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A critical study of 69 cases of collodion baby observed between 1976 and 1984 is compared with the study conducted in 1976. These 69 cases are divided into 32 examined by the authors personally and 37 published in the literature. The total number of collodion babies is 267. A collodion baby is a high risk newborn who must be nursed in a specialized intensive care unit during the neonatal period. The vital prognosis has been improved by the prevention of metabolic disorders (dehydration with hypernatraemia) and of percutaneous intoxication with topical products, and by the early treatment of infections. The mortality rate, which was 33 p. 100 in 1976, had fallen to 11 p. 100 in 1984. Skin infection with systemic spread remains the major complication (the present situation is summarized in table II). The collodion baby is the neonatal expression of different disorders of keratinization and as such, constitutes a syndrome. Several conclusions can be drawn from a comparison of the keratinization disorders observed in our two series (table VI). Congenital ichthyosiform erythroderma and lamellar ichthyosis are responsible for 60 p. 100 of collodion babies. These two lesions may be the first signs of an ichthyosis transmitted as an autosomal dominant trait in 9.7 p. 100 of the cases. Trichothiodystrophy may be heralded by a collodion baby syndrome. In 9.7 p. 100 of the cases this syndrome disappears without sequelae. Sex-linked ichthyosis never begins with a collodion baby syndrome.

Trichothiodystrophy is a congenital dysplasia of hairs characterized by: a pathognomonic image of the hair shaft under polarized light (the hair is plaited with alternately dark and light oblique bands) and trichoschisis with a clear-cut break; a deficiency of sulphurated aminoacids in the hair. We report a case where trichothiodystrophy was associated with abnormalities of the nails, teeth and eyes, growth and mental retardation, neurological syndrome and hypogonadism. From this case and a review of the cases published so far the following points emerge: 1. Congenital abnormalities of the neuroectodermal system are frequent. They include: skin lesions, such as ichthyosis, photosensitivity and atopic eczema; ungueal dysplasia; dysmorphic syndrome; growth and mental retardation; hypogonadism; neurological and ophthalmic abnormalities. 2. The condition seems to be transmitted as an autosomal recessive trait. A common element, dysplasia, enables us to include in the same nosological group as trichothiodystrophy a number of other pathological conditions, viz.: syndromes hitherto described under various names but almost identical, such as Brown's syndrome and BIDS syndrome (brittle hair, intellectual deficit, decreased fertility, small stature); IBIDS and PIBIDS syndromes which, in addition to the former, comprise ichthyosis and photosensitivity, as well as Tay's syndrome (characterized by the presence in all cases of ichthyosis associated with hair dysplasia); and perhaps some anecdotic cases with either a pathognomonic image under polarized light or a suggestive biochemical profile. Finally, since typical trichothiodystrophy has been reported in patients with Siemens' syndrome or with Marviesco-Sjögren syndrome, these two syndromes may perhaps also be classified in the same category, as borderline forms of the disease.