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Summary

There is a very limited number of treatments that can be utilized for nail disorders in children as topical drugs are often not effective and most systemic treatments are not approved for administration in children. This review will focus on management of the most common nail disorders in children.

Introduction

Content

Congenital Malalignment of the Big Toenail

Congenital malalignment of the big toenail is characterized by lateral deviation of the nail plate with respect to the longitudinal axis of the distal phalanx (Fig. 1). Congenital malalignment is possibly caused by an abnormality in the ligament that connects the matrix to the periosteum of the distal phalanx. The condition improves spontaneously with age, but may persist into adulthood and may be complicated by nail ingrowing.

 

Treatment

If the nail deviation is mild, the nail may overcome the initial slight embedding produced by the distal lip and sufficient normal nail may grow to the tip of the digit to prevent further secondary traumatic changes. If the deviation is severe, it may be corrected surgically before the age of 2 years under very light anesthesia and a proximal toe block. The nail apparatus is dissected from the bone and rotated medially into its correct axis.

Ingrown Toenails

Lateral ingrown nail is the final result of several factors, including:  i) lateral deviation of the nail plate with respect to the longitudinal axis of the digit (congenital malalignment of the big toenail); ii) congenital hypertrophy of the lateral nail folds; iii) improper cutting or manual removal of the distal edges of the nail plate (Fig. 2); and iv) hyperhidrosis of the feet, which facilitates nail plate softening and breaking.

Penetration of the lateral edges of the nail plate into the nail folds and the breakage of the nail plate with formation of sharp spicules produce pain and periungual inflammation (stage I). With time, the injured dermis of the nail fold gives rise to granulation tissue (pyogenic granuloma) (stage II). If the condition lasts longer, the granulation tissue induces the growth of a newly formed skin epithelium that partially covers the nail plate (stage III). Distal ingrown nail can be a consequence of congenital hypertrophy of the anterior nail fold, or overgrowth of distal soft tissue after nail loss. The hypertrophic distal nail fold appears as an asymptomatic lip that partially covers the nail plate. This abnormal growth of soft tissues may deviate the nail laterally and/or may cause nail embedding with acute inflammatory reaction and pain. The congenital form usually appears at birth or shortly thereafter and is frequently bilateral, regressing spontaneously in a few years.

Treatment

The first stage of treatment involves removing the embedded spicula, if applicable. The taping technique proposed by Arai and Haneke is a safe and painless method of treating ingrowing nails in children. A 10-15 mm wide anchor tape (Elastopore^®) is used to pull the bulging away from the nail plate, allowing nail growth to proceed without interference. The tape is replaced every day by the parents until sufficient nail has grown out. The tape should be applied starting from the diseased lateral nail fold and then around the fingertip diagonally thus pulling the nail fold away from the offending nail edges. The other end of the tape must be fixed on the dorsal side of the finger/toe and a further surgical tape is used to fix the previous one to the nail plate and skin.   In the second stage topical steroids (clobetasol propionate once a day under occlusion) and topical antibiotics (fusidic acid once a day) are used to reduce the granulation tissue. The taping technique described above can then be used.   The third stage involves performing chemical matricectomy (using 88% phenol or NaOH) of the lateral horn of the matrix. This method has also been shown to be safe and effective in the pediatric population.

Psoriasis

Nail involvement in children with psoriasis is less common than in adults. Psoriasis limited to the nails is rare. Pitting is the most common symptom: pits are typically large, deep and irregularly distributed (Fig. 3). Pits in the toenails, which are exceptional in adults, may occur. Other signs of nail psoriasis include onycholysis with erythematous border and nail thickening due to subungual hyperkeratosis.

 

Treatment

There are no effective treatments for pitting in children since intralesional steroids are too painful and systemic treatments are not indicated. For subungual hyperkeratosis possible choices include topical emollients (urea), vitamin D₃ derivates twice a day, and tazarotene 0.05% gel once a day.

Parakeratosis Pustulosa

Parakeratosis pustulosa is a nail condition exclusive to children and most commonly affects girls aged 5-7 years more than boys. The condition is usually limited to one digit, most commonly a finger, especially the thumb and the middle finger (Fig. 4). The nail shows psoriasiform abnormalities with onycholysis and mild subungual hyperkeratosis. The abnormalities are often more marked on one side of the nail. Mild pitting may be associated. The distal pulp may be normal or show mild erythema and scaling. Pustules are almost never seen.  Parakeratosis pustulosa is a benign condition that usually regresses spontaneously as the child grows up. Some children may develop mild nail psoriasis afterwards.

 

Treatment

Treatment includes topical emollients (urea) and tretinoin 0.05% cream twice a day.

Lichen Planus

In children nail lichen planus is rare, but more common than skin lichen planus. It is possibly underestimated due to the reluctance to perform nail biopsies in children. It affects boys more frequently than girls and it is usually not associated with skin or mucosal signs of the disease. Three different clinical presentations may be seen. The first is typical nail lichen planus in which the nail abnormalities are usually mild with nail thinning associated with longitudinal ridging and splitting (Fig. 5). Nail bed involvement with distal onycholysis is often associated. Severe onychorrhexis and pterygium are very rare. The second is trachyonychia, in which the nail plate surface is abnormal due to excessive longitudinal ridging that causes roughness and opacity. Trachyonychia may affect all nails and is therefore also called "twenty nail dystrophy". The third type of clinical presentation is idiopathic atrophy of the nails. This is the rarest form and the nails are rapidly destroyed with or without pterygium formation.

 

Treatment

The treatment for typical nail lichen planus is systemic steroids (intramuscular triamcinolone acetonide 0.3-0.5 mg/kg/month for 3-6 months). No treatment is required for trachyonychia. The condition improves spontaneously as the child grows up in most cases. In idiopathic atrophy of the nail treatment is useless, since the nails have already been destroyed.

Onychomycosis

Dermatophytes

Onychomycosis due to dermatophytes is uncommon in children and extremely rare before the age of 6 years. Distal subungual onychomycosis due to Trichophyton rubrum is the most common type (Fig. 6). White superficial onychomycosis can be observed in prepubertal children, in which case it is due to T. rubrum. Clinically, it may appear to be a classical form or more commonly it may affect the nail plate more deeply and diffusely. The nail is homogeneously white, opaque and friable, resembling a proximal subungual onychomycosis extending to the superficial nail plate. Involvement of the whole thickness of the nail plate depends on the fact that the nail plate is thin in children. Tinea pedis is often associated.

Treatment

Topical antifungal medications are scarcely effective and require prolonged application; topical treatment can be considered only in white superficial onychomycosis or when the infection is limited to the distal nail. Treatment should be prolonged at least for 6-12 months and compliance is often a problem. Possible options include amorolfine 5% nail lacquer to be applied once or twice a week (not available in the USA); ciclopiroxolamine 8% nail lacquer to be applied daily; and bifonazole 40% urea cream, especially for thick nails (available only in a few European countries).   Systemic antifungals are effective but not approved for use in children. Clinical assessment is needed toassess the potential benefits and risks to the patient. Blood tests are mandatory at baseline and every 4-8 weeks in children on systemic therapy, including: terbinafine (weight < 20 kg: 62.5 mg/day; weight 20-40 kg: 125 mg/day; weight > 40 kg: 250 mg/day), 6 weeks for fingernails and 3 months for toenails; and itraconazole (5 mg/kg/day as pulse treatment 1 week per month), 6 weeks for fingernails and 3 months for toenails.

Candida

Candida onychomycosis may occur in healthy newborns and spontaneously resolve in a few months. Clinically, the nails show an opaque milky white discoloration. Nail infection has been associated with contamination during delivery due to vaginal candidiasis in the mother. Chronic mucocutaneous candidiasis often presents in childhood. One or most nails may be affected with paronychia and nail plate crumbling and discoloration. Thickening of the periungual tissues produces a bulbous appearance of the fingers.   Treatment Treatment options include itraconazole (200 mg/day as continuous treatment or 400 mg/day for 1 week per month) for 6 weeks to 3 months, and fluconazole (single weekly 3-6 mg/kg dose for 12-16 weeks forfingernails, and 18-26 weeks for the toenails). Drugs for the treatment of fungal infection in children should ideally be available in a tasty liquid formulation; however, oral antifungals are available as tablets or capsules that are difficult to swallow and cannot easily be divided into fractions to obtain the perfect dosage per weight. Itraconazole is available as an oral solution which, however, contains cyclodextrin that may cause diarrhea in children. Fluconazole is available as an oral suspension, but this drug has been approved for treatment of systemic fungal infections in children but not for onychomycosis. Strategies that can be utilized for administering systemic antifungals in children therefore include: i) chopping the tablet into small pieces and putting them into the chocolate cream of a chocolate-filled biscuit, or ii) opening the capsule and mixing the content with fatty food such as peanut butter, jelly or bread.

Melanonychia

Melanonychia describes the presence of melanin within the nail plate. Melanonychia can be due to melanocytic activation or to benign (lentigo, nevus) or malignant melanocytic hyperplasia (melanoma). In children melanocytic activation and nail melanoma are very rare and melanonychia is generally due to nail matrix nevi.   The clinical and dermoscopic features of nail matrix nevi in children are frequently alarming as it is common to see a pigmentation of the periungual tissues as well as a progressive enlargement and darkening of the band (Fig. 7). Thinning and fissuring of the pigmented nail plate may also occur. In some children it is possible to observe a gradual fading of the band that may disappear with time. Fading of the pigmentation is not an indication of regression of the nevus but just a sign of decreased melanin production.

Treatment

Management of longitudinal melanonychia in children is still a matter for discussion. In general, it is advisable to excise lesions that grow and darken. An incisional biopsy is not recommended as it may delay the diagnosis of nail melanoma. A shave biopsy of the nail matrix is possibly the best approach as it allows the examination of the whole lesion with minimal residual scarring.

Warts

Periungual warts are common in children and teenagers with a peak between the ages of 12 and 16 years. Nail biting facilitates occurrence and spreading of warts. They appear as hyperkeratotic papules on the proximal and lateral nail folds, or as a diffuse hyperkeratosis of the cuticle (Fig. 8). Subungual warts cause onycholysis that may sometimes have a linear pattern with splinter hemorrhages.

Treatment

Warts often disappear spontaneously, but recurrences are common. Aggressive approaches are unnecessary and may even cause enlargement of the wart. Keratolytic agents (salicylic acid in concentrations ranging from 10-40%) are the best first-line treatment if applied once a day. Immunotherapy with strong topical sensitizers (SADBE - squaric acid dibutylester or DPCP - diphenylcyclopropenone) can be an option for multiple recalcitrant warts. The objective of treatment is to induce mild contact dermatitis that causes wart regression through an immunological reaction. First, the patient is sensitized using 2% solution in acetone with a patch test for 48 hours. After 3 weeks, SADBE or DPCP is applied at a concentration ranging from 0.0001-0.1% and application is repeated once a week.   Freezing warts with liquid nitrogen is a rapid method of treatment. It is contraindicated in small children, since it is frequently associated with intense pain secondary to edema under the nail bed. Application of EMLA® (lidocaine and prilocaine cream) 1-2 hours prior to therapy is not useful in reducing pain in the periungual region. Cryosurgery should be performed with caution for warts on the proximal nail fold, since nail matrix damage is a common complication.

Conclusion

Tables & figures

References

Suggested Reading

- Arai, H., Arai, T., Nakajima, H., Haneke, E. Simple and effective treatment for ingrowing nail. Rinsho Derma (Tokyo) 2002, 44: 1321-8 (in Japanese) .

- Baran, R., Hay, R.J., Garduno, J.I. Review of antifungal therapy, part II: Treatment rationale, including specific patient populations. J Dermatolog Treat 2008, 19[3]: 168-75. - Braun, R.P., Baran, R., Le Gal, F.A. et al. Diagnosis and management of nail pigmentations. J Am Acad Dermatol 2007, 56[5]: 835-47.

- Daniel, C.R. 3rd, Iorizzo, M., Tosti, A., Piraccini, B.M. Ingrown toenails. Cutis 2006, 78[6]: 407-8.

- Diluvio, L., Campione, E., Paternò, E.J., Mordenti, C., El Hachem, M., Chimenti, S. Childhood nail psoriasis: a useful treatment with tazarotene 0.05%. Pediatr Dermatol 2007, 24[3]: 332-3.

- Ginter-Hanselmayer, G., Weger, W., Smolle, J. Onychomycosis: A new emerging infectious disease in childhood population and adolescents. Report on treatment experience with terbinafine and itraconazole in 36 patients. J Eur Acad Dermatol Venereol 2008, 22[4]: 470-5.

- Iorizzo, M., Tosti, A., Di Chiacchio, N. et al. Nail melanoma in children: differential diagnosis and management. Dermatol Surg 2008, 34[7]: 974-8. - Islam, S., Lin, E.M., Drongowski, R. et al. The effect of phenol on ingrown toenail excision in children. J Pediatr Surg 2005, 40[1]: 290-2.

- Nishioka, K., Katayama, I., Kobayashi, Y., Takijiri, C., Nishioka, K. Taping for embedded toenails. Br J Dermatol 1985, 113[2]: 246-7. - Richert, B., Choffray, A., de la Brassinne, M. Cosmetic surgery for congenital nail deformities. J Cosmet Dermatol 2008, 7[4]: 304-8.

- Yang, G., Yanchar, N.L., Lo, A.Y., Jones, S.A. Treatment of ingrown toenails in the pediatric population. J Pediatr Surg 2008, 43[5]: 931-5.