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.
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Article info
Publication history
Published online: February 14, 2022
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