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CASE REPORT
Year : 1994  |  Volume : 60  |  Issue : 1  |  Page : 43-44

Pachyonychia congenita tarda




Correspondence Address:
A K Jaiswal


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  Abstract 

A case of Pachyonychia congenita is being reported. The interesting feature being the onset of nail changes beyond the first few years of life.


Keywords: Pachyonychia Congenita Tarda


How to cite this article:
Jaiswal A K, Badrinath S, Pillai J, Bhardwaj M. Pachyonychia congenita tarda. Indian J Dermatol Venereol Leprol 1994;60:43-4

How to cite this URL:
Jaiswal A K, Badrinath S, Pillai J, Bhardwaj M. Pachyonychia congenita tarda. Indian J Dermatol Venereol Leprol [serial online] 1994 [cited 2020 Jan 26];60:43-4. Available from: http://www.ijdvl.com/text.asp?1994/60/1/43/3984



  Introduction Top


Pachyonychia congenita (PC) is a rare, autosomal dominant disorder characterized by discoloration and thickening of the nails, usually begining within the first month of life. [1],[2] Reports of the onset of nail changes beyond the first few years of life are rare. The term pachyonychia congenita tarda (PCT) has been suggested by Paller et al for the late onset form of PC. [3] We herein report one such case.


  Case Report Top


A 30-year-old soldier had discoloration and marked thickening of the fingernails and toenails that began at 8 years of age and persisted unchanged. He was otherwise well except for a recent history of paronychial infection. The patient had no history of cutaneous blisters, natal teeth or corneal dystrophy. His niece was similarly affected with thickened nails that developed at 6 years of age.

Physical examination revealed yellow discoloration and marked thickening of the fingernails and toenails, with subungual hyperkeratosis but a smooth surface [Figure - 1]. Some of the nails had a "pinched" appearance at the distal aspect, and the nail tip was angulated upward. The 'patient in addition had mild palmoplantar hyperhidrosis. No leukokeratosis and/or palmoplantar keratoderma was noticed. Rest of the physical examination was unremarkable.

Results of KOH examinations were negative. Fungus cultures from the nails yielded no organisms. X-rays of hands/feet did not reveal any abnormality. Patient refused biopsy.


  Comments Top


PC was originally described by Wilson [4] in 1905, although the association of the disorder with palmoplantar keratoderma and other ectodermal defects was first reported by Jadassohn- Lewandowsky. [5] PC has been subdivided into four groups, based on the clinical features associated with nail changes. [6] Common to almost all patients who have been described, regardless of the form of inheritance or subclassification of the disorder, is the onset of pachyonychia in infancy. Only 9 cases have been reported so far with late onset. [1],[3] No established classification mentions a late onset form of the disorder. We agree with Paller et al (1991) that this form should constitute a subset of PC and the positive family history of pachyonychia in our patient further supports the opinion that PCT is also inherited in an autosomal dominant manner . [3]

 
  References Top

1.Su WP, Chun SI, Hammond DE, Gordon H. Pachyonychia congenita : a clinical study of 12 cases and review of the literature. Pediatar Dermatol 1990; 7 : 33-8.  Back to cited text no. 1  [PUBMED]  
2.Feinstein A, Friedman J, Schewach-Millet M. Pachyonychia congenita. J Am Acad Dermatol 1988; 19 : 705-11.  Back to cited text no. 2  [PUBMED]  
3.Paller SA, Moore JA, Seher R. Pachyonychia congenita tarda : A late-onset form of Pachyonychia congenita. Arch Dermatol 1991; 127 : 701-3.  Back to cited text no. 3    
4.Wilson AG. Three cases of hereditary hyperkeratosis of the nail bed. Arch Dermatol Syphilol 1905; 2 13-4.  Back to cited text no. 4    
5.Jadassohn J, Lewandowsky F, Pachyonychia congenita. In : Inkonographia Dermatologica (Neisser A, Jacobi E, eds), Baltimore : Md, Urban and Schwarzenberg, 1906;29.  Back to cited text no. 5    
6.Dawber RPR, Baran R. Disorders of Nails. In : Textbook of Dermatology (Champion RH, Burton JL, Ebling FJG, eds), 5th edn. Oxford : Blackwell Scientific Publications, 1992; 2530-1.  Back to cited text no. 6    


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