A Case of Recessive X‐Linked Ichthyosis: Scale‐Specific Abnormalities of Lipid Composition May Explain the Pathogenesis of the Skin Manifestation

Hamanaka, Sumiko; Ujihara, Mayumi; Serizawa, Shou; Nakazawa, S.; Otsuka, Fujio

doi:10.1111/j.1346-8138.1997.tb02763.x

Cited by 5 publications

(2 citation statements)

References 16 publications

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“…A numberof skin pathologies directly cause structural alterations in the stratum corneum as a result of malfunction of epidermallipid metabolism, resulting in a loss of barrier function. In this respect, fue most cited skin pathologies are psoriasis, [32,113,[143][144][145][146][147] atopic dermatitis, [146,[148][149][150][151][152][153][154]] some forms of ichthyosis, [98,[155][156][157][158][159] and severe xerosis. [123,128,160,16I] 4.3.2 Atopic Dermatitis Atopic dermatitis ski n tends to be easily irritated and appears to be dry.…”

Section: Pathological Ski N Disordersmentioning

confidence: 99%

Ceramides and Skin Function

Coderch

López²,

Maza³

et al. 2003

American Journal of Clinical Dermatology

331

246

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Ceramides are the major lipid constituent of lamellar sheets present in the intercellular spaces of the stratum corneum. These lamellar sheets are thought to provide the barrier property of the epidermis. It is generally accepted that the intercellular lipid domain is composed of approximately equimolar concentrations of free fatty acids, cholesterol, and ceramides. Ceramides are a structurally heterogeneous and complex group of sphingolipids containing derivatives of sphingosine bases in amide linkage with a variety of fatty acids. Differences in chain length, type and extent of hydroxylation, saturation etc. are responsible for the heterogeneity of the epidermal sphingolipids. It is well known that ceramides play an essential role in structuring and maintaining the water permeability barrier function of the skin. In conjunction with the other stratum corneum lipids, they form ordered structures. An essential factor is the physical state of the lipid chains in the nonpolar regions of the bilayers. The stratum corneum intercellular lipid lamellae, the aliphatic chains in the ceramides and the fatty acids are mostly straight long-chain saturated compounds with a high melting point and a small polar head group. This means that at physiological temperatures, the lipid chains are mostly in a solid crystalline or gel state, which exhibits low lateral diffusional properties and is less permeable than the state of liquid crystalline membranes, which are present at higher temperatures. The link between skin disorders and changes in barrier lipid composition, especially in ceramides, is difficult to prove because of the many variables involved. However, most skin disorders that have a diminished barrier function present a decrease in total ceramide content with some differences in the ceramide pattern. Formulations containing lipids identical to those in skin and, in particular, some ceramide supplementation could improve disturbed skin conditions. Incomplete lipid mixtures yield abnormal lamellar body contents, and disorder intercellular lamellae, whereas complete lipid mixtures result in normal lamellar bodies and intercellular bilayers. The utilization of physiological lipids according to these parameters have potential as new forms of topical therapy for dermatoses. An alternative strategy to improving barrier function by topical application of the various mature lipid species is to enhance the natural lipid-synthetic capability of the epidermis through the topical delivery of lipid precursors.

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Section: Pathological Ski N Disordersmentioning

confidence: 99%

Ceramides and Skin Function

Coderch

López²,

Maza³

et al. 2003

American Journal of Clinical Dermatology

331

246

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show abstract

“…Several skin conditions with perturbed barrier function have been demonstrated to have abnormal lipid composition such as Xlinked ichthyosis (Hamanaka et al, 1997). A disrupted lipid organization has also been demonstrated to result in a decreased barrier function in psoriasis and atopic dermatitis (Fartasch, 1997).…”

mentioning

confidence: 99%

Barrier Characteristics of Different Human Skin Types Investigated with X-Ray Diffraction, Lipid Analysis, and Electron Microscopy Imaging

Schreiner

Gooris

Pfeiffer

et al. 2000

Journal of Investigative Dermatology

122

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The stratum corneum requires ceramides, cholesterol, and fatty acids to provide the cutaneous permeability barrier. The lipids are organized in intercellular membranes exhibiting short- and long-periodicity lamellar phases. In recent years, the phase behavior of barrier lipid mixtures has been studied in vitro. The relationship of human stratum corneum lipid composition to membrane organization in vivo, however, has not been clearly established. Furthermore, the special function of the different ceramide species in the stratum corneum is largely unknown. We examined lipid organization and composition of stratum corneum sheets from different subtypes of healthy human skin (normal, dry, and aged skin). Lipid organization was investigated using X-ray diffraction and demonstrated that the 4.4 nm peak attributed to the long periodicity phase was frequently missing for skin with a low Cer(EOS)/Cer(total) ratio, indicating an important part for Cer(EOS), which contains omega-hydroxy fatty acid (O) ester-linked to linoleic acid (E) and amide-linked to sphingosine (S). A deficiency in the 4. 4 nm peak was predominantly observed in young dry skin. In one case of aged skin, however, and less often in young normal skin this peak was also missing. Furthermore, the ceramide composition of samples without the 4.4 nm peak showed a deficiency of Cer(EOH), which contains 6-hydroxy-4-sphingenine (H), and an increase in Cer(NS) and Cer(AS), which contain nonhydroxy (N) or alpha-hydroxy fatty acids (A). In addition, a 3.4 nm peak attributed to crystalline cholesterol occurred in most cases of aged and dry skin, but was not observed in young normal skin. Our results do not indicate a definite pattern of correlation between lipid organization and types of human skin. They demonstrate, however, that Cer(EOS) and Cer(EOH) are key elements for the molecular organization of the long periodicity lamellar phase in the human stratum corneum.

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FTIR spectroscopic analyses of the temperature and pH influences on stratum corneum lipid phase behaviors and interactions

Mimeault

Bonenfant

2002

Talanta

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A Case of Recessive X‐Linked Ichthyosis: Scale‐Specific Abnormalities of Lipid Composition May Explain the Pathogenesis of the Skin Manifestation

Cited by 5 publications

References 16 publications

Ceramides and Skin Function

Ceramides and Skin Function

Barrier Characteristics of Different Human Skin Types Investigated with X-Ray Diffraction, Lipid Analysis, and Electron Microscopy Imaging

FTIR spectroscopic analyses of the temperature and pH influences on stratum corneum lipid phase behaviors and interactions

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