A congenital disease in which the hairs exhibit beadlike or fusiform enlargements and become brittle. Onset usually at birth, with hair progressively thinning up to second month, when there is complete loss. Later onset, up to adult life, also reported. Other hairy regions than the head may also be affected, with resulting areas of alopecia. Aetiology unknown. Both sexes affected. Autosomal dominant inheritance, but families with recessive inheritance also described.
First described by Walter George Smith (1844-1932) in 1879. The first comprehensive description by Sabouraud in 1892. The term Sabouraud's syndrome was introduced by the English dermatologist Henry Radcliffe Crocker (1845-1909) in 1903.
- W. G. Smith:
A rare nodose condition of the hair.
British Medical Journal, London, 1879, 2: 291-292.
- R. Sabouraud:
Sur les cheveux moniliformes (Trichorhexies et monilethrix).
Annales de dermatologie et de veneréologie, Paris, 1892, 3: 781-793.
- H. R. Crocker:
Diseases of the skin. 3rd edition, London 1903. First edition in London 1888.