# Idiopathic Acquired Leukonychia in a 34-Year-Old Patient

Authors: Maria Rita Bongiorno; Mario Aricò
Journal: Case Reports in Medicine
 (2009)

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## Abstract

We present a rare case of a 34-year-old patient with persistent, progressive, acquired leukonychia
totalis and partialis. Idiopathic acquired leukonychia is a rare chromatic disorder of the nail not associated with other abnormalities and discernible etiology. Our case report did not link the inheritance of leukonychia with diverse clinical syndromes. To our knowledge, only five cases of idiopathic, acquired, true total leukonychia were found in  literature. This case was the sixth patient with asymptomatic idiopathic, white fingernails, and toenails without a hereditary cause.


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## Body

## 1. Introduction

Leukonychia refers  to whiteness of the nails which can
occur either  in patches or involving the entire nail [1]. The condition can be either true
leukonychia, with involvement of the nail plate, or pseudoleukonychia, caused by subungual and nailbed
abnormalities.Leukonychia may be acquired or inherited. Acquired
leukonychia is frequently associated with trauma, drugs such as chemotherapeutic agents, [2]
systemic or local infections—such as typhoid fever, hepatic cirrhosis, ulcerative colitis or
leprosy, hypocalcaemia, and, very commonly, minor trauma. True leukonychia can be inherited as an
isolated condition or as one of several other reported syndromes. There are a number of autosomal,
dominantly inherited leukonychia syndromes, including leukonychia totalis, in which
leukonychia occurs in combination with kidney stone and sebaceous cysts [3, 4],  as well as
leukonychia, with sensory-neural deafness and knuckle pads, known as the Bart-Pumphrey
syndrome [5, 6]
(Table 1).Table 1
Classification of true leukonychia.
(A) Hereditary(1) Isolated(2) Associated with(a) Bart-Pumphrey syndrome(b) Bauer syndrome(c) Heimler syndrome(d) Knuckle pads-leukonychia-deafness syndrome(e) Keratoderma, hypotrichosis and leukonychia totalissyndrome(f) Lowry-Wood syndrome(g) FLOTCH syndrome(h) Congenital keratosis palmaris et plantaris,
deafnessand total leukonychia(i) LEOPARD syndrome(B) Acquired(1) Idiopathic(2) Associated with(a) Trauma(b) Drugs(c) Systemic infections(d) Local infections(e) Inflammatory disease.Classification of the so-called true leukonychia may also
be based on the distribution of white blotches, known as leukonychia punctata, leukonychia striata,
leukonychia partialis, or leukonychia totalis. Idiopathic true leukonychia is a much rarer condition,
with only a few reported
[7–11] cases. The following is the case history of a man with
persistent, progressive idiopathic leukonychia.


## 2. Case Presentation

The 34-year-old male patient was admitted in the
Department of Dermatology, University of Palermo (Italy), complaining of color changes on the
nails. The symptoms were observed synchronously and had apparently been present for 11
years. When the patient was 23, he developed simultaneous leuconychia partialis of both
the fingers and toenails. There was no evidence of atopy, lichen planus, alopecia areata, or
psoriasis.The patient was without a previous medical history. He
reported not having taken any drug specific and not to be exposed to chemical agents. He also had
no peripheral neurovascular disorders. On examination, striata and total leukonychia of all
twenty nails was found
(Figure1). Further clinical
examination revealed soft nails with slow growth,
without other cutaneous or visceral
abnormalities.(a)-(b) Both hands had opaque nail plates
characteristic of leukonychia totalis.
(c)-(d) The patient  toenails exhibited
leukonychia
totalis and partialis.
(a)(b)(c)(d)A progression over the years from leuconychia
partialis to leuconychia totalis was demonstrated. On examination, the patient had no central nervous
system, eye, ear, hair, teeth, or skin
abnormalities.There was no family history of nail disease, the
patient was born to nonconsanguineous parents and had two older siblings, a brother and sister, without
any features of the disease. There was no family history of atopy, psoriasis, lichen planus,
alopecia areata, or any other illnesses. Repeated potassium hydroxide preparations and fungal
cultures of the white nails were negative.


## 3. Discussion

Leukonychia is a whitening of the nail plate. It was
first described by Mees in 1919, as an associated finding in arsenic intoxication  [12].The physiologic mechanism leading to this phenomenon
is not entirely clear. According to Newton's theorem, a surface appears white when it
reflects the radiation of visible light. This mechanism can be proposed in explaining leukonychia.
Because true leuconychia is thought to be due to abnormal matrix keratinization, with persistent
parakeratosis and keratohyaline granules in the nail plate, parakeratosis and
dissociation of the keratin bundles may play a role in the modification of the solar light reflection by the
ungueal plates.Several studies have provided evidence for the
association of total leukonychia with diverse clinical syndromes, including leukonychia with
palmoplantar keratoderma and atrophic fibrosis, pili torti [13], congenital hypoparathyroidism,
hypoparathyroidism, onychorrhexis, and cataracts and the LEOPARD syndrome [1].  Total leukonychia has
also been associated with peptic ulcer disease and cholelithiasis as well as with keratoderma
and hypotrichosis [14].Our case report did not link the inheritance of
leukonychia with any of the above-mentioned syndromes. To our knowledge, only five cases of
idiopathic, acquired, true total leukonychia were found in  literature
(Table2). This  case was the
sixth patient with asymptomatic idiopathic, white fingernails and toenails without a hereditary
cause.Table 2
True acquired idiopathic leukonychia.
AuthorsPresentationsActive disease (years)Claudel et al. [8]Leukonychia totalis and partialis2Grossman et al. [1]Leukonychia partialis to a
combined partialis and totalis3Stewart et al. [7]Leukonychia totalis and partialisUnrelatedPark et al. [10]Leuconychia partialis to leuconychia totalis13Butterworth [11]Leukonychia totalis and partialisUnrelatedOur caseLeuconychia partialis to leuconychia totalis11



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