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 Table of Contents  
CASE REPORT
Year : 2012  |  Volume : 13  |  Issue : 1  |  Page : 46-47

Microtia secondary to morphea in a 5-year-old male child: An unusual presentation


Department of Dermatology, Venereology, and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Date of Web Publication23-Oct-2012

Correspondence Address:
Amrinder J Kanwar
Department of Dermatology, Venereology, and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh- 160 012
India
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Source of Support: None, Conflict of Interest: None


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  Abstract 

Morphea, also known as localized scleroderma, is characterized by fibrosis of skin and underlying tissue. Report of morphea lead to microtia is not reported till now and we report an unusual case of localized morphea lead to microtia in a 5 year old male child.

Keywords: Morphea, microtia, scleroderma, lichen sclerosus et atrophicus


How to cite this article:
Kumar R, Agrawal S, Kanwar AJ. Microtia secondary to morphea in a 5-year-old male child: An unusual presentation. Indian J Paediatr Dermatol 2012;13:46-7

How to cite this URL:
Kumar R, Agrawal S, Kanwar AJ. Microtia secondary to morphea in a 5-year-old male child: An unusual presentation. Indian J Paediatr Dermatol [serial online] 2012 [cited 2020 Jul 5];13:46-7. Available from: http://www.ijpd.in/text.asp?2012/13/1/46/102815


  Introduction Top


Morphea is an uncommon disorder of unknown etiology. It begins with a patchy lilac erythema at the border and a slowly spreading yellow-white lesion in the center. It is characterized by thickening and/or hardening of the skin and subcutaneous tissue as a result of excess collagen deposition.


  Case Report Top


A 5-year-old male child presented with his parents to the Pediatric Dermatology clinic with complaints of thickening and hardening of the skin over the right ear of 1.5 years duration. It started from the right ear lobule and posterior aspect of the ear pinna. This progressively involved the helix, retroauricular area and concha. Over a period of 1 year, this led to resorption of the ear lobule and decrease in the size of the right ear. Apart from cosmetic disfigurement, it was otherwise asymptomatic and there was no difficulty in hearing. On cutaneous examination, there was an ill-defined, yellowish-white plaque with surrounding erythematous margins and bound-down texture involving, almost, the whole of the right ear [Figure 1]. Similar smaller plaques were present on the infra- and retro-auricular areas. A clinical diagnosis of localized morphea and lichen sclerosus et atrophicus was made and skin biopsy was performed. Histologically, the epidermis was found to be normal. The collagen bundles appeared thickened and closely packed along with increased hyalinization in between mature and immature hair follicles in the dermis. The overall features were consistent with morphea. He had no other facial abnormality and no extracutaneous manifestations of scleroderma.
Figure 1: Normal left ear and decreased size of right ear (microtia)

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  Discussion Top


Morphea involving the ear is uncommon and has rarely been reported in the literature. [1] Etiology of morphea is unknown, but autoimmune, [2] infections [3] and microchimerism [4] have been proposed. Linear pattern is the most common type of morphea in children. [5]

To the best of our knowledge, this is the first case of morphea to be reported that led to microtia. The child was referred to a pediatric plastic surgeon for correction of the cosmetic disfigurement.

 
  References Top

1.Martin JM, Alonso V, Monteagudo C, Jorda E. Localised retroauricular morphea in a boy with LENNOX-GASTAUT syndrome and hypospadias. Pediatr Dermatol 2007;24:196-7.  Back to cited text no. 1
    
2.Zulian F, Athreya BH, Laxer R, Nelson AM, Feitosa de Oliveira SK, et al. Juvenile localized scleroderma: Clinical and epidemiological features in 750 children. An international study. Rheumatology (Oxford) 2006;45:614-20.  Back to cited text no. 2
[PUBMED]    
3.Eisendle K, Grabner T, Zelger B. Morphoea: A manifestation of infection with Borrelia species? Br J Dermatol 2007;157:1189-98.  Back to cited text no. 3
[PUBMED]    
4.McNallan KT, Aponte C, el-Azhary R, Mason T, Nelson AM, Paat JJ, et al. Immunophenotyping of chimeric cells in localized scleroderma. Rheumatology (Oxford) 2007;46:398-402.  Back to cited text no. 4
[PUBMED]    
5.Morel Z, Benadon E, Faugier E, Maldonado Mdel R. Clinical characterstics of children with scleroderma in a referral hospital. Rheumatol Clin 2009;4:158-62.  Back to cited text no. 5
    


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Abstract
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Case Report
Discussion
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