Triangular lunulae. A clue to nail-patella syndrome

Abstract

The nail-patella syndrome has been of interest to dermatologists because the diagnosis of this multisystem complex may be suggested simply by observing the patient's abnormal nails. It is the purpose of this article to call attention to triangular lunulae and other nail abnormalities associated with this syndrome, as well as other causes of triangular lunulae.

Renal involvement in nail-patella syndrome: report of three cases

“…In a retrospective study of 123 cases of NPS reported in the literature by Meyrier et al, 60% of patients presented with non-nephrotic range proteinuria. The proteinuria may remain asymptomatic, remit spontaneously, or may progress to nephrotic syndrome in less than 20% of the patients [8,10,11]. In this study, 15% of the patients progressed to ESRD.…”

A novel mutation in LMX1B gene in a newborn with nail‐patella syndrome: Clinical and dermoscopic findings

“…NPS is a rare autosomal dominant disorder characterized by a broad spectrum of clinical manifestations with variable presentation 2‐5 . The “classic” tetrad includes patellar or elbows dysplasia, presence of iliac horns and nail dystrophy 2,3 .…”

“…Renal involvement with proteinuria occurs in 30%‐50% of cases and can lead to severe glomerulonephritis. Nail alterations occur more frequently on the fingernails as hypoplasia, thinning, fragility, longitudinal/horizontal ridging, pitting, clefting, or discoloration 2‐4 . The triangular or V‐shaped lunula is the most specific sign 4,5 .…”

“…NPS is a rare autosomal dominant disorder characterized by a broad spectrum of clinical manifestations with variable presentation. [2][3][4][5] The "classic" tetrad includes patellar or elbows dysplasia, presence of iliac horns and nail dystrophy. 2,3 Renal involvement with proteinuria occurs in 30%-50% of cases and can lead to severe glomerulonephritis.…”

“…Nail alterations occur more frequently on the fingernails as hypoplasia, thinning, fragility, longitudinal/horizontal ridging, pitting, clefting, or discoloration. [2][3][4] The triangular or V-shaped lunula is the most specific sign. 4,5 The diagnosis of NPS is usually clinical but genetic testing can be helpful in uncertain cases.…”