Peer Reviewed

Photo Essay

An Atlas of Nail Disorders, Part 5

Volume 60 - Issue 3 - March 2020

AUTHORS:
Alexander K. C. Leung, MD
Clinical Professor of Pediatrics, University of Calgary; Pediatric Consultant, Alberta Children’s Hospital, Calgary, Alberta, Canada

Benjamin Barankin, MD
Dermatologist, Medical Director and Founder, Toronto Dermatology Centre, Toronto, Ontario, Canada

Kin Fon Leong, MD
Pediatric Dermatologist, Pediatric Institute, Kuala Lumpur General Hospital, Kuala Lumpur, Malaysia

 

CITATION:
Leung AKC, Barankin B, Leong KF. An atlas of nail disorders, part 5. Consultant. 2020;60(2):48-50. doi:10.25270/con.2020.03.00002

EDITOR’S NOTE: This article is part 5 of a 15-part series of Photo Essays describing and differentiating conditions affecting the nails. Parts 5 through 15 will be published in upcoming issues of Consultant. To access previously published articles in the series, visit the Consultant archive at www.Consultant360.com and click the “Journals” tab.

Anonychia

Anonychia refers to partial or complete absence of one or more fingernails and/or toenails. The anomaly can be complete or partial (rudimentary).1 The exact incidence is not known, but anonychia is believed to be rare.2,3

Anonychia can be congenital or acquired. Congenital anonychia may occur as an isolated anomaly (also known as simple anonychia or anonychia simplex) or, more often, as part of a syndrome or condition.4 Simple anonychia can be inherited as an autosomal dominant or autosomal recessive trait.5-8 Mutations in the R-spondin 4 gene (RSPO4) and peptidyl arginine deiminase 3 (PADI3) gene account for some of the cases.2,6,9,10 Congenital anonychia from prenatal exposure to phenytoin and warfarin has also been reported.11,12 Syndromes and conditions associated with anonychia include nail patella syndrome, Iso-Kikuchi syndrome, Coffin-Siris syndrome, Cooks syndrome, popliteal pterygium syndrome, DOOR (deafness, onychodystrophy, osteodystrophy, and developmental delay and intellectual disability [previously called mental retardation]) syndrome, Robinson syndrome, Pfeiffer syndrome, Bart syndrome, Sézary syndrome, Zimmermann-Laband syndrome, Hoyeraal-Hreidarsson syndrome, glossopalatine ankylosis syndrome, dyscephalic mandibulo-oculofacial syndrome, oto-onycho-peroneal syndrome, hypohidrotic and anhidrotic ectodermal dysplasias, anonychia with hypoplasia and dysplasia of the distal phalanges, total anonychia congenita with microcephaly, and onychodystrophy-deafness syndrome.1,3,6-8,12-18 Patients with congenital anonychia should be examined thoroughly for other anomalies.

Acquired anonychia may result from mechanical trauma to the nail matrix, severe infection, medications (eg, thiazide, azathioprine), Stevens-Johnson syndrome, lichen planus, acro-osteolysis, acral porokeratosis, scleroderma, and epidermolysis bullosa (as illustrated in Figures 1 and 2).3,5,16,19-24

Anonychia figure 1

Anonychia Figure 2

 

REFERENCES:

  1. Al Hawsawi K, Al Aboud K, Alfadley A, Al Aboud D. Anonychia congenita totalis: a case report and review of the literature. Int J Dermatol. 2002;41(7):​397-399. doi:10.1046/j.1365-4362.2002.01535_1.x
  2. Avhad G, Jerajani HR. Anonychia congenita. Indian Dermatol Online J. 2012;3(2):156. doi:10.4103/2229-5178.96729
  3. Brennan CB, Buehler T, Lesher JL Jr. Cooks syndrome: a case report and brief review. Pediatr Dermatol. 2013;30(4):e52-e53. doi:10.1111/j.1525-​1470.2011.01668.x
  4. Wasif N, Ahmad W. A novel nonsense mutation in RSPO4 gene underlies autosomal recessive congenital anonychia in a Pakistani family. Pediatr Dermatol. 2013;30(1):139-141. doi:10.1111/j.1525-1470.2011.01587.x
  5. Afsar FS, Karakuzu A. Total congenital anonychia. Pediatr Dermatol. 2014;​31(6):743-744. doi:10.1111/pde.12474
  6. Balta I, Kalkan G. A case report on autosomal recessive total congenital anonychia. Pediatr Dermatol. 2013;30(6):e268-e269. doi:10.1111/pde.​12143
  7. Ramassamy S, Gibikote S, George RE. Anonychia with absent phalanges and brachydactyly: a report of two unrelated cases. Indian J Dermatol Venereol Leprol. 2016;82(6):693-695. doi:10.4103/0378-6323.184198
  8. Rigopoulos D, Petropoulou H, Nikolopoulou M, Kalogirou O, Katsambas A. Total congenital anonychia in two children of the same family. J Eur Acad Dermatol Venereol. 2006;20(7):894-896. doi:10.1111/j.1468-3083.2006.​01585.x
  9. Hsu C-K, Romano MT, Nanda A, et al. Congenital anonychia and uncombable hair syndrome: coinheritance of homozygous mutations in RSPO4 and PADI3. J Invest Dermatol. 2017;137(5):1176-1179. doi:10.1016/j.jid.2016.​12.015
  10. Khalil S, Hayashi R, Daou L, et al. A novel mutation in the RSPO4 gene in a patient with autosomal recessive anonychia. Clin Exp Dermatol. 2017;42(3):​313-315. doi:10.1111/ced.13052
  11. Babu S, Agarwal N. Anonychia due to prenatal phenytoin exposure. J Assoc Physicians India. 2012;60:64.
  12. Qureshi A, Wong KY, Grant I. Congenital anonychia. J Hand Surg Eur Vol. 2016;41(3):348–349. doi:10.1177/1753193415583245
  13. Chatterjee D. Congenital anonychia and brachydactyly of the left foot—Cooks syndrome variant: case report and review of literature. Indian J Hum Genet. 2014;20(2):206-208. doi:10.4103/0971-6866.142914
  14. Damasco FM, Geskin LJ, Akilov OE. Nail Changes in Sézary syndrome: a single-center study and review of the literature. J Cutan Med Surg. 2019;​23(4):380-387. doi:10.1177/1203475419839937
  15. Ozdemir O, Tiftikcioglu YO, Karaaslan O, Ozdemir R, Kocer U. Total anonychia congenita: a rare heterogeneic disorder. Genet Couns. 2004;15(1):43-46.
  16. Sonthalia S, Das A. Congenital Non-syndromic partial anonychia. Indian Pediatr. 2015;52(12):1099.
  17. Tirelli LL, Luna PC, Cano R, Giraldo JP, Larralde M. Iso-Kikuchi syndrome: report of 3 pediatric cases. Actas Dermosifiliogr. 2018;109(5):e33-e36. doi:10.1016/j.ad.2017.06.014
  18. Ward SC, Savage SA, Giri N, Alter BP, Cowen EW. Progressive reticulate skin pigmentation and anonychia in a patient with bone marrow failure. J Am Acad Dermatol. 2017;77(6):1194-1198. doi:10.1016/j.jaad.2017.07.018
  19. Lam C, Vleugels RA. Images in clinical medicine. Epidermolysis bullosa acquisita. N Engl J Med. 2013;368(13):e17. doi:10.1056/NEJMicm1204895
  20. Mian A, Jorwal P. Congenital non-syndromic anonychia totalis with acroosteolysis. BMJ Case Rep. 2017;2017:bcr2017222743. doi:10.1136/bcr-2017-222743
  21. Pall A, Gupta RR, Gulati B, Goyal P. Twenty nail anonychia due to lichen planus. J Dermatol. 2004;31(2):146-147. doi:10.1111/j.1346-8138.2004.tb00527.x
  22. Parsapour K, Reep MD, Mohammed L, Church A, Shwayder T. Herlitz junctional epidermolysis bullosa presenting at birth with anonychia: a case report and review of H-JEB. Pediatr Dermatol. 2001;18(3):217-222. doi:10.1046/j.1525-1470.2001.018003217.x
  23. Rajesh G, Devan P, Keerthi S, Karthikeyan K. Acral porokeratosis associated with anonychia. Indian J Dermatol Venereol Leprol. 2018;84(1):81-82. doi:10.4103/ijdvl.IJDVL_940_16
  24. Singh S, Kumar S. Linear scleroderma with partial anonychia. Indian J Dermatol Venereol Leprol. 2009;75(6):623-625. doi:10.4103/0378-6323.57736

Next: Congenital Double Nail of the Fifth Toe

Congenital Double Nail of the Fifth Toe

Congenital double or rudimentary accessory nail of the little toe (Figure) is common in the Chinese, especially Han Chinese, population.1,2 The condition, however, is often underdiagnosed and rarely reported.2 Congenital double nail of the little toe has a autosomal mode of inheritance with incomplete penetrance and variable expression.2 Although not common, congenital double nail of the little toe has also been observed in persons from countries such as Norway, Switzerland, the Netherlands, Germany, Portugal, Belgium, Thailand, and Benin.2,3

Congenital double nail of the fifth toe

Clinically, a double or rudimentary accessory nail is abnormally wide and split or shows a longitudinal depression corresponding to a slight protuberance of the cuticle (Figure).2 The condition is usually asymptomatic. Occasionally, it may cause cosmetic concerns and, rarely, discomfort or pain when wearing tight-fitting shoes.1,2 A double or rudimentary accessory nail typically affects the little toe and is most often bilateral.1,2 Rarely, a double or rudimentary accessory nail of the middle finger has been described.3

The differential diagnosis of congenital double nail includes traumatic double nail, congenital onychodysplasia, and onychoheterotopia.4-9

Treatment is usually not necessary. For those who desire treatment, segmental excision of the entire accessory nail unit and surgical matricectomy may be considered.1-3

 

REFERENCES:

  1. Chi C-C, Wang S-H. Inherited accessory nail of the fifth toe cured by surgical matricectomy. Dermatol Surg. 2004;30(8):1177-1179. doi:10.1111/j.​1524-4725.2004.30351.x
  2. Haneke E. Double nail of the little toe. Skin Appendage Disord. 2016;1(4):​163-167. doi:10.1159/000443378
  3. Randhawa MA, Hussain S, Bilal M, Shabbir N, Qadir A, Ali N. A unique case of accessory/double nail of middle finger. Int J Surg Case Rep. 2018;​44:103-104. doi:10.1016/j.ijscr.2018.02.006
  4. Afshar A. Posttraumatic double nail deformity. J Hand Surg Am. 2012;​37(7):1511. doi:10.1016/j.jhsa.2012.05.008
  5. Chauhan CG, Diwana VK, Thakur JS. Traumatic double fingernail. J Hand Surg Eur Vol. 2007;32(4):477-478. doi:10.1016/J.JHSE.2007.02.207
  6. Lade NR, Jain DB, Singh AL. Post-traumatic acquired polyonychia: a rare occurrence. Dermatol Online J. 2012;18(6):10.
  7. Yadav S, Khullar G, Dogra S. Congenital onychoheterotopia involving multiple toe nails. J Am Podiatr Med Assoc. 2013;103(5):445-447. doi:10.7547/1030445
  8. Youn SH, Kwon OS, Park KC, Youn JI, Chung JH. Congenital onychodysplasia of the index fingers—Iso-Kikuchi syndrome. A case involving the second toenail. Clin Exp Dermatol. 1996;21(6):457-458. doi:10.1111/j.1365-2230.​1996.tb00157.x
  9. Zaouak A, Boufarguine S, Hammami H, Fenniche S. A tale of a double-nailed toe. Skin Appendage Disord. 2019;5(3):191-192. doi:10.1159/​000493402

Next: Nail Pitting

Nail Pitting

Nail pits are superficial punctate depressions of the dorsal nail plate surface (Figure).1 The condition results from focal areas of defective keratinization of the proximal nail matrix with production of foci of parakeratotic cells in the dorsal nail plate.1,2 As the nail grows outward, these parakeratotic cells are shed off from the nail plate, leaving distinct pits in the nail plate.3,4

Nail pitting

Generally, the distance of the pits from the proximal nail fold can provide an estimate of the time of the insult to the nail matrix, based on an average growth rate of 1 mm per month for toenails and 1 mm per 6 to 10 days for fingernails. Fingernails are more commonly affected than toenails.4,5

The most common cause of nail pitting is psoriasis. Nail disease is seen in 40% to 50% of patients with cutaneous psoriasis, and pitting occurs in approximately 70% of these patients.1 The pits of psoriasis are multiple, large, deep, and irregular in size, shape, and distribution.3,6 It has been proposed that 20 to 60 pits are suggestive of psoriasis, whereas greater than 60 pits are highly specific for psoriasis.3 Pseudo-pitting in the form of “oil drops” and “salmon patches” in the nail plate in psoriasis may be related to compact parakeratosis and psoriatic acanthosis beneath the nail plate giving rise to the yellow color (“oil drop” sign) and the dilated capillaries in the psoriatic plaque on the nail bed giving rise to the salmon-pink color.2

Pitting is by far the most common manifestation of alopecia areata apart from hair loss, with an average prevalence of 20%.7 The pits of alopecia areata are superficial, small, regular in size and shape, and with a gridlike distribution.4,6,7

Other causes of nail pitting include eczematous dermatitis, occupational trauma, chronic paronychia, trachyonychia, lichen planus, lichen nitidus, mycosis fungoides, dermatomyositis, chronic renal failure, reactive arthritis, Darier disease, secondary syphilis, pemphigus vulgaris, parakeratosis pustulosa, Langerhans cell histiocytosis, and sarcoidosis.2,4,5,7-11 Nail pitting can also be idiopathic and an isolated finding.

The underlying cause should be treated if possible. Nail pitting in isolation is benign and need not be treated.3

 

REFERENCES:

  1. Tan EST, Chong W-S, Tey HL. Nail psoriasis: a review. Am J Clin Dermatol. 2012;13(6):375-388. doi:10.2165/11597000-000000000-00000
  2. Di Chiacchio N, André J, Haneke E, Di Chiacchio NG, Fonseca Noriega L, Ocampo-Garza J. Pseudo-pitting of the nail in psoriasis. J Eur Acad Dermatol Venereol. 2017;31(7):e347-e348. doi:10.1111/jdv.14141
  3. Chu DH, Rubin AI. Diagnosis and management of nail disorders in children. Pediatr Clin North Am. 2014;61(2):293-308. doi:10.1016/j.pcl.2013.11.005
  4. Jadhav VM, Mahajan PM, Mhaske CB. Nail pitting and onycholysis. Indian J Dermatol Venereol Leprol. 2009;75(6):631-633. doi:10.4103/0378-6323.57740
  5. Adya KA, Inamadar AC, Palit A. “Pitted” lesions in dermatology. Int J Dermatol. 2017;56(1):3-17. doi:10.1111/ijd.13358
  6. Starace M, Alessandrini A, Piraccini BM. Nail disorders in children. Skin Appendage Disord. 2018;4(4):217-229. doi:10.1159/000486020
  7. Chelidze K, Lipner SR. Nail changes in alopecia areata: an update and review. Int J Dermatol. 2018;57(7):776-783. doi:10.1111/ijd.13866
  8. Ashena Z, Alavi S, Arzanian MT, Eshghi P. Nail involvement in Langerhans cell histiocytosis. Pediatr Hematol Oncol. 2007;24(1):45-51. doi:10.1080/​08880010601001362
  9. Ehsani AH, Nasimi M, Azizpour A, et al. Nail changes in early mycosis fungoides. Skin Appendage Disord. 2018;4(1):55-59. doi:10.1159/000478946
  10. Salem A, Al Mokadem S, Attwa E, Abd El Raoof S, Ebrahim HM, Faheem KT. Nail changes in chronic renal failure patients under haemodialysis. J Eur Acad Dermatol Venereol. 2008;22(11):1326-1331. doi:10.1111/j.​1468-3083.2008.02826.x
  11. Sehgal VN, Chatterjee K, Chaudhuri A, Verma P, Sharma S. Twenty-nail dystrophy and Darier’s (Darier-White) disease. Skinmed. 2015;13(4):313-315.