ISOLATED CONGENITAL NAIL CANDIDIASIS: Report of 6 Cases : The Pediatric Infectious Disease Journal

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ISOLATED CONGENITAL NAIL CANDIDIASIS

Report of 6 Cases

Sánchez-Schmidt, Julia M. MD*; Vicente-Villa, M. Asunción MD*; Viñas-Arenas, Miquel MD*; Gené-Giralt, Amadeu MD; González-Enseñat, M. Antonia MD*

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The Pediatric Infectious Disease Journal: October 2010 - Volume 29 - Issue 10 - p 974-976
doi: 10.1097/INF.0b013e3181ed6c25
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Abstract

Candida infections of the newborn are a group of uncommon disorders in which the infection can be acquired either in utero (congenital candidiasis) or during labor and delivery (neonatal candidiasis).

Neonatal candidiasis is the most common form of candidal infection in the newborn. This disorder usually develops from 3 to 7 days after birth and it is often manifested as oral thrush and diaper dermatitis.

In contrast, candida infection in utero is rare and can lead to congenital candidiasis, which appears within 12 hours to 6 days of birth. It includes a spectrum of disorders of variable clinical manifestations and evolution. Systemic congenital candidiasis is a severe life-threatening infection that can lead to a fatal evolution, especially in premature infants. In other instances, an isolated cutaneous involvement known as congenital cutaneous candidiasis (CCC) is noted. Nail candidiasis in the neonate is rare and has been usually associated to CCC.1–4 Few cases of congenital candida infection limited to nail plates have been described.5,6 We report 6 cases of congenital nail candidiasis without associated skin rash.

REPORT OF CASES

Cases were collected in a tertiary referral pediatric hospital in Barcelona during the period between January 1996 and December 2009. Six newborns, 4 females and 2 males, with isolated nail involvement as a manifestation of congenital candidiasis were reviewed (Table 1). Five infants were white and 1 girl was Arab. All infants were born by vaginal delivery, 4 at term and 2 preterm at 29 (case 4) and 36 (case 1) weeks of pregnancy. A vaginal candida infection was diagnosed during the third trimester of pregnancy in 3 cases but only 1 of them received specific treatment (case 1). Nail changes were detected at birth in 2 cases (cases 2 and 4), and shortly afterward (3 weeks–1.5 months of life) in the other cases. Involvement of several fingernails was present in all patients, and several toenails were also affected in 2 cases. The observed nail changes included the following: erythema, onycholysis, yellowish coloration and roughness of the nail plates, hyperkeratosis, and paronychia (Fig. 1A). None of the infants developed skin rash. Oral thrush developed in 2 cases (cases 3 and 4) and interdigital spreading in 1 (case 5) after the onset of nail involvement. Case 3 had thrombocytopenia (43,000 × 103 platelets/L) since birth but no systemic involvement with Candida sp was demonstrated. No associated symptoms or signs of immunodepression or chronic mucocutaneous disease were found in any of these cases.

T1-21
TABLE 1:
Description of Cases
F1-21
FIGURE 1.:
A, Clinical aspect of congenital nail candidiasis in case 6. B, Microscopic examination of nail clippings in case 4 with periodic acid-Schiff stain.

Microscopic examination of the nail clippings revealed invasion by pseudohyphae and candida-like yeasts in 4 cases (Fig. 1B). The presence of Candida albicans was confirmed in all cases through the culture of the nail plates. In Case 5, C. albicans was also isolated from interdigital swab culture.

A partial spontaneous improvement in several nail plates in case 1 was observed. Four cases received topical treatment with clotrimazole 1% cream, 1 with ciclopirox olamine 1% cream, and case 2 received both. Complete resolution of nail changes was observed during 4 to 8 months of follow-up after diagnosis in 5 cases without sequelae. Case 3 was lost to follow-up.

DISCUSSION

Candidal vulvovaginitis is a highly prevalent disorder during pregnancy (10%–35%) and therefore, Candida species are abundantly present in the neonatal environment. Candida species affect mainly skin and mucosa in newborn, however, nails are usually spared. Nevertheless, CCC is a rare condition and nail involvement occurs occasionally. As far as we could determine, only 9 cases have been reported with this association.4 Nail infection as the only clinical manifestation of congenital candidiasis is exceedingly rare and has been previously reported on only 2 occasions.5,6 Most such cases were in newborns delivered at 26 to 37 weeks of pregnancy. Systemic infection confirmed by a positive C. albicans blood culture was present in one of these cases. No associated immune dysfunction or chronic mucocutaneous candidiasis was detected in any of the reported cases. To our knowledge, no invasive candidal infection arising from nail candidiasis has been reported.

Nail changes secondary to congenital candida infection can be present at birth but also can develop between 2 to 6 weeks of life. These are clinically manifested as thickening, discolored changes with yellowish and whitish areas, horizontal ridging, pitted, roughened, opaque or slightly raised nail plates4 and paronychia.2 The nail changes usually resolve after 2 to 5 months without sequelae as the nail plate grows out. Some authors have postulated that the linear nail growth is faster in the neonatal period than in adult life, thus enhancing a fungal elimination.

Congenital candidiasis seems to be an intrauterine infection acquired during the last days of pregnancy. Candidal vulvovaginitis is often asymptomatic. An ascending infection by Candida sp occurs in fewer than 1%. After crossing the fetal membranes, C. albicans gives rise to chorioamnionitis and amniotic fluid contamination. Direct contact of the fetus with the infected material may be the cause of CCC and nail infection: flexion of fingers and fisting can result in entrapment of infected amniotic fluid around the nail bed.2Candida can produce onychomycosis from the surrounding periunguium by invasion of the nail plate through the hyponychial epithelium. In our series, candidal vaginitis during the third trimester of gestation was diagnosed in 3 mothers.

The diagnosis of nail candidiasis in infants is usually established by microbiologic tests, such as direct examination with potassium hydroxide and fungal cultures, and histopathologic study with periodic acid-Schiff stain.7C. albicans has been isolated in all reported cases in the literature as well as in our cases. Congenital nail candidiasis must be differentiated from other causes of congenital nail dystrophy such as congenital or hereditary syndromes (hereditary onycho-osteodysplasia, Bart syndrome, hereditary ectodermal dysplasias, congenital onychodysplasia of the index fingers) or those caused by drugs (phenytoin, valproic acid, carbamazepine, warfarin, alcohol abuse).

Topical treatment with cotrimazole2 and ciclopirox olamine6 have been used in CCC, but there is no clear-cut evidence that topical or systemic antifungal therapies are effective in the treatment of this infection. Isolated cases with spontaneous resolution have been reported, but antifungal treatment is usually recommended to avoid the spread of the infection, especially in premature infants. Some authors also recommend oral nystatin to eradicate organisms that might have seeded the gastrointestinal tract. Maternal antifungal treatment of vaginal candidiasis remains controversial because it does not achieve reliable protection against congenital candidiasis.

In conclusion, it is an uncommon and probably misdiagnosed condition which may be considered a specific late-onset clinical manifestation of CCC, usually associated with a good prognosis. Increased awareness regarding this peculiar clinical manifestation seems important to establish an early diagnosis, and to distinguish congenital nail candidiasis from other nail dystrophies observed during the neonatal period.

REFERENCES

1. Perel Y, Taïeb A, Fontan I, et al. Candidose cutanée congénitale: une observation avec revue de la littérature. Ann Dermatol Venereol. 1986;113:125–130.
2. Raval DS, Barton LL, Hansen RC, et al. Congenital cutaneous candidiasis: case report and review. Pediatr Dermatol. 1995;12:355–358.
3. Gibney MD, Siegfried EC. Congenital cutaneous candidiasis: a case report. Pediatr Dermatol. 1995;12:359–363.
4. Clegg HW, Prose NS, Greenberg DN. Nail dystrophy in Congenital cutaneous candidiasis. Pediatr Dermatol. 2003;20:342–344.
5. Arbegast KD, Lamberty LF, Koh JK, et al. Congenital candidiasis limited to the nail plates. Pediatr Dermatol. 1990;7:310–312.
6. Sardana K, Garg VK, Manchanda V, et al. Congenital candidal onychomycoses: effective cure with ciclopirox olamine 8% nail lacquer. Br J Dermatol. 2006;154:573–575.
7. Kurgansky D, Sweren R. Onychomycosis in a 10-week-old infant. Arch Dermatol. 1990;126:1371.
Keywords:

mycosis; congenital candidiasis; cutaneous candidiasis; nail dystrophy; newborn

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