Letters to the Editor released into the extracellular environment these stress proteins are very immunogenic, facilitating the presentation of other Hyperpigmentary disorders antigens to dendritic cells and eliciting an immune response.[2] in children: A hospital‑based Koebnerization of following radiotherapy is rare and fewer than 15 reports have been described in literature including one study in a tertiary care from India.[3] Most of the reports describe depigmentation limited to areas of irradiation. There are only two reports of disseminated center vitiligo developing after radiotherapy. However, in both the cases, there was no prior history of vitiligo. Polat, et al.[4] reported a Sir, case of disseminated vitiligo developing after radiotherapy in Pigmentary disorders encompass a commonly encountered a case of nasopharyngeal carcinoma, with no prior history of group of dermatoses in the pediatric age group. Presence [5] vitiligo. Abood, et al. reported a case of , with no of hyperpigmented skin lesions in children, besides being prior history, developing generalized vitiligo after radiotherapy. an insignificant or isolated finding, may be indicative of more serious underlying systemic or genetic disorder. We carried The mechanism of development of disseminated depigmentation out a study in our dermatology out patient department (OPD) is not known. A “two‑hit” hypothesis has been proposed. over a period of 22 months (August 2007‑June 2009) with the Localized radiotherapy leads to increased oxidative stress thereby triggering the process of haptenation and resulting in objective of evaluating the pattern of hyperpigmentary disorders the formation of highly immunogenic neoantigens. This leads in children with an emphasis on whether the skin changes were to systemic autoimmune response to resulting in early markers of any systemic or genetic disorder. sites of depigmentation distant to the irradiated field.[5] There were 58 children (32 boys, and 26 girls) in our study. Thus, we present a rare case of radiotherapy induced A total of 34392 children attended the Dermatology OPD during koebnerization and dissemination of vitiligo. Patients should be the study period. The frequency of hyperpigmentary disorders made aware of such a possibility and appropriately counseled. 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 Swapnil A. Sanghavi, Atul M. Dongre, Uday S. Khopkar 13 years). Twenty six out of 58 (44.8%) children had onset at Department of Skin and VD, Seth G S Medical College and birth. The mean duration of the disorders was 3 years (range KEM Hospital, Parel, Mumbai, Maharashtra, India of 1 day to 14 years). Address for correspondence: Dr. Swapnil Sanghavi, Department of Skin and VD, Seth G S Medical College and The most common hyperpigmentary disorder in our KEM Hospital, Parel, Mumbai ‑ 400 012, India. study was café‑au‑lait macule (CALM) [Figure 1] seen E‑mail: [email protected] in 12 children (20.6%), followed by post‑inflammatory REFERENCES (9/58, 15.5%), pigmentary mosaicism (8/58, 13.8%) [Figures 2 and 3], congenital melanocytic 1. Njoo MD, Westerhof W. Vitiligo. Pathogenesis and treatment. Am J Clin Dermatol 2001;2:167‑81. 2. Weiss G, Shemer A, Trau H. The Koebner phenomenon: Review of the literature. J Eur Acad Dermatol Venereol 2002;16:241‑8. 3. Munshi A, Jain S, Budrukkar A, Jalali R, Sarin R. Radiotherapy‑induced depigmentation in a patient with breast cancer. Indian J Cancer 2007;44:157‑8. 4. Polat M, Yalçin B, Alli N. Vitiligo at the site of radiotherapy for nasopharyngeal carcinoma. Am J Clin Dermatol 2007;8:247‑9. 5. Daneshpazhooh M, Shokoohi A, Dadban A, Raafat J. The course of melanoma‑associated vitiligo: Report of a case. Melanoma Res 2006;16:371‑3.

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

148 Indian Dermatology Online Journal- April-June 2013 - Volume 4 - Issue 2 Letters to the Editor nevus (7/58, 12%), (5/58, 8.6%), Mongolian and hypertrichosis over the upper limb, shoulder, back spots (4/58, 6.8%) [Figure 4] and fixed drug eruption (FDE) and face. (b) Mucopolysaccharidoses – corneal haziness, (3/58, 5.2%) [Figure 5]. Two (3.4%) children each had urticaria developmental delay, mild left ventricular hypertrophy, pigmentosa [Figure 6] and Becker’s nevus [Figure 7]. One child wide‑open anterior fontanelle with enlarged calvarium and each (1.7%) had Chédiak‑Higashi Syndrome (CHS), discoid minimal widening on skull X‑ray, hepatosplenomegaly, lupus erythematosus, [Figure 8], hypertrichosis over the upper limb, shoulder, back and lentigines [Figure 9], lichen striatus, nevus of Ota, pityriasis face. (c) Centofacial [Figure 9] – hypertelorism. rubra pilaris and speckled lentiginous nevus. Table 1 (d) CHS – recurrent upper respiratory tract infection, recurrent shows the hyperpigmentary disorders (with systemic diarrhoea. (e) Tuberous sclerosis complex – seizures. associations) noted in our study. Chest and lower limb (f) McCune‑Albright syndrome – short stature. (g) Incontinentia were the most commonly affected sites (16.2%), followed pigmenti – spastic diplegia. (h) One child with congenital by face (15.3%), upper limb (11.7%), back (9.9%), and melanocytic nevus had limb length discrepancy (length of left abdomen (8.1%). Parental consanguinity was present in leg 3 cm more than right leg), hypertrophy of thigh and lymphatic 19 (32.7%) cases (2nd consanguinity‑10, 3rd consanguinity‑9). malformation on Doppler study of affected limb. Delayed milestones were observed in three children. In our study, CALMs occurred in 7.2% of all children with all The following systemic features were noted in our patients: pigmentary disorders in the study and in 20.6% of children with (a) Hurler’s syndrome – corneal haziness, flattened nasal bridge, hyperpigmentary lesions. The frequency of CALM in Osburn, developmental delay, left atrial and left ventricular hypertrophy et al.[1] study was 2.8% among all the infants included in the study, with prolapsed aortic valve and mild to moderate mitral and 9.2% among infants with pigmented lesions. In a McLeean regurgitation, abdominal distension due to hepatosplenomegaly and Gallagher[2] study prevalence of CALM was more than 25%

Table 1: Hyperpigmentary disorders noted in our study Hyperpigmentary No. of Associations/comments disorders children (%) Café au lait macule 12 (20.6) Isolated CALM‑4, atopic diathesis‑2, tuberous sclerosis complex‑1, McCune‑Albright syndrome‑1, nevus of Ota and ‑1, psoriasis/lichen sclerosus et atrophicus/congenital melanocytic nevus‑1 each Post‑inflammatory 9 (15.5) Secondary to tinea infections‑8, chronic bullous disease of childhood‑1 hyperpigmentation Pigmentary mosaicism 9 (15.5) Linear and whorled nevoid hypermelanosis‑3, segmental pigmentation‑3, linear nevoid hyperpigmentation‑2, unilateral pigmentation‑1 Congenital melanocytic 7 (12) Giant “bathing‑trunk” nevus (>20 cm)‑1, intermediate size‑4, small nevus (<1.5 cm)‑2 nevus Lichen planus 5 (8.6) Classical LP‑3, hypertrophic LP‑1 (with associated obesity, alopecia areata and ), lichen planus pigmentosus‑1 Mongolian spots 4 (6.8) Present since birth in all. Single lesions in boys,[2] multiple (>20) lesions both girls. One girl had mucopolysaccharidosis and other girl had Hurler syndrome. One boy had congenital melanocytic nevus Fixed drug eruption 3 (5.2) Mean duration was 3 years. Multiple lesions were seen in 2 children and single lesion in 1 Becker’s nevus 2 (3.4) Both were 14 years of age. Appeared at 12 years of age in one and at birth in the other. No other abnormality in either Urticaria pigmentosa 2 (3.4) Both were girls. Onset–birth and 2 years. No systemic involvement except raised alkaline phosphatase in both Chediak‑higashi syndrome 1 (1.7) ‑ Discoid lupus erythematosus 1 (1.7) DLE on the face for 2 years Incontinentia pigmenti 1 (1.7) 1‑year‑old boy (46 XY). Lesions appeared at 6 months of age. Also had development delay, spastic diplegia and esotropia, delayed dentition and cone shaped teeth Centrofacial lentiginosis 1 (1.7) 11‑year‑old boy (onset‑9 years). Hairs–brown. Had hypertelorism. Echocardiography- normal. Similar lesions in the patient’s brother Lichen striatus 1 (1.7) ‑ Nevus of ota 1 (1.7) Onset‑6 years. Also had CALM and nevus depigmentosus on the thigh Pityriasis rubra pilaris 1 (1.7) ‑ Speckled lentiginous nevus 1 (1.7) ‑ *Some cases had two disorders, CALM: Café‑au‑lait macule, DLE: Discoid lupus erythematosus, LP: Lichen planus

Indian Dermatology Online Journal - April-June 2013 - Volume 4 - Issue 2 149 Letters to the Editor

Figure 2: Pigmentary mosaicism affecting one side of the face

Figure 3: Pigmentary mosaicism affecting the trunk

Figure 5: Fixed drug eruption lesions over the trunk

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 Figure 4: Multiple Mongolian spots in Hurler’s syndrome baby of one child with 20 macules had neurofibromatosis.

150 Indian Dermatology Online Journal- April-June 2013 - Volume 4 - Issue 2 Letters to the Editor

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

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

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

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 Figure 9: Lentigenes affecting the face 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] FDE (no mucosal involvement). The incriminated drugs were study 80.3% of CMN were medium‑sized, and 19.7% were paracetamol and co‑trimoxozole. large‑sized. Majority of CMN were located in the lumbar area, followed by the back and face. CHS was seen in a 2‑year‑old girl who had generalized at birth and later developed slate‑gray Drug reactions are believed to be uncommon in children. hyperpigmentation all over the body sparing the axillae, neck, We noted 3 cases of hyperpigmentation due to past popliteal fossae, and inguinal region. The hyperpigmentation

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