Labial talon cusp in a child with incontinentia pigmenti achromians: case report.

Incontinentia pigmenti achromians (IPA), first reported by Ito (Ito 1952), is a rare disease, involving the skin, hair, eyes, central nervous system, and musculoskeletal system. The cutaneous manifestations consist of macular hypopigmented whorls, streaks, and patches in a bilateral or unilateral distribution affecting almost any portion of the body surface. Central nervous system dysfunction, ocular and musculoskeletal anomalies also have occurred with significant frequency (Schwartz et al. 1977; Takematsu et al. 1983). IPA superficially resembles incontinentia pigmenti (IP), but IPA has been regarded as distinct and separate from IP (Jelinek et al. 1973; Takematsu et al. 1983). IP is seen almost exclusively in females and has been considered an X-linked inherited disease (Gorlin et al. 1976; Jelinek et al. 1973; Schwartz et al. 1977; Takematsu et al. 1983). On the other hand, the mode of inheritance of IPA remains unclear (Schwartz et al. 1977; Takematsu et al. 1983). IP is associated frequently with delayed tooth eruption, pegged or conical crowned teeth, missing teeth, and malformed teeth (Gorlin et al. 1964). IPA also may have associated dental anomalies, but only a few reports have been published. Browne and Byrne (1976) reported on an unusual form of dental dysplasia and the microscopic structure of the teeth in IPA. Happle and Vakilzadeh (1982) reported multiple dental cusps in both primary and permanent incisors. Bartholomew et al. (1987) reported congenital absence of a maxillary primary central incisor. This paper describes a patient with IPA having an abnormally shaped maxillary permanent central incisor.