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Harlequin fetus with abnormal lamellar granules and giant mitochondria
- Source :
- Journal of cutaneous pathology. 19(3)
- Publication Year :
- 1992
-
Abstract
- A case of harlequin ichthyosis was studied with electron microscopy. From the basal cell to granular cell layers, keratinocytes contained giant mitochondria (860 nm) which exhibited vesicular cristae. Typical lamellar granules were absent; instead, numerous dense cored granules (DCG) and particles containing cored granules (PCG) were produced. Some of these were discharged into the intercellular spaces of granular cells, but the majority failed to be released from the cytoplasm. These retained DCG and PCG coalesced to form large vacuoles and cavities in the stratum corneum. In the hair follicle, keratinized cells failed to loosen and desquamate into the hair canal; instead, they formed concentric keratin rings surrounding hair, a diagnostic feature of this disease. It is suggested that the abnormal lamellar granule underlies the pathogenesis of this disease and that giant mitochondria may be related to an abnormal lipid metabolism of keratinocytes which may affect lipid composition of lamellar granules.
- Subjects :
- Adult
Keratinocytes
Male
Pathology
medicine.medical_specialty
Histology
Hyperkeratosis
Dermatology
Lamellar granule
Biology
Cytoplasmic Granules
Pathology and Forensic Medicine
Fetus
Pregnancy
Keratin
medicine
Stratum corneum
Humans
chemistry.chemical_classification
integumentary system
Infant, Newborn
Harlequin Ichthyosis
Hair follicle
medicine.disease
Mitochondria
Microscopy, Electron
medicine.anatomical_structure
chemistry
Cytoplasm
Ultrastructure
Keratins
Female
Ichthyosis, Lamellar
Hair
Subjects
Details
- ISSN :
- 03036987
- Volume :
- 19
- Issue :
- 3
- Database :
- OpenAIRE
- Journal :
- Journal of cutaneous pathology
- Accession number :
- edsair.doi.dedup.....18c58899686598829b71264dee469378