Abstract
Autosomal recessive congenital ichthyosis (ARCI) is a clinically and genetically heterogeneous
category of nonsyndromic ichthyosis. Nail changes in ARCI are generally frequent but
have been rarely reported and studied in the literature. This stimulated us to conduct
a study to describe nail changes in ARCI using a combined literature review and prospective
examination from March 2019 to August 2019 (6 months) in the Dermatology Department
of Habib Thameur Hospital, Tunis, Tunisia. A total of 25 patients with ARCI had a
clinical and dermatoscopic review. The mean age was 19.8 years (range, 1-43), with
a female predominance (17 women [68%] and 8 men [32%]). Seventy-two percent had nail
unit changes involving more than one nail, none had single nail disease, 64% had involvement
of fingernails, and 68% had involvement of toenails, with cases including periungual
hyperkeratosis (64%), xanthonychia (40%), pachyonychia (40%), macrolunula (36%), digital
clubbing (32%), and onychomycosis (24%). Rarer findings included pseudoainhum, transverse
leukonychia, longitudinal melanonychia, and subungual hemorrhages, each in one patient
(4%). There was a statistically significant increased frequency of nail changes in
adults over children (P = .001). Nail abnormalities statistically associated with ARCI were macrolunula,
periungual hyperkeratosis, xanthonychia, and pachyonychia. A comprehensive review
of the literature was performed, creating the first comprehensive review addressing
nail disease in ARCI.
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Clinics in DermatologyAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Revised nomenclature and classification of inherited ichthyoses: results of the First Ichthyosis Consensus Conference in Sorèze 2009.J Am Acad Dermatol. 2010; 63: 607-641
- Autosomal recessive congenital ichthyosis.Actas Dermo-Sifiliográficas Engl Ed. 2013; 104: 270-284
- Mutations in CERS3 cause autosomal recessive congenital ichthyosis in humans.PLoS Genet. 2013; 9e1003536
- The impact of nail disorders on quality of life.Eur J Dermatol. 2013; 23: 366-371
- Clinico-epidemiological study of congenital ichthyosis in a tertiary care center of Eastern India.Indian J Dermatol. 2017; 62: 606-611
- Postnatal 2- and 3-dimensional sonography of the skin and nail in congenital autosomal recessive ichthyosis correlated with cutaneous histologic findings.J Ultrasound Med. 2011; 30: 1437-1439
- Lamellar ichthyosis associated with pseudoainhum of the toes and eye changes.Clin Exp Dermatol. 2003; 28: 493-495
- Hourglass-like leuconychia in congenital lamellar ichthyosis.Br J Dermatol. 2004; 150: 619-620
- Congenital ichthyosiform erythroderma superimposed with chronic dermatophytosis: a report of three siblings.Pediatr Dermatol. 2016; 33: e6-e9
- Calpain 12 function revealed through the study of an atypical case of autosomal recessive congenital ichthyosis.J Invest Dermatol. 2017; 137: 385-393
- Successful experimental treatment of congenital ichthyosis in an infant.BMJ Case Rep. 2013; bcr2013008688
- Lamellar ichthyosis associated bilateral pseudoainhum of fingers and toes successfully treated with tazarotene.Dermatol Ther. 2017; 30: e12516
- Ichthyosis and dermatophyte fungal infection.J Am Acad Dermatol. 1991; 24: 321
- Congenital ichthyosis associated with Trichophyton rubrum tinea, imitating drug hypersensitivity reaction.Med Mycol Case Rep. 2020; 29: 15-17
- Lamellar ichthyosis, dwarfism, mental retardation, and hair shaft abnormalities.J Am Acad Dermatol. 1980; 2: 309-317
- Ichthyosis with an unusual constellation of ectodermal dysplasias.Clin Genet. 2008; 35: 455-461
- Acitretin-induced transverse leukonychia.Int J Dermatol. 2014; 53: e221-e222
- Acitretin-induced subungual hemorrhage.Int J Dermatol. 2007; 46: 494-495
- Cellular basis of secondary infections and impaired desquamation in certain inherited ichthyoses.JAMA Dermatol. 2015; 151: 285
- Ichthyosis associated with widespread tinea corporis: report of three cases.An Bras Dermatol. 2013; 88: 627-630
- Treatment of childhood dermatophyte infections with oral terbinafine.Pediatr Dermatol. 1995; 12: 53-54
- Twenty-nail dystrophy and ichthyosis vulgaris.Arch of Dermatol. 1981; 117: 316
- Macrolunula: case reports of patients with trauma-associated enlarged lunula and a concise review of this nail finding.Cureus. 2018; 10: e2998
- Techniques for toddlers: linear band incision for harlequin ichthyosis with associated compartment syndrome.Pediatr Dermatol. 2014; 31: 625-629
- Onychogryphosis: a report of ten cases.Skinmed. 2015; 13: 355-359
Article Info
Publication History
Published online: February 14, 2022
Identification
Copyright
© 2022 Elsevier Inc. All rights reserved.
