Clinical, histologic, and cell kinetic discriminants between lamellar ichthyosis and nonbullous congenital ichthyosiform erythroderma

Hazell, M.

doi:10.1001/archderm.121.4.489

Cited by 31 publications

(23 citation statements)

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“…Lipid droplets in the stratum basale of the epidermis were reported in eight patients (including the present case) with INLSD (3-6,8-10) but Oilred-O-or Sudan Ill-stained frozen section studies were not performed in several other cases (2,7), and the foamy vacuolation of the stratum basale (5,6,8,9) iniglit have been overlooked on Ihc hcnialoxylineosin-staincd skin biopsy seetions (2). Exeepl for llieso lipid vaeuoles, the ichlhyosis of patients with lNLSD is compatible with NIJCIH (11,12).…”

Section: Discussionmentioning

confidence: 73%

Ichthyosis and Neutral Lipid Storage Disease (Dorfman‐Chanarin Syndrome)

Venencie

Armengaud²,

Földes³

et al. 1988

Pediatric Dermatology

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Ichthyosis and neutral lipid storage disease (INLSD) is a nonlysosomal, multisystemic, triglyceride storage disorder. It is characterized by nonbullous congenital ichthyosiform erythroderma (NBCIE), leukocyte vacuoles, and variable involvement of the liver, muscles, eyes, and central nervous system. In our patient fat-containing vacuoles were also demonstrated in the epidermis. In patients with NBCIE, the diagnosis of INLSD is readily made by direct examination of a peripheral blood smear demonstrating cytoplasmic lipid vacuoles within most granulocytes and monocytes.

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Section: Discussionmentioning

confidence: 73%

Ichthyosis and Neutral Lipid Storage Disease (Dorfman‐Chanarin Syndrome)

Venencie

Armengaud²,

Földes³

et al. 1988

Pediatric Dermatology

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“…For comparison, a biopsy of normal buttock skin, incubated with tritiated thymidine for i h in vitro, gave a LI of unpublished), so the LI values reported here are slightly higher than for normal skin. Hazell and Marks (1985) suggested that a higher LI may be a useful indicator when taken with other parameters to differentiate CIE from lamellar ichthyosis. Unfortunately our data are not directly comparable due to the different labelling techniques used and the different methods used to calculate the LI.…”

Section: Discussionmentioning

confidence: 99%

Congenital non-bullous ichthyosiform erythroderma-cell kinetics before and after treatment with etretinate

et al. 1986

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Cell birth-rates and the labelling index (LI) were measured in a patient with congenital nonbullous ichthyosiform erythroderma (CIE). Measurements were made before and after a course of oral etretinate. The cell birth-rate, following treatment, was less than half of the pretreatment rate. The LI showed little change. The therapeutic effects of oral etretinate may be, to some extent, due to a suppression of cell renewal. The fact that the LI was unchanged when cell production was reduced indicates that the LI alone is an unreliable indicator of proiiferative activity.

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“…Previous studies indicated two variants of this disorder (28,29). Transit times were determined as between four and five days (30.31) with a ^Hthymidine labeling index of 9.36 ± 4 for lamellar ichthyosis and 19.7 ± 6 for nonbullous congenital ichthyosiform erythroderma (NCIF| (28). The labeling index of lamellar ichthyosis was not significantly different from that of normoproliferative skin (30,32).…”

Section: Ichthyosismentioning

confidence: 99%

“…The accumulation of excessive scale on the surface of the skin is thought to be due to excessive proliferation of the keratinocytes. Previous studies indicated two variants of this disorder (28,29). Transit times were determined as between four and five days (30.31) with a ^Hthymidine labeling index of 9.36 ± 4 for lamellar ichthyosis and 19.7 ± 6 for nonbullous congenital ichthyosiform erythroderma (NCIF| (28).…”

Section: Ichthyosismentioning

confidence: 99%

Epidermal Growth Factor Receptors in Genetically Induced Hyperproliferative Skin Disorders

Nanney

King

Dale

1990

Pediatric Dermatology

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The presence and morphologic distribution of epidermal growth factor receptor (EGF-R) were investigated in a variety of genetic disorders that affect human epidermis. These diseases included various forms of ichthyoses as well as restrictive dermopathy and the CHILD syndrome (congenital hemidysplasia-ichthyosiform erythroderma-limb defects). The distribution of EGF-R was detected by immunohistochemical techniques. Increased staining of immunoreactive EGF-R was frequently, but not always, seen in lesions with experimental or clinical evidence of hyperproliferation, suggesting an increased potential to respond to endogenous levels of either transforming growth factor-alpha or EGF. The finding that EGF-R levels are not always increased in congenital epidermal disorders indicated that the presence of this receptor pathway is not simply a marker for aberrant epidermis.

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Clinical, histologic, and cell kinetic discriminants between lamellar ichthyosis and nonbullous congenital ichthyosiform erythroderma

Cited by 31 publications

References 0 publications

Ichthyosis and Neutral Lipid Storage Disease (Dorfman‐Chanarin Syndrome)

Ichthyosis and Neutral Lipid Storage Disease (Dorfman‐Chanarin Syndrome)

Congenital non-bullous ichthyosiform erythroderma-cell kinetics before and after treatment with etretinate

Epidermal Growth Factor Receptors in Genetically Induced Hyperproliferative Skin Disorders

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