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 Table of Contents  
CASE REPORT
Year : 2015  |  Volume : 3  |  Issue : 2  |  Page : 302-305

Idiopathic total leukonychia involving fingernails: A report of two cases


Department of Dermatology, Sri Venkata Sai Medical College, Mahbubnagar, Telangana, India

Date of Web Publication16-Dec-2015

Correspondence Address:
Gnaneshwar Rao Angoori
Department of Dermatology, Sri Venkata Sai Medical College, Mahbubnagar - 500 060, Telangana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2321-4848.171934

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  Abstract 

Total leukonychia is a rare nail disorder that may be inherited or associated with systemic disease or idiopathic where the aetiology cannot be established. Herein, we report idiopathic total leukonychia in two young healthy men, presenting with leukonychia of fingernails since childhood. The cause for leukonychia could not be established in both of them as there was negative family and drug history and there was no evidence of systemic disease.

Keywords: KOH mount, large keratohyalin granules, partial leukonychia, sabouraud dextrose agar, total leukonychia


How to cite this article:
Angoori GR, Koppada D. Idiopathic total leukonychia involving fingernails: A report of two cases. Arch Med Health Sci 2015;3:302-5

How to cite this URL:
Angoori GR, Koppada D. Idiopathic total leukonychia involving fingernails: A report of two cases. Arch Med Health Sci [serial online] 2015 [cited 2019 Jul 21];3:302-5. Available from: http://www.amhsjournal.org/text.asp?2015/3/2/302/171934


  Introduction Top


Whitish discoloration of the nails is called leukonychia. It is derived from the Greek words Leuko (means white) and Onyx (means nail). It was first described by Mees in the year 1919 in association with chronic arsenic poisoning. [1] The exact mechanism of whitening is not known. However, abnormal matrix keratinization, persistent parakeratosis, and dissociation of the keratin bundles may play a role in the modification of the solar light reflection by the ungual plates. Moreover, disorganization of the keratin fibrils leads to diffraction of light in parakeratotic cells, which results in opaque appearance of the nail plate.

Leukonychia is a rare nail disorder and is difficult to establish the cause although a number of investigations are available in the armory of dermatologists. Herein, we report total leukonychia involving fingernails in two young healthy men.


  Case Report Top


A 30-year-old male (case 1) presented with whitening of the fingernails since childhood, and another 32-year-old male (case 2) also presented with whitening of the nails since the age of 8 years with complaint of itchy rash on the sun-exposed areas for 1-month duration. Both of them were born to non-consanguineous parents and had normal developmental milestones. In both the cases, dyschromia started in few nails initially and progressed to involve all the fingernails. Family history was negative for leukonychia in them. They denied history of prolonged illness or trauma to nails and were not on long-term medication, systemic or topical. In both the patients all the fingernails were completely white involving the entire nail plate with normal cuticles. Lunula was invisible, and there was no subungual debris or onycholysis. [Figure 1] and [Figure 2] All the toe-nails were normal in both the cases. [Figure 3] They had normal hair, and there was no abnormality of oral or genital mucosa. In case 2, additionally, there were grouped fine papules distributed on face, sides of neck, and extensor aspects of forearms. Both were provisionally diagnosed as total leukonychia of fingernails, and in addition, case 2 was associated with polymorphic light eruption. They were subjected to investigations. Routine blood investigations; complete blood picture, blood sugar, blood urea, liver function tests, serum proteins, serum calcium, and serum electrolytes were normal. Stool was negative for cyst and ova. Serology for HIV 1 and 2 was non-reactive. Thyroid profile, chest X-ray, ultrasonography of abdomen, electrocardiogram, and 2D Echo were normal. Nail clippings for potassium hydroxide (KOH) mount and culture on Sabouraud's dextrose agar was negative in both the cases. Nail biopsy could not be done as they refused. However, distal part of the fingernail plate from case 1 was subjected to histopathological examination, which revealed large keratohyalin granules suggestive of abnormal keratinization [Figure 4].
Figure 1: Dorsum of both hands of case 1 showing complete leukonychia involving all finger nails

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Figure 2: Dorsum of both hands of case 2 showing complete leukonychia involving all finger nails

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Figure 3: Dorsum of both feet of case 1 showing normal toe-nails

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Figure 4: Photomicrograph of nail plate of case 1 X 40 H and E stain showing large keratohyalin granules

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  Discussion Top


Unna classified leukonychia into five clinical types based on the distribution of whiteness:

  1. Leukonychia totalis.
  2. Leukonychia partialis.
  3. Leukonychia striata.
  4. Leukonychia transversalis.
  5. Leukonychia punktata. Nonetheless, Butterworth considers leukonychia partialis as a phase of leukonychia totalis, and not a distinct entity. [2] Furthermore, other researchers classified it into true leukonychia, where the pathology is in the nail plate and apparent leukonychia, where the pathology is in the subungual tissue.
Considering the details in the history, clinical examination and investigations, idiopathic total leukonychia is likely diagnosis in both the cases as the cause could not be established. However, nail biopsy and genetic studies were not done in both the cases, which could have helped in establishing the cause of leukonychia. Nonetheless, histopathological examination of nail plates from case 1 revealed large keratohyalin granules indicative of abnormal keratinization. Because leukonychia is present from childhood in them and involved all the fingernails uniformly, heredity may be attributable, but negative family history and absence of genetic studies do not substantiate the hereditary cause. Trauma as the cause of leukonychia is unlikely as there is no history of injury, and there is complete involvement of all the fingernails. Absence of systemic disease and drug intake in them undermines their role as the cause of total leukonychia.

Total leukonychia is usually an autosomal dominantly inherited condition but can also be inherited autosomal recessively. Kiuru et al. identified PLCD1 gene that is linked to chromosome 3p21.3-p22 and that encodes phosphoinositide-specific phospholipase C delta 1 subunit, a key enzyme in phosphoinositide metabolism, which is involved in molecular control of nail growth, and its mutation is said to result in hereditary leukonychia. [3] Total leukonychia can be part of Bart-Pumphrey syndrome in which there is associated knuckle pads, deafness, and palmoplantar keratoderma [4] or Buschkell-Gorlin syndrome in which there is also associated sebaceous cysts and renal calculi. Crosti et al. have also reported total leukonychia in association with palmoplantar keratoderma, hair, and dental anomalies in two siblings and ascribed it to single defect gene. [5] Total leukonychia is also known to be associated with LEOPARD syndrome, congenital hyperparathyroidism, and pili torti. However, there are no symptoms or signs suggestive of such syndromic association in both of them. Systemic conditions that may cause total leukonychia include cirrhosis of liver, congestive heart failure, leprosy, typhoid fever, and trichinosis. [6] Emmanuel described acquired total leukonychia involving all finger and toe-nails in a 72-year-old lady with multiple co-morbidities like diabetes mellitus, hypertension, peripheral vascular disease, and coronary artery disease. Interestingly, all her nails returned to normalcy 6 months after coronary angioplasty, suggesting that leukonychia associated with systemic diseases may be transient and reversible. [7] However, there is no evidence of systemic disease in both the index cases. Leukonychia was also reported in patients with mycosis fungoides treated with vorinostat, a zinc-dependent histone deacetylase (HDAC) inhibitor. [8] Furthermore, HIV infection, [9] hypocalcemia, [10] cyclophosphamide therapy, [11] trauma to nail, [12] and prednisone [13] are known to cause transverse leukonychia of fingers. However, the precise cause for transverse leukonychia in HIV patients has not been elucidated. It could be due to systemic infections associated with HIV infection [8] or the antiretroviral therapy. Moreover, it was hypothesized that digital arteriolar spasm leading to disorganization of hard keratin is responsible for leukonychia in hypocalcemic patients. [9] Of note, is the complete involvement of fingernails in both the index cases in contrast with the transverse leukonychia seen in the above conditions. Furthermore, both the patients were normocalcemic and were negative for HIV serology and not on cyclophosphamide or prednisone therapy. Interestingly, longitudinal leukonychia has been reported in 71% of patients with Hailey-Hailey disease, usually is the initial sign of the disease and seems to predominantly involve the thumb nails. [14] Dermoscopy is known to help in early detection of longitudinal leukonychia and thus help in the diagnosis of Hailey-Hailey disease. [15] Idiopathic total and partial leukonychia is a rarity, and there have been few case reports in the literature similar to our cases where the cause could not be established. [16],[17],[18],[19] [Table 1] Notably, leukonychia initially started in them as leukonychia partialis and gradually progressed to leukonychia totalis.
Table 1: Showing cases of idiopathic partial and total leukonychia reported by various authors

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In conclusion, idiopathic true total leukonychia is intriguing aesthetic muddle and nothing much can be offered in the management of this niggling condition except for proper counseling and advice regarding camouflaging of the nails. Nonetheless, total leukonychia due to systemic disorders appears to have reasonably good prognosis as it seems to be transient and reversible. Proper management of the systemic disease and reassurance may help in regaining normalcy of the nails in these patients.

 
  References Top

1.
Mees RA. Een verschijnsel bij polyneuritis arsenicasa. Nederlands Tijdschrift Voor Geneeskunde 1919;1:391-6.  Back to cited text no. 1
    
2.
Butterworth T. Leukonychia partialis. A phase of leukonychia totalis. Cutis 1982;29:363-4, 367.  Back to cited text no. 2
    
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;88:839-44.  Back to cited text no. 3
    
4.
Balighi K, Moeineddin F, Lajevardi V, Ahmadreza R. A family with leukonychia totalis. Indian J Dermatol 2010;55:102-4.  Back to cited text no. 4
[PUBMED]  Medknow Journal  
5.
Crosti C, Sala F, Bertani E, Gasparini G, Menni S. Leukonychia totalis and ectodermal dysplasia. Report of 2 cases. Ann Dermatol Venereol 1983;110:617-22.  Back to cited text no. 5
    
6.
Ekmekçi TR, Köslü A. Lökonisi totalis. Türkderm 2004;38:146-7.  Back to cited text no. 6
    
7.
Antonarakis ES. Images in clinical medicine. Acquired leukonychia totalis. N Engl J Med 2006;355:e2.  Back to cited text no. 7
    
8.
Anderson KA, Bartell HL, Olsen EA. Leukonychia related to vorinostat. Arch Dermatol 2009;145:1338-9.  Back to cited text no. 8
    
9.
de Carvalho VO, da Cruz CR, Marinoni LP, Lima JH. Transverse leukonychia and AIDS. Arch Dis Child 2006;91:326.  Back to cited text no. 9
    
10.
Foti C, Cassano N, Palmieri VO, Portincasa P, Conserva A, Lamuraglia M, et al. Transverse leukonychia in severe hypocalcemia. Eur J Dermatol 2004;14:67-8.  Back to cited text no. 10
    
11.
Naumann R, Wozel G. Transverse leukonychia following chemotherapy in a patient with Hodgkin's disease. Eur J Dermatol 2000;10:392-4.  Back to cited text no. 11
    
12.
Howard SR, Siegfried EC. A case of leukonychia. J Pediatr 2013;163:914-5.  Back to cited text no. 12
    
13.
Grossman M, Scher RK. Leukonychia: Review and classification. Int J Dermatol 1990;29:535-41.  Back to cited text no. 13
    
14.
Burge SM. Hailey-Hailey disease: The clinical features, response to treatment prognosis. Br J Dermatol 1992;126:275-82.  Back to cited text no. 14
    
15.
Bel B, Jeudy G, Vabres P. Dermoscopy of longitudinal leukonychia in Hailey-Hailey disease. Arch Dermatol 2010;146:1204.  Back to cited text no. 15
    
16.
Stewart L, Young E, Lim HW. Idiopathic leukonychia totalis and partialis. J Am Acad Dermatol 1985;13:157-8.  Back to cited text no. 16
    
17.
Claudel CD, Zic JA, Boyd AS. Idiopathic leukonychia totalis and partialis in a 12-year-old patient. J Am Acad Dermatol 2001;44:379-80.  Back to cited text no. 17
    
18.
Bongiorno MR, Aricò M. Idiopathic acquired leukonychia in a 34-year-old patient. Case Rep Med 2009;2009:495809.  Back to cited text no. 18
    
19.
Arsiwala SZ. Idiopathic acquired persistent true partial to total leukonychia. Indian J Dermatol Venereol Leprol 2012;78:107-8.  Back to cited text no. 19
[PUBMED]  Medknow Journal  


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
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