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HEREDITARY LEUKONYCHIA TOTALIS - A CASE REPORT

Authors:
  • Government Ayurveda College Osmanabad
  • Govt ayurved college osmanabad

Abstract

Leukonychia totalis is a rare disorder which is seen in the form of complete whitening of nails. The disorder may be observed as Hereditary and/ or acquired. True Leukonychia is typically inherited in autosomal recessive manner or it can be acquired because of trauma and other factors. In few cases pseudoleukonychia have also been reported which is caused by the changes of nail bed. Acquired Leukonychia totalis may represent several systemic disorders. A case of 22 year healthy male with Leukonychia totalis is reported here who is having all 20 nails completely white and hard with positive family history. Thorough investigations reveled that he does not suffer from any systemic disease related to Leukonychia totalis.
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Binorkar et al. World Journal of Pharmacy and Pharmaceutical Sciences
HEREDITARY LEUKONYCHIA TOTALIS - A CASE REPORT
Mayur Deshmukh1, Sandeep Binorkar*2 and Ashwin Bagde3
1Assistant Professor, Dept. of Rognidan and Vikriti Vigyan, Government Ayurved College
and Hospital, Osmanabad.
2Assistant Professor, Dept. of Agadtantra, Government Ayurved College and Hospital,
Nanded.
3Assistant Professor, Dept. of Sanskrit, Samhita, Siddhant, Government Ayurved College
and Hospital, Osmanabad.
ABSTRACT
Leukonychia totalis is a rare disorder which is seen in the form of
complete whitening of nails. The disorder may be observed as
Hereditary and/ or acquired. True Leukonychia is typically inherited
in autosomal recessive manner or it can be acquired because of trauma
and other factors. In few cases pseudoleukonychia have also been
reported which is caused by the changes of nail bed. Acquired
Leukonychia totalis may represent several systemic disorders. A case
of 22 year healthy male with Leukonychia totalis is reported here who
is having all 20 nails completely white and hard with positive family
history. Thorough investigations reveled that he does not suffer from
any systemic disease related to Leukonychia totalis.
KEYWORDS: Leukonychia totalis, Hereditary, PLCD1
INTRODUCTION
Nail examination is important aspect of general examination. Changes in nails especially with
respect to its color and structure may represent systemic diseases many times. Pallor in nails
represents Anaemia. In Iron deficiency Nails become pale, thin and brittle. In advanced and
long standing Iron deficiency Anaemia nails become spoon shaped i.e. „koilonychia‟. In
pulmonary and Cyanotic Heart disease nails may be bluish colored indicating peripheral
cyanosis. Clubbing of nails is found in COPD and Cor Pulmonale.[1] Whereas complete
Whitening of nails called as Leukonychia. It was explained for the first time in 1919 by R. A.
Mees which was associated with chronic toxicity of Arsenic.[2] Although it may occur due to
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*Correspondence for
Author
Dr. Sandeep Binorkar
Assistant Professor, Dept.
of Agadtantra,
Government Ayurved
College and Hospital,
Nanded.
Article Received on
02 Jan 2016,
Revised on 24 Jan 2016,
Accepted on 14 Feb 2016,
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Binorkar et al. World Journal of Pharmacy and Pharmaceutical Sciences
injury to nail matrix, Leuconychia can be classified in several ways. Broadly speaking, it is
classified as true leukonychia, which occurs due to pathology in the matrix and
pseudoleukonychia, is produced by the changes of nail bed.[3] However it can also be
classified based on the extent of whiteness seen on the nail as 1) Leuconychia partialis 2)
Leuconychia totalis. In Leuconychia partialis small white dots are found over nail plate.
Three types of variations are found in Leuconychia partialis- a) Punctate b) Transverse c)
Longitudinal.[4] Study shows that prevalence of leukonychia partialis in healthy female
children was 42.72% with opacities in their nails were of either the punctate or the striate
type.[5] But no literature is available indicating the prevalence of Leukonychia totalis.
1) Leukonychia partialis; 2) Leukonychia totalis; 3) Leukonychia striata; 4)
Leukonychia transversalis; 5) Leukonychia punctata
Fig 1: - Types of Leukonychia[4]
Table No. 1 Classification of true leukonychia
Hereditary
Acquired
Associated
Idiopathic
Associated
Bart-Pumphrey syndrome
Bauer syndrome
Heimler syndrome
Knuckle pads-leukonychia-
deafness syndrome
Keratoderma, hypotrichosis and
leukonychia totalis
syndrome
Lowry-Wood syndrome
FLOTCH syndrome
Congenital keratosis palmaris et
plantaris
deafness and total leukonychia
LEOPARD syndrome
Trauma
Drugs
Systemic infections
Local infections
Inflammatory disease
Leuconychia punctata
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This is called as true leuconychia. It is most common form. Small white spots appear on
nails. Children most of the time has habit of biting nails, cause damage of nail cuticle.
Trapping of air between cells may also cause Punctata Leuconychia.
Leuconychia striata (transverse Leuchonychia)
It is also called as Mees‟ line. They are found in nail trauma, heavy metal poisoning, Measles,
Malaria, Leprosy.
Longitudinal Leuconychia
It is far less common type, 1mm white longitudinal lines seen under nail plates.
The half and half nails- Proximal half white and distal half red or brown is seen in some
CRF patients.
Leuconychia totalis
Complete whitening of entire nail plate is called as Leukonychia totalis. It is also called as
„porcelin nails‟, „Non syndromic congenital nail disorder‟ and hereditary white nails. It may
be acquired or inherited. Acquired leukonychia may be due to trauma, Liver cirrhosis, liver
failure, Hypoalbuminaemia, nehrotic syndrome, Ulcerative Colitis, Protein mal-absorption,
Protein losing enteropathy, side effects of sulphonamide & chemotherapeutic drugs.[6]
Inherited Leukonychia is a genetic condition. It is usually autosomal dominant or may be
autosomal recessive. The inherited forms can be caused by mutations in the PLCD1 gene and
generally involve the entire plate of all 20 nails.[7]
Table No. 2 Causes of Leuconychia
Sr No
Acquired
Congenital
1
Addison disease
Leukonychia only
2
After the treatment with
antimetabolites
Leukonychia with koilonychia
3
Cardiac failure
Leukonychia with knuckle pads and deafness
4
Systemic lupus
erythematosus
Leukonychia totalis, multiple sebaceous
cysts and renal calculi
5
Arsenic poisoning
--
6
Exfoliative dermatitis
--
7
Hodgkin disease
--
8
Infectious fever
--
9
Menstrual cycle
--
10
Myocardial infarct
--
11
Leprosy, malaria,
pneumonia
--
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Binorkar et al. World Journal of Pharmacy and Pharmaceutical Sciences
12
Pellagra
--
13
Thallium poisoning
--
14
Tuberculosis
--
15
Trauma
--
16
Herpes zoster
--
17
Fungal infections
--
18
Tuberculosis
--
19
Zinc deficiency
--
20
Trichinosis
--
CASE REPORT
A 22 year male attended Rognidan OPD of Government Ayurved College, Osmanabad on
27/11/2015, complaining of white nails. Whitening of Nails was present since childhood. His
all 20 nails (fingers & toes) were totally white. On complete systemic examination there was
no evidence of any systemic disease. He was not having previous significant medical or
surgical history. He was physically healthy and active. He had not having past history of drug
intake or allergy. There was no any skin lesion indicating dermatological diseases. Also
neither he was consuming any medicines nor was he exposed to any chemical agents in his
past history. His all nails were totally white and hard on touch. (Fig-2) Being working as a
sales representative in a fertilizer company by occupation, he was not having direct contact
with chemicals. Moreover he has joined the occupation from 2-3 years while in fact the
symptoms were present from the childhood. There was no family history of dermatological
lesions like atopy, psoriasis, lichen planus etc.
Fig 2 Finger Nails of Patient with Leuconychia totalis
Thorough investigations were conducted to rule out any underlying disease. Laboratory
investigations showed normal complete blood count, where as urine analysis showed
detection of bile salt and bile pigment but USG abdomen pelvis was normal. HbsAg and
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Binorkar et al. World Journal of Pharmacy and Pharmaceutical Sciences
Tridot for HIV were also negative. Family history reveled that Uncle (mother‟s brother) and
Aunt (mother‟s sister) were having similar features with Leuconychia totalis which confirms
hereditary origin of the disease in this patient.
DISCUSSION
Leuconychia totalis is a rare disorder. Very few cases of Leuconychia totalis have been
reported so far.[8,9,10,11,12,13,14,15] The patho-physiology behind the Leuconychia totalis can not
demonstrated by the medical science till date. It is proposed that leuconychia is due to
abnormal matrix keratinization, with persistent parakeratosis and keratohyaline granules in
the nail plate.[16] It is hypothesised by few scholars that Parakeratosis and dissociation of the
keratin bundles might be playing role in the modification of the light reflection by the finger
plates. In present case report the patient did not show any association of leukonychia with any
of the above-mentioned syndromes. Genetic evaluation of a French family on four
generations with hereditary leukonychia provided evidence of its link to chromosome 12q13,
which contains the basic type II keratins locus.[17][18] There is no worldwide successful
treatment for Leukonychia totalis.[7] Appropriate medications for dealing with the underlying
illness and systemic disorder may improve the situation.
CONCLUSION
There are several disorders of nail which may lead to white discoloration but hereditary
Leukonychia totalis has a sporadic cause. It may be present as a sole congenital anomaly or in
association with several other dermatologic problems & systemic syndromes. Symptomatic
treatment for the underlying cause and associated systemic diseases if any is the only line of
treatment known till date.
REFERENCES
1. Hutchison‟s Clinical Methods – Michel Swash, Twentieth Edition, 52-53.
2. R. A. Mees, “Een verschijnsel bij polyneuritis arsenicasa,” Nederlands Tijdschrift voor
Geneeskunde, 1919; 1: 391396.
3. Şendur N, Karaman G, Şavk E. Tırnak Hastalıkları. In: Dermatoloji. Ed. Tüzün Y, Gürer
MA, Serdaroğlu S, Oğuz O, Aksungur VL. 3rd Ed. İstanbul, Nobel Tıp Kitapevleri, 2008;
1345-1376.
4. Yalçın Tüzün, Özge Karakuş, Leukonychia, J Turk Acad Dermatol, 2009; 3(1): 93101r.
5. C. M. Tiwari, H. N. Mehrotra, Prevalence of leukonychia in healthy female children, The
Indian Journal of Pediatrics, 1972; 39(7): 243-245.
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6. A. Yoruk and H. Yukselgungor, “Chemotherapy induced transverse leukonychia in
children,” International Journal of Dermatology, 2003; 42(6): 468469.
7. Marla J. F. O'Neill. Nail Disorder, Nonsyndromic Congenital, 3; NDNC3. OMIM.
August 11, 2011; http://omim.org/entry/151600. Accessed 07.12.2015.
8. Sibley, K. Leuconychia striata. Brit. J. Derm. Syph, 1922; 34: 238-239.
9. Becker, S. W. Leukonychia striata: report of a congenital case. Arch. Derm. Syph, 1930;
21: 957-960.
10. Kruse, W. T., Cawley, E. P., Cotterman, C. W. Hereditary leukonychia totalis. J. Invest.
Derm. 17: 135-140, 1951. [PubMed: 14880728, related citations].
11. Medansky, R. S., Fox, J. M. Hereditary leukonychia totalis. Arch. Derm, 1960; 82: 412-
414.
12. Albright, S. D., Wheeler, C. E., Jr. Leukonychia: total and partial leukonychia in a single
family with a review of the literature. Arch. Derm, 1964; 90: 392-399
13. Butterworth, T. Leukonychia partialis: a phase of leukonychia totalis. Cutis, 1982; 29:
363-367.
14. Stevens, K. R., Leis, P. F., Peters, S., Baer, S., Orengo, I. Congenital leukonychia. J. Am.
Acad. Derm, 1998; 39: 509-512.
15. Kiuru, M., Kurban, M., Itoh, M., Petukhova, L., Shimomura, Y., Wajid, M., Christiano,
A. M. Hereditary leukonychia, or porcelain nails, resulting from mutations in PLCD1.
Am. J. Hum. Genet, 2011; 88: 839-844.
16. Maria Rita Bongiorno & Mario Aricò, Idiopathic Acquired Leukonychia in a 34-Year-
Old Patient, Case Reports in Medicine, 2009; 1-3.
17. Norgett EE, Wolf F, Balme B, Leigh IM, Perrot H, Kelsell DP, et al. Hereditary „white
nails‟: A genetic and structural study. Br J Dermatol, 2004; 151: 6572.
18. Kamran Balighi et.al. A Family with Leukonychia totalis, Indian J Dermatol, Jan-Mar
2010; 55(1): 102104.
ResearchGate has not been able to resolve any citations for this publication.
Article
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