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The keratinization disorder in collodion babies evolving into lamellar ichthyosis. Its possible relevance for
Insights
Collodion baby skin shows early ultrastructural changes in the stratum granulosum, preceding the epidermal changes seen in developing lamellar ichthyosis. These findings suggest a prenatal origin for some ichthyosis alterations.
Area of Science:
- Dermatology
- Genetics
- Histopathology
Background:
- Collodion baby is a rare neonatal condition characterized by a generalized membrane covering the skin.
- It can evolve into various forms of ichthyosis, a group of genetic skin disorders.
- Understanding the early skin pathology is crucial for diagnosing and managing these conditions.
Purpose of the Study:
- To investigate the light and electron microscopic features of collodion baby skin.
- To compare the ultrastructural findings in collodion baby skin with those in established lamellar ichthyosis.
- To determine if early signs of lamellar ichthyosis are present in collodion baby skin.
Main Methods:
- Light and electron microscopy were used to examine skin biopsies from two collodion baby girls.
- Histopathological analysis focused on the stratum corneum, stratum granulosum, and stratum spinosum.
- Mitotic activity in the epidermis was also assessed.
Main Results:
- Neonatal collodion skin showed a thick stratum corneum and a thin stratum granulosum with diminished tonofibrils and keratohyalin.
- Electron microscopy revealed pathological changes in the upper stratum corneum and underlying stratum granulosum.
- By 2 weeks, skin biopsies showed typical lamellar ichthyosis with hyperkeratosis, acanthosis, and increased mitotic activity.
Conclusions:
- Ultrastructural changes in the stratum granulosum, characteristic of lamellar ichthyosis, are present at birth in collodion babies.
- These changes occur before the epidermis shows increased mitotic activity, suggesting prenatal development of some ichthyosis features.
- Collodion baby represents an early stage of lamellar ichthyosis with identifiable ultrastructural abnormalities.
Abstract:
Two collodion baby girls with disorder evolving into lamellar ichthyosis were followed by light and electron microscopy. Light microscopically, the neonatal colloidion skin was characterized by a thick compact stratum corneum which was PAS positive in its upper two thirds, by a thin stratum granulosum and by a non-acanthotic stratum spinosum with normal mitotic activity. Electron microscopically, the upper stratum corneum appeared pathological, whereas the lower part was normal except for some minor parakeratosis. The main alterations in the underlying stratum granulosum were diminished tonofibrils and keratohyalin. Biopsy specimens taken at the age of 2 weeks were typical for lamellar ichthyosis and showed hyperkeratosis with focal parakeratosis, a thickened stratum granulosum in which the cellular content of keratohyalin and tonofibrils was moderately diminished, and acanthosis with increased mitotic activity. It appears that the ultrastructural changes of the stratum granulosum, seen in lamellar ichthyosis, are already present in the collodion skin of the newborn, at a time when the epidermis does not yet show an increase in mitotic activity.