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Year : 2013  |  Volume : 4  |  Issue : 2  |  Page : 148-152  

Hyperpigmentary disorders in children: A hospital-based study in a tertiary care center

Department of Dermatology and STD, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, India

Date of Web Publication17-Apr-2013

Correspondence Address:
Devinder Mohan Thappa
Department of Dermatology and STD, The Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry - 605 006
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2229-5178.110641

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How to cite this article:
Sori T, Jaisankar T J, Thappa DM, Nath AK. Hyperpigmentary disorders in children: A hospital-based study in a tertiary care center. Indian Dermatol Online J 2013;4:148-52

How to cite this URL:
Sori T, Jaisankar T J, Thappa DM, Nath AK. Hyperpigmentary disorders in children: A hospital-based study in a tertiary care center. Indian Dermatol Online J [serial online] 2013 [cited 2022 Jan 23];4:148-52. Available from: https://www.idoj.in/text.asp?2013/4/2/148/110641


Pigmentary disorders encompass a commonly encountered group of dermatoses in the pediatric age group. Presence of hyperpigmented skin lesions in children, besides being an insignificant or isolated finding, may be indicative of more serious underlying systemic or genetic disorder. We carried out a study in our dermatology out patient department (OPD) over a period of 22 months (August 2007-June 2009) with the objective of evaluating the pattern of hyperpigmentary disorders in children with an emphasis on whether the skin changes were early markers of any systemic or genetic disorder.

There were 58 children (32 boys, and 26 girls) in our study. A total of 34392 children attended the Dermatology OPD during the study period. The frequency of hyperpigmentary disorders was 1.54 per 1000 children. The mean age of the children was 6.89 years and mean of age of onset was 3.89 years (birth to 13 years). Twenty six out of 58 (44.8%) children had onset at birth. The mean duration of the disorders was 3 years (range of 1 day to 14 years).

The most common hyperpigmentary disorder in our study was cafι-au-lait macule (CALM) [Figure 1] seen in 12 children (20.6%), followed by post-inflammatory hyperpigmentation (9/58, 15.5%), pigmentary mosaicism (8/58, 13.8%) [Figure 2] and [Figure 3], congenital melanocytic nevus (7/58, 12%), lichen planus (5/58, 8.6%), Mongolian spots (4/58, 6.8%) [Figure 4] and fixed drug eruption (FDE) (3/58, 5.2%) [Figure 5]. Two (3.4%) children each had urticaria pigmentosa [Figure 6] and Becker's nevus [Figure 7]. One child each (1.7%) had Chιdiak-Higashi Syndrome (CHS), discoid lupus erythematosus, incontinentia pigmenti [Figure 8], lentigines [Figure 9], lichen striatus, nevus of Ota, pityriasis rubra pilaris and speckled lentiginous nevus. [Table 1] shows the hyperpigmentary disorders (with systemic associations) noted in our study. Chest and lower limb were the most commonly affected sites (16.2%), followed by face (15.3%), upper limb (11.7%), back (9.9%), and abdomen (8.1%). Parental consanguinity was present in 19 (32.7%) cases (2 nd consanguinity-10, 3 rd consanguinity-9). Delayed milestones were observed in three children.
Table 1: Hyperpigmentary disorders noted in our study

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Figure 1: Café-au-lait macule over the abdomen

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Figure 2: Pigmentary mosaicism affecting one side of the face

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Figure 3: Pigmentary mosaicism affecting the trunk

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Figure 4: Multiple Mongolian spots in Hurler's syndrome baby

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Figure 5: Fixed drug eruption lesions over the trunk

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Figure 6: Pigmented lesions in urticaria pigmentosa demonstrating Darier's sign

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Figure 7: Becker's naevus in beard area of face

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Figure 8: Chinese letter pattern pigmentation over the abdomen in incontinentia pigmenti

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Figure 9: Lentigenes affecting the face

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The following systemic features were noted in our patients: (a) Hurler's syndrome - corneal haziness, flattened nasal bridge, developmental delay, left atrial and left ventricular hypertrophy with prolapsed aortic valve and mild to moderate mitral regurgitation, abdominal distension due to hepatosplenomegaly and hypertrichosis over the upper limb, shoulder, back and face. (b) Mucopolysaccharidoses - corneal haziness, developmental delay, mild left ventricular hypertrophy, wide-open anterior fontanelle with enlarged calvarium and minimal widening on skull X-ray, hepatosplenomegaly, hypertrichosis over the upper limb, shoulder, back and face. (c) Centofacial lentiginosis [Figure 9] - hypertelorism. (d) CHS - recurrent upper respiratory tract infection, recurrent diarrhoea. (e) Tuberous sclerosis complex - seizures. (f) McCune-Albright syndrome - short stature. (g) Incontinentia pigmenti - spastic diplegia. (h) One child with congenital melanocytic nevus had limb length discrepancy (length of left leg 3 cm more than right leg), hypertrophy of thigh and lymphatic malformation on Doppler study of affected limb.

In our study, CALMs occurred in 7.2% of all children with all pigmentary disorders in the study and in 20.6% of children with hyperpigmentary lesions. The frequency of CALM in Osburn, et al. [1] study was 2.8% among all the infants included in the study, and 9.2% among infants with pigmented lesions. In a McLeean and Gallagher [2] study prevalence of CALM was more than 25% in three ethnic groups. It was isolated finding in 10 children whereas two patients had syndromic associations (Tuberous Sclerosis Complex and McCune-Albright Syndrome). Father of one child with 20 macules had neurofibromatosis.

Post inflammatory hyperpigmentation (PIH) was a very common reason for patient visits. It commonly follows tinea infection, chronic bullous dermatosis of childhood, pyodermas, lichen planus, insect bite reactions, etc. In most of the cases, site and pattern of hyperpigmentation give you the clue to the antecedent dermatosis.

Congenital melanocytic nevi (CMN) occurred in 12% (7 children) in our study. Most children (4/7) had intermediate sized nevi. Giant "bathing-trunk" CMN was seen in one child, and small CMN was seen in 2 children. Lesions were seen on the face (2), lower limbs (2), back (2) and upper limbs (1). In Ingordo, et al. [3] study 80.3% of CMN were medium-sized, and 19.7% were large-sized. Majority of CMN were located in the lumbar area, followed by the back and face.

Drug reactions are believed to be uncommon in children. We noted 3 cases of hyperpigmentation due to past FDE (no mucosal involvement). The incriminated drugs were paracetamol and co-trimoxozole.

CHS was seen in a 2-year-old girl who had generalized hypopigmentation at birth and later developed slate-gray hyperpigmentation all over the body sparing the axillae, neck, popliteal fossae, and inguinal region. The hyperpigmentation was preceded by topical application of native preparation followed by sun-exposure. Hair was light coloured. Child also had developmental delay, recurrent fever, cough, breathing difficulties, cyanosis, hepatosplenomegaly, diarrhea, photophobia, and bleeding manifestations. Peripheral smear showed large granules in the neutrophils, confirming the clinical diagnosis. Hair light microscopy showed pigmentary dilution with clumping of melanin pigment within the hair shaft. Pigmentary dilution in the skin is the norm in CHS, but hyperpigementation has been reported by Al-Khenaizan [4] in a 4΍-year-old Saudi boy who had speckled hypopigmentation and hyperpigmentation due to repeated sun-exposure.

To conclude, it can be said that a number of hyperpigmented skin lesions could be indicative of more serious underlying systemic or genetic disorders, e.g., CALMs (in association with neurofibromatosis, tuberous sclerosis, and McCune-Albright Syndrome), mosaicism (with skeletal and neurological abnormalities), CHS, Hurler's syndrome, mucopolysaccharidoses etc., unlike benign and common conditions like post inflammatory hyperpigmentation.

   References Top

1.Osburn K, Schosser RH, Everett MA. Congenital pigmented and vascular lesions in newborn infants. J Am Acad Dermatol 1987;16:788-92.  Back to cited text no. 1
2.McLean DI, Gallagher RP. "Sunburn" freckles, café-au-lait macules, and other pigmented lesions of schoolchildren: The vancouver mole study. J Am Acad Dermatol 1995;32:565-70.  Back to cited text no. 2
3.Ingordo V, Gentile C, Iannazzone SS, Cusano F, Naldi L. Congenital melanocytic nevus: An epidemiologic study in Italy. Dermatology 2007;214:227-30.  Back to cited text no. 3
4.Al-Khenaizan S. Hyperpigmentation in Chediak-Higashi syndrome. J Am Acad Dermatol 2003;49:S244-6.  Back to cited text no. 4


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9]

  [Table 1]

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