A rare case of congenital,
sporadic, true Leukonychia Totalis
Dr. Satish Ramteke1, Dr. Sharmila
Ramteke2, Saurabh Nagar3, Dr. Mahendra
Kumar Jain4
1Assistant
Professor, Department of Medicine, Shyam Shah Medical
College and Associated Sanjay Gandhi Memorial Hospital, Rewa,
M.P., India.
2Assistant
Professor, Department of Pediatrics, Gandhi Medical College, Bhopal, M.P.,
India.
3RMO,
Department of Medicine, Shyam Shah Medical College
and associated Sanjay Gandhi Memorial Hospital, Rewa,
M.P., India.
4Professor
& Head, Department of Medicine, Shyam Shah
Medical College and Associated Sanjay Gandhi Memorial Hospital, Rewa, M.P., India.
ABSTRACT:
We report a rare case of a 40 year old
patient with sporadic, congenital, true, leukonychia totalis, not associated with any clinical syndromes or any
systemic disease. This is the third case to be reported in literature.
KEYWORDS: Leukonychia totalis.
CASE PRESENTATION:
A 40 year male, known case of hypertension,
was admitted with uncontrolled hypertention .On
examination total leukonychia of all twenty nails
(figure 1) was found. Patient informed that his all twenty nails have been
white ever since he could remember. He had trauma over left thumbnail after
which it got distorted, which was totally white similar to other nails earlier
to the trauma. There was no evidence of atopy, lichen planus, alopecia areata or psoriasis. There was no history of exposure to
any chemical agent and of any drug intake other than antihypertensive. He had
no central nervous system, eye, ear, hair, teeth, or skin abnormalities. The
patient was born to non consanguineous parents. His sister also had similar
white nails in all twenty digits since birth but none of their parents had such
a phenotype. Routine blood investigations including USG abdomen were normal.
Fig 1
DISCUSSION:
Leukonychia is whiteness of nails which may
occur either in patches or may involve the entire nail1,8. The condition
can be either true leukonychia, with involvement of nailplait, or pseudoleukonychia,
caused by subungual and nailbed
abnormalities. Leukonychia may be acquired or
inherited .Aquired leukonychia
is associated with trauma, drugs (chemotherapeutic agents2,8)
systemic or local infection such as typhoid, hepatic cirrohsis
,ulcerative colitis , leprosy etc .True leukonychia
can be inherited as an isolated condition or as a part of some syndromes. There
are number of autosomal dominantly inherited leuconychia syndromes, including leukonychia
totalis, where it is seen in combination with kidney
stone, sebaceous cysts 3,4,8, sensoryneural
deafness and knuckle pads5,6,8.
Leuconychia is whitening of the nail plate.
It was first described by Mees in 1919 as an
associated finding in arsenic intoxication7,8 . Abnormal karatinization of nail plate is belived
to be responsible for true leuconychia. According to
Newton’s theorem, a surface appears white when it reflects the radiation of
visible light. Because true leukonychia is
thought to be due to abnormal matrix
keratinisation , with persistent
parakerosis .
Large karatohyline granules are found in karatinocytes in nail plate. This parakerotosis
and dissociation of karatin bundles may play role in
the modification of the light reflection by ungueal
plates. True leukonychia
can be hereditary or acquired8. Hereditary leukonychia
can be as an isolated finding or may be associated with some symdromes like 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,
while acquired leoconychia can be idiopathic or
associated with trauma, drug, systemic infection, local infection, and inflamatory disease. Appearance wise leuconychia
can be classified as leuconychia punctata
, leukonychia striata, leokonychia partialis and totalis.
Our case is not associated with any of
above mentioned syndromes. Leuconychia totalis either hereditary or acquired is a rare
entity. Only six cases of idiopathic,
acquired, true leukonychia totalis
without hereditary cause has been found in literature8, whereas only two cases of sporadic congenital true leuconychia totalis reported in
literature9 before our case.
One of which was associated with deafness and vitiligo
while the other was isolated, like our case.
It is importance to report this case not only because the entity is rare
but also it may have associated abnomalties. If no
association is detected, proper counselling of the
patient regarding the benign nature of the condition should suffice.
REFERENCES:
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“leuconychia: review and classification, ”International journal of Dermatology,vol.29,
no.8, pp.535-541,1990.
2 A. Yoruk and
H. Yukselgungor, “ Chemotherapy induced transverse leukonychia in children,” International Journal of Dermatology, vol.42,no.6, pp.468-469,2009.
3 L.L.Bushkell
and R.J.Gorlin, “leukonychia
totalis, multiple sebaceous cysts,and
renal calculi. A syndrome,” Archives of
Dermatology,vol.111,no.7, pp.
899-901,1975.
4 R.S. Medansky
and J.M. Fox , “Hereditary Leukonychia totalis,”Archives of Dermatology, vol.82, no.3, pp.412-414,1960.
5 R.S. Bart and R.E Pumphrey,
“knuckle pads,leukonychia and deafness. Adominantly
inherited syndrome, ” The New England Journal of Medicine, vol. 276, no. 4, pp. 202-207,1967.
6 K.R Ong, S. Visram,S. McKaig, and L.A.Brueton, “Sensorineural deafness,enamel abnormalities and nail abnormalities: a
case report of Heimler syndrome in identical twin girls,” European Journal of Medical Genetics,vol.49,no.
2, pp.187-193,2006.
7 R.A. Mee, “Een verschijnsel bij polyneuritis arsenicasa,” Nederlands Tijdschrift voor Geneeskunde,vol.1,pp.391-396,1919
8 Maria Rita Bongiorno
and Mario Arico,Idiopathic Acquired Leuconychia in 34-Year Old Patient : Case Report in Medicine , Volume 2009, Article ID 495809, 3pages
,doi:10.1155/2009/495809.
9 Milano A , and Bonifazi
E, “Sporodic congenital leukonychia
totalis .Two cases,
Eur. J. Pediat
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Received on 29.12.2012
Modified on
Accepted on
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Research J. Pharmacology and
Pharmacodynamics. 5(5): September–October 2013, 289-290