%) had hypopigmentary disorders, whereas two parents/guardians (of children less than 14 years of age) are (2.1%) boys had both. Out of 74 girls, 22 (29.7%) had not consenting for participation in the study were excluded. hyperpigmentation disorders and 48 (64.8%) had A detailed clinical history was elicited with regard to hypopigmentary disorders, whereas four (5.4%) girls had patients’ age, gender, address, age of onset of disorder, type both. (hypo-/hyper pigmented), site and size of pigmented lesions, familial involvement, associated skin and systemic Age and Gender Distribution conditions and the details were recorded on a proforma. In our study, most common age group was 11-14 years Detailed examination of the pigmentary lesion/lesions was contributing to 51 out of 167 (30.5%) children, followed by done and findings were noted in terms of site, size, shape, 6-10 years contributing to 49 out of 168 (29.34%). Thirty morphology of lesions etc. General physical examination four out of 93 boys were in the 11-14 years age group, was done to see any associated cutaneous or systemic whereas 28 out of 74 girls were in the 6-10 years age group. involvement and the relevant findings were recorded. Table 1. Age and gender distribution of all children Results Age groups (years) Boys Girls Total A total of 34392 children attended the Dermatology OPD <1 13 7 20 during the study period. Thus the frequency of pigmentary 1-5 26 22 48 disorders among children attending the Dermatology OPD 6-10 20 28 49 was 4.85 per 1000 children. 11-14 34 17 51 Out of 167 children, 53 (31.7%) had hyperpigmentation Total 93 74 167 lesions only, whereas 108 (64.7%) had hypopigmentary lesions only. Six children had presence of both hyper- and Weight and Height hypopigmentary lesions. Five children (2.9%) had overlap The mean weight of all children was 22.27 kg (SD-12.78, of unrelated hyper-and hypopigmentary lesions. Only one SEM-0.98), ranging from 2.7 kg to 78 kg, and median of 22. child (dyschromatosis universalis hereditaria) had ‘true’ The mean height of all children was 110.07 cm (SD-30.93, combination of hyper-and hypopigmentary lesions, the SEM-2.4), ranging from 44 cm to 168 cm and median of presence of both of were together were required for the 119 cm. diagnosis. Thus, for the ease of analysis, total number of Table 2: Weight and height characteristics in hyper-and hypopigmentary disorders Parameters Pigmentary disorder No. of children Mean Median Min Max SD SEM Hyperpigmentation 58 21.179 20.000 3.5 78.0 14.580 1.9145 Weight (Kg) Hypopigmentary 113 22.34 22.00 3 55 11.602 1.09 Hyperpigmentation 58 107.43 112.00 42 168 32.231 4.232 Height (cm) Hypopigmentary 113 112.48 120.00 44 162 28.61 2.6 There were 58 children (34.7%) with hyperpigmentation melanocytic nevus (7/58, 12%), lichen planus (5/58, 8.6%), disorders including 32 boys (55.2%) and 26 girls (44.8%). Mongolian spots (4/58, 6.8%) and fixed drug eruption (3/58, The mean age of children with hyperpigmentation disorders 5.2%). Two (3.4%) children each had urticaria pigmentosa was 6.89 years. and Becker’s nevus. One child each (1.7%) had Chediak- Most common hyperpigmentation disorder in our study was Higashi syndrome (CHS), discoid lupus erythematosus, café-au-lait macule seen in 12 children (20.6%), followed incontinentia pigmenti (IP), lentigines, lichen striatus, nevus by post-inflammatory hyperpigmentation (9/58, 15.5%), of Ota, pityriasis rubra pilaris and speckled lentiginous pigmentary mosaicism (8/58, 13.8%), congenital nevus.