Idiopathic total leukonychia involving fingernails: A report of two cases

Congenital true leukonychia totalis

“…Family history was elicited in nearly 79% of the cases. 2 Although, hereditary leukonychia is predominantly inherited in an autosomal dominant pattern, there are a few case reports of autosomal recessive inheritance as reported by Frydman et al 3 Mutations in PLCD1 gene on chromosome 3p21.3-p22 was identified as cause of hereditary leukonychia totalis. This PLCD1 gene is localized to nail matrix and encodes phosphoinositide specific phospholipase C delta 1 subunit which is important enzyme in phosphoinositide metabolism required for molecular control of color and growth of nail.…”

“…However, it has been proposed that it is attributable to reflection of light from large keratohyalin granules present in the parakeratotic cells of the ventral portion of nail plate thus preventing the visualisation of normal vascular tissue underlying the nail plate. 2,3 Unna classified leukonychia into five clinical types based on the distribution of whiteness leukonychia totalis, leukonychia partialis, leukonychia striata, leukonychia transversalis, and leukonychia punctata. However, Butterworth proposed that leukonychia partialis as a phase of leukonychia totalis, and not a distinct entity.…”

“…However, Butterworth proposed that leukonychia partialis as a phase of leukonychia totalis, and not a distinct entity. 3 Leuconychia is also classified in to true leukonychia, where the pathology is in the nail plate, apparent leukonychia, where the pathology is in the subungual tissue and pseudo-leukonychia due to external factors such as trauma. 3 Leukonychia totalis is a relatively rare condition.…”

“…1,2 Leukonychia totalis is associated with multiple systemic diseases such as Bart Pumphrey syndrome, Buschkell-Gorlin syndrome, LEOPARD syndrome diabetes, liver cirrhosis, renal failure, cardiac failure, gall stones, renal calculi, duodenal ulcers, pili torti, trichilemmal cyst, sebaceous cyst, congenital hyperparathyroidism, Hodgkin's lymphoma, epiphyseal dysplasia syndrome, mental retardation, etc. 1,3,4 However, there are very few reports in the literature on sporadic congenital leukonychia totalis without any systemic abnormalities.…”

“…2 Hereditary leukonychia is associated with the mutations in PLCD1 gene on chromosome 3p21.3-p22 encoding an important enzyme in phosphoinositide metabolism responsible for control of color and growth of nail. 3 Here we report a case a 24 years old lady with asymptomatic complete white discoloration of all the nails (congenital true leukonychia totalis) with family history and with absence of associated conditions.…”