Hereditary ciliary and superciliary hypotrichosis of a dominant character.

CONGENITAL lack of lashes and eyebrows, whether total or partial, is seldom encountered. Associated at times with other developmental defects, mostly ectodermal, it may appear in conjunction with general alopecia, faulty dentition, and/or cataract formation (Duke-Elder, 1952). In the so-called Ullrich-Dohna syndrome, it has been seen in combination with multiple ocular malformations, strabismus, micrognathia, trigonocephaly, and hypogenitalism (Ullrich and Fremerey-Dohna, 1953; Weyers, 1954; Leffertstra, 1956); the disease is not hereditary. In one instance, at least, it has also been observed as part of a complex syndrome consisting of retinitis pigmentosa, opacities of the lens, anisocoria, oligophrenia, and some degree of somatic hypotrophy (Sanchez Salorio, 1955); the affection was transmitted according to a simple recessive pattern. Its early occurrence in cases of hyperkeratosis follicularis spinulosa decalvans (Siemens, 1926) has likewise been recorded (Wessely, 1929); in this condition, which shows a sex-linked recessive mode of inheritance (Holthuis, 1943; Jonkers, 1950), fine, superficial punctate opacities of the cornea have been described, which, according to Franceschetti, Rossano, and Jadassohn (1956), Franceschetti, Jaccottet, and Jadassohn (1957), and Sendi (1957), occur primarily and not as a consequence of the scarcity of cilia. As an almost isolated anomaly, ciliary alopecia is still less common, since, as far as we know, it has been observed but once (Traquair, 1912), in a father and son, in whom not only the eyelashes but the intermarginal area of the lower lid was absent. On account of its considerable rarity, we feel justified in presenting the pedigree of a family in which the defect was strictly localized to the eyebrows and lashes and appeared in three generations (Figure). All the members of this family except the affected subject in the first generation were seen personally.