Onychogryphosis in tuberous sclerosis complex: an unusual feature

Abstract

Onychogryphosis is an acquired nail plate change. It often affects the toenail and is characterized by an opaque, yellow-brownish nail plate that is distorted, grossly thickened, elongated, and partly curved resembling a ram's horn. Tuberous sclerosis complex is a multisystem disorder associated with high rates of mental retardation, autism, cognitive impairment, behavioral problems, or seizures. Nail disease can also be associated, which is a concern to patients due to pain and nail distortion. We reported a typical tuberous sclerosis complex patient with distinctive clinical features of a ram's horn nails, which presented a great challenge to surgical treatment and nail restoration.

Onychogryphosis: Case Report and Review of the Literature

“…Haim-Munk syndrome is an autosomal recessive genodermatosis caused by mutations in a lysosomal protease gene, cathepsin C [12]. Onychogryphosis has also been rarely reported to be associated with tuberous sclerosis, which may be due to poor nursing care, cognitive impairment, or subungual fibromas [13]. There have been reports of onychogryphosis as a feature of several congenital syndromes, including Papillon-Lefèvre syndrome [14], ichthyosis hystrix [15], and ectodermal dysplasia-syndactyly syndrome type I [16].…”

Dermoscopy of subungual red comets associated with tuberous sclerosis complex

“…Patients affected by tuberous sclerosis complex (TSC) may have different nail abnormalities, including periungual or subungual fibromas, longitudinal leukonychia, onychogryphosis, cuticular hyperkeratosis, splinter hemorrhages, and subungual red comets (SRCs) . We report here our experience with the dermoscopy of SRCs.…”