Hereditary twenty-nail dystrophy in a Sicilian family.

SUMMARY Over three successive generations four members of a Sicilian family showed early onset, slowly progressive dystrophy of the finger and toe nails, without any other anomalies or skin signs. Histological examination performed in two patients enabled us to discount lichen planus and other underlying diseases. The hereditary and idiopathic nature of the disease is underlined and a classification of the dystrophies affecting all the 20 nails is proposed. In 1977 Hazelrigg et a1l described 'twenty-nail dystrophy of childhood', an apparently idiopathic disease characterised by dystrophic changes of all 20 nails in children entirely free of other skin, hair, and teeth abnormalities. The idiopathic nature of this disorder has been questioned by other workers, who believe it is an expression of alopecia areata2 3 or lichen planus4 confined to the nails. Even if most cases of this disease are sporadic, some families have been reported in whom a clinically identical disease to that described by Hazelrigg et all was transmitted through successive generations with an autosomal dominant pattern of inheritance.5-8 In this paper we report a family with four affected members in three successive generations. Nail biopsy was performed in two patients. Our results suggest that a hereditary form of twenty-nail dystrophy exists and is a distinct clinical entity. Case reports THE PROBAND The proband (fig 1, 111.2), a 17-year-old male farmer, was admitted to the Dermatology Department of the University of Catania because of nail loss (fig 2). At birth his parents had noticed that his nails were uniformly thin and opalescent with longitudinal striations. Lamellar splitting of all nails occurred over the following years until there was almost complete nail loss at 10 years. Apart from the nails, his past history was unremarkable. On examination there were no signs of eczema, lichen planus, or psoriasis on the skin or mucous membranes. Hair and teeth were also normal. No alterations of the patellae or other bones were present. Cultures for dermatophytes and yeasts were repeatedly negative.