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LETTERS IN RESPONSE TO PREVIOUS PUBLICATION IN IJD®
Year : 2016  |  Volume : 61  |  Issue : 6  |  Page : 675
Author's reply: Pachyonychia congenita Type 1: Case report and review of the literature


Department of Dermatology, Medical College and Hospital, Kolkata, West Bengal, India

Date of Web Publication9-Nov-2016

Correspondence Address:
Anupam Das
Department of Dermatology, Medical College and Hospital, Kolkata, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.193686

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How to cite this article:
Das A. Author's reply: Pachyonychia congenita Type 1: Case report and review of the literature. Indian J Dermatol 2016;61:675

How to cite this URL:
Das A. Author's reply: Pachyonychia congenita Type 1: Case report and review of the literature. Indian J Dermatol [serial online] 2016 [cited 2019 Jun 17];61:675. Available from: http://www.e-ijd.org/text.asp?2016/61/6/675/193686


Sir,

We thank the authors for taking an interest in our recent article. [1] We, in our report, had described a case of an 8-year-old male child with nail dystrophy, subungual hyperkeratosis, oral leukokeratosis, and numerous follicular papules, who was diagnosed with pachyonychia congenita Type I. [2]

The authors have emphasized the need of genetic and molecular testing for reaching a pinpoint diagnosis, consistent with the features reported by McLean et al. [3] In our case, the diagnosis was done on the basis of clinical findings, histological features, and clinicopathological correlation. We agree on the point that genetic testing should have been done. Otherwise, cases which are actually not pachyonychia congenita and close mimickers such as Clouston syndrome or Plack syndrome may be misreported as pachyonychia congenita. However, it is worth mentioning that in a setting like ours, where most of the patients belong to an extremely poor socioeconomic status, it becomes impossible for us to ask for such investigations. Besides, the lack of infrastructure adds to the miserability. In addition to this, such genodermatoses have poor prognostic outcome and we try our best to provide symptomatic management to the patient and most importantly counseling so as to enable them to lead a better life. Our patient had been prescribed Vitamin A after consultation with the Department of Pediatrics and Ophthalmology and referred to the Department of Physical Medicine and Rehabilitation for weight control exercises. Unfortunately, he is lost to follow-up.

We thank the authors for enlightening us regarding Pachyonychia Congenita Project (http://www.pachyonychia.org) so that we are able to proceed for genetic testing in case we find a case with similar clinical findings in future.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Agarwala MK, Schwartz ME, Smith FJ. Pachyonychia congenita: New classification and diagnosis. Indian J Dermatol 2016;61:567.  Back to cited text no. 1
[PUBMED]  Medknow Journal  
2.
Rathore PK, Khullar V, Das A. Pachyonychia congenita type 1: Case report and review of the literature. Indian J Dermatol 2016;61:196-9.  Back to cited text no. 2
[PUBMED]  Medknow Journal  
3.
McLean WH, Hansen CD, Eliason MJ, Smith FJ. The phenotypic and molecular genetic features of pachyonychia congenita. J Invest Dermatol 2011;131:1015-7.  Back to cited text no. 3
    




 

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