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Year : 2014  |  Volume : 5  |  Issue : 5  |  Page : 65-66  

Idiopathic congenital true leukonychia totalis

Department of Dermatology, L. T. M. Medical College and General Hospital, Sion, Mumbai, India

Date of Web Publication13-Nov-2014

Correspondence Address:
Dr. Avhad Ganesh
Room No 110, First Floor, New RMO Hostel, LTMMC and GH, Sion, Mumbai - 400 022
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2229-5178.144551

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How to cite this article:
Ganesh A, Priyanka G. Idiopathic congenital true leukonychia totalis. Indian Dermatol Online J 2014;5, Suppl S1:65-6

How to cite this URL:
Ganesh A, Priyanka G. Idiopathic congenital true leukonychia totalis. Indian Dermatol Online J [serial online] 2014 [cited 2021 Dec 8];5, Suppl S1:65-6. Available from: https://www.idoj.in/text.asp?2014/5/5/65/144551

A 16-year-old male presented with white-colored fingernails since birth [Figure 1]. Toenails were normal. None of the family members were affected. Hematological investigations were normal. On cutaneous examination all finger nails were milky, porcelain white in color and smooth surfaced. No knuckle pads, palmoplantar keratoderma, or hearing loss was observed. General and systemic examination was normal. On clinical correlation, a diagnosis of idiopathic congenital true leukonychia totalis was made.
Figure 1: Milky, porcelain, white-colored smooth-surfaced finger nails

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Leukonychia is the white discoloration of nail that loses its normal pink color, with disappearance of the lunula. It is the commonest chromatic abnormality of the nail apparatus. It is also referred to as white nail, milk spots, and fortune or gift spots. It is derived from the Greek word "leukos" means white and "onyx" means nail. [1],[2] Leukonychia is classified as: [Table 1].
Table 1: Classification of leukonychia

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The proposed white discoloration of the nail is attributable to reflection of light from large keratohyaline granules present in the parakeratotic cells of the ventral portion of nail plate thus preventing the visualisation of normal vascular tissue underlying the nail plate. [2],[3]

Total congenital leukonychia is very rare hereditary condition in which all nails are milky white with an autosomal dominant inheritance but it can be autosomal recessive in few cases. Hereditary leukonychia involves whole nail plate as against acquired one which shows transverse or punctate leukonychia. Mutations in PLCD1 gene on chromosome 3p21.3-p22 was identified as cause of hereditary leukonychia. This PLCD1 gene is localized to nail matrix and encodes phosphoinositide specific phospholipase C delta 1 subunit which is important enzyme in phosphoinositide metabolism required for molecular control of color and growth of nail. [4]

Leukonychia totalis is associated with multiple systemic diseases such as diabetes, liver cirrhosis, renal failure, cardiac failure, gall stones, renal calculi, duodenal ulcers, pili torti, trichilemmal cyst, sebaceous cyst, congenital hyperparathyroidism, Hodgkin's lymphoma, Leopard syndrome, epiphyseal dysplasia syndrome, Bart Pumphrey syndrome, mental retardation, etc., However, there are very few reports in the literature on sporadic congenital leukonychia totalis without any systemic abnormalities. [1],[3]

Treatment is usually supportive with detailed investigation to check for association of any systemic or genetic disorders.

   References Top

De D, Handa S. Hereditary leukonychia totalis. Indian J Dermatol Venereol Leprol 2007;73:355-7.  Back to cited text no. 1
[PUBMED]  Medknow Journal  
Kwon NH, Kim JE, Cho BK, Jeong EG, Park HJ. Sporadic congenital leukonychia with koilonychia. Int J Dermatol 2012;51:1400-2.  Back to cited text no. 2
Lee YB, Kim JE, Park HJ, Cho BK. A case of hereditary leukonychia totalis and partialis. Int J Dermatol 2011;50:233-4.  Back to cited text no. 3
Kiuru M, Kurban M, Itoh M, Petukhova L, Shimomura Y, Wajid M, et al. Hereditary leukonychia, or porcelain nails, resulting from mutations in PLCD1. Am J Hum Genet 2011;88:839-4.  Back to cited text no. 4


  [Figure 1]

  [Table 1]


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