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Biomedical subjects

E H Epstein

Publications and source records attributed to E H Epstein.

At least 73 records · Page 4Linked to original sources
PubMed1983

Failure of steroid sulfatase to desulfate vitamin D3 sulfate.

Patients with recessive X-linked ichthyosis lack activity of the enzyme steroid sulfatase. One of the more striking clinical findings in these patients is the improvement in stratum corneum shedding in the summer. Since vitamin D is one steroid responsive to sunlight, we examined whether or not vitamin D3 sulfate is a substrate for steroid sulfatase. Enzyme preparations active against other sulfated steroids caused no desulfation of vitamin D3 sulfate.

Cholecalciferol↗
PubMed1983

Mycosis fungoides of the mastoid, middle ear, and CNS. Literature review of mycosis fungoides of the CNS.

Facial nerve paralysis developed in a man with tumor-stage mycosis fungoides (MF). Mastoidectomy disclosed that MF had involved the mastoid and middle ear. Meningeal lymphoma, confirmed by the finding of Sézary cells in the CSF, was subsequently established. Autopsy disclosed MF lymphoma in the leptomeninges, medulla, spinal cord, and cranial nerves. A unique feature was the formation of a communicating hydrocephalus. Case reports of 23 patients with MF of the CNS, including 21 autopsies, are reviewed. Practically all had tumor-stage or erythrodermic MF. Atypical mononuclear cells were found ante mortem in the CSF in eight patients. In contrast to other CNS lymphomas, bone marrow involvement was uncommon. Cranial, especially facial, nerve paralyses were often premonitory signs of meningeal lymphomas. Patients with MF having such symptoms should have cytologic examination of the CSF.

Adult↗
PubMed1982

Mycosis fungoides presenting as areas of hypopigmentation: a report of three cases.

In three patients with histologically documented mycosis fungoides, all or most of the lesions were hypopigmented. Unsuspected microscopic foci of mycosis fungoides were found in a purely macular hypopigmented area in one of these patients who had been treated with vitiligo 15 years earlier. Two of the patients were black, and one was Latin American. Repigmentation in all patients followed treatment with carmustine (BCNU) and mechlorethamine.

Adult↗
PubMed1982

Glucocorticoid receptors of normal human epidermis.

Epidermal cytosol of adult humans can bind dexamethasone, and this binding resembles that by glucocorticoid receptors of other tissues as judged by 4 criteria: affinity of binding, specificity of binding, molecular size of receptor-steroid complexes, and ability of these complexes to bind to nuclei. For demonstration of these receptors, epidermis can be separated by trypsinization but only after preliminary incubation of the skin with molybdate ion, which prevents inactivation of receptors when heated to 22 degree C. The presence of these receptors suggest that glucocorticoids may act directly on the epidermis. Five glucocorticoids that are used topically all displaced 3H-dexamethasone from the receptor in vitro but the affinity of this binding correlates imperfectly with the potency of the steroids when used in vivo.

Binding, Competitive↗
PubMed1981

Steroid sulfatase of human leukocytes and epidermis and the diagnosis of recessive X-linked ichthyosis.

Patients with recessive X-linked ichthyosis, one of the inherited types of excessive stratum corneum cohesion, have deficient steroid sulfatase in fibroblasts grown from their dermis. Because of the expense and long period required to grow such cells, we have assayed this enzyme in peripheral blood leukocytes and found it to be undetectable in those from patients with this type of ichthyosis, but normal in those from patients with other hereditary or acquired types of ichthyosis. In addition, steroid sulfatase activity is less in leukocytes from women who are carriers of this disease than normal women, and this assay can be used to detect such carriers. Despite previous studies demonstrating that the gene for this enzyme escapes the inactivation of other x-chromosome genes, normal women have leukocyte steroid sulfatase activity only 1.3 times that of normal men, suggesting that some gene dosage compensation occurs. Normal human epidermis, the tissue most affected clinically, also expresses steroid sulfatase activity. The epidermal enzyme is similar in its subcellular localization, its molecular size, and kinetically to that of placenta, leukocytes, and fibroblasts.

Cells, Cultured↗
PubMed1981

Glucocorticoid receptors of mouse epidermis and dermis.

The epidermis and dermis of the skin of newborn mice both contain glucocorticoid receptor molecules. These receptors resemble those of other tissues in their binding affinities, quantities, and steroid specificities. Having bound glucocorticoids, the complexes then can bind to nuclei. When chromatographed on gel filtration columns, they elute as large aggregates in low ionic strength buffers and as moieties with a Stokes' radius of 5.5 nm in high ionic strength buffers. Exposure to CaCl2 releases a still smaller binding component with a Stokes' radius of 2.8 nm. Molybdic acid stabilizes these receptors not only in cell-free preparations but also in organ cultures of whole skin. (Endocrinology 108: 703, 1981)

Animals↗
PubMed1980

Mycosis fungoides: clinical course and cellular abnormalities.

Mycosis fungoides is a lymphoma that appears to begin in skin. Although variable in its clinical course, it tends to affect middle-aged people, is present for a median of 4 yr before diagnosis, and generally results in death of the patient 4 to 5 yr after diagnosis. Cutaneous tumors and palpably enlarged lymph nodes are associated with shortened survival, and each of these frequently is accompanied by extracutaneous dissemination of the disease. The malignant cells are T lymphocytes with highly infolded nuclei, and they frequently express a helper cell function. Their chromosome complement has been found to be abnormal in karyotyping studies, and measurement of the amount of DNA by cytophotometry may permit diagnosis of the disease before the histological characteristics are detectable by light microscopy.

Adult↗
PubMed1979

Dithiothreitol separation of newborn rodent dermis and epidermis.

Incubation of newborn mouse skin in media containing 0.01 M dithiothreitol at 4 degrees C for 2 hr reduces dermal-epidermal cohesion, and the 2 layers may be separated readily. Unlike other methods of separation, this technique does not require heating, nonphysiologic pH or ionic strength, or addition of proteolytic enzymes. The split occurs beneath the basal lamina. The separated epidermal sheet retains the periodic acid Schiff-positive "basement membrane," and the basal cells can incorporate 3H-thymidine.

Animals↗
PubMed1979

Status report of 376 mycosis fungoides patients at 4 years: Mycosis Fungoides Cooperative Group.

The frequency and prognostic importance of various characteristics of patients registered by the Mycosis Fungoides Cooperative Group between November 1974 and December 1977 are reported. Variables which were considered include demographic and historical factors, symptoms, extent of disease, and other physical findings. A staging system which is based on the extent of skin involvement and the number of nodal sites clinically involved is described. Finally, a description of therapeutic results to date for patients randomized into Mycosis Fungoides Cooperative Group protocols is presented.

Adult↗
PubMed1979

Topical chemotherapy of mycosis fungoides.

Mycosis fungoides is a T-cell lymphoma which is often localized to the skin in the early stages. Untreated, the process eventually progresses through eczematous, plaque, and tumor stages to systemic involvement. Its course, however, is unpredictable. Topical chemotherapy is effective in early stages of mycosis fungoides. Possibly prognostic benefits can occur from the early use of these agents. Nitrogen mustard and BCNU, both alkylating agents, have been used topically to control the disease. A dermatitis may develop in persons treated with nitrogen mustard but systemic side-effects are rare. However, BCNU may rarely lead to marrow depression when used topically. The use of these agents in mycosis fungoides is discussed herein.

Administration, Topical↗
PubMed1978

Human skin collagen. Presence of type I and type III at all levels of the dermis.

Human skin was sliced with a dermatome, and the ratio of type I to type III collagens at various depths was assayed by comparing the quantities of peptides of each derived from cyanogen bromide digestion of the cut skin. Although immunofluorescent studies have suggested type III collagen is located predominantly beneath the epidermis and around appendages, biochemical determination demonstrates the same ratio of type I to type III collagen at all levels of the dermis even in the absence of cutaneous appendages.

Collagen↗
PubMed1978

Keratoacanthoma recurrent after surgical excision.

A 62-year-old man developed a recurrent keratoacanthoma within two and a half weeks after grossly and microscopically complete removal of the primary tumor. X-irradiation of the recurrent lesion caused partial regression, and then intralesional injections of a suspension of triamicinolone caused final resolution of it. Although dermatologists generally practice partial destruction and surgeons almost always do total excision, neither method invariably cures this benign tumor, and repeated treatment may be necessary.

Humans↗
PubMed1977

Staphylococcal scalded skin syndrome. Clinical features, pathogenesis, and recent microbiological and biochemical developments.

The essential clinical features of staphylococcal scalded skin syndrome (SSSS) and otherforms of toxic epidermal necrolysis (TEN) are contrasted. Whereas TEN is a devastating disease of multiple causes and of high fatality affecting all age groups, SSSS comprises many clinical entitles that occur primarily in early childhood and is caused by certain phage group 2 staphylococci. Because of the high cleavage plane, the barrier is only translently perturbed, and rapid recovery is the rule. Although the early stages of SSSS may resemble other widespread dermatoses clinically, the correct diagnosis is suggested, even prior to frank exfoliation, by the presence of cutaneous tenderness and a positive Nikolski sign. However, rapid bedside confirmation is now possible with exfoliative cytology and frozen sections. Recent availability of in vivo and in vitro animal models of SSSS have advanced the knowledge of the disease: the responsible epidermolytic toxin has been characterized, and the purely extracellular pathogenesis of SSSS has been established. The epidermolytic toxin is strikingly species and tissue specific, attacking only certain keratinizing epithelia of mice, hamsters, monkeys, and man. The lower incidence of SSSS in adults is primarily due to a supreior capacity to metabolize and excrete the toxin, as well as more efficient immune capabilities. The mechanisms of epidermolytic toxin action and the molecular site of action are still the focus of active investigation.

Animals↗
PubMed1977

Biosynthesis of type I and III collagens by cultured smooth muscle cells from human aorta.

The synthesis of collagen by human aortic smooth muscle cells was studied after incubating the cells with [3H]proline and [3H]glycine for 48 hr. The culture medium and cells were lyophilized and then digested with cyanogen bromide (CNBr) in 70% (wt/vol) formic acid. The resultant peptides were subjected to ion exchange chromatography on CM-cellulose and gel filtration on agarose. On the basis of the molar ratios of the alpha1(I)-CB8 and alpha1(III)-CB8 peptides of the alpha1(I) and alpha1(III) chains, approximately one quarter of the collagen synthesized by these cells was identified as type III and three quarters as type I. These data indicate that the smooth muscle can synthesize at least two types of collagen found in the arterial wall.

Aorta↗
PubMed1975

Isolation and characterization of CNBr peptides of human (alpha 1 (III) )3 collagen and tissue distribution of (alpha 1 (I) )2 alpha 2 and (alpha 1 (III) )3 collagens.

[Alpha 1(III)]3 collagen was solubilized by pepsin digestion of normal human placental membranes and was purified by differential salt precipitation and carboxymethylcellulose chromatography. This collagen was digested with CNBr, and the resultant nine peptides were isolated and characterized. The chains are cross-linked by cysteinyl residues in the COOH-terminal peptide. Isolation of peptides derived from CNBr digestion of insoluble tissues was used as an assay for the presence of [alpha 1(I)]2alpha 2 and [alpha 1(III)]3 collagens. Both types are present in human skin, intestine, liver, spleen, kidney, lung, aorta, umbilical cord, placental membranes, and myocardium. Bone and tendon contain [alpha 1(I)]2alpha 2 collagen but, unlike the other tissues, lack [alpha 1(III)]3 collagen. Both [alpha 1(I)]2alpha 2 and[alpha 1(III)]3 collagens are present in scars of human skin, myocardium, tendon, and liver and of rabbit skin. The degree of hydroxylation of proline was 4 to 5% lower in the same peptides in skin, bone, and tendon than in the other tissues. The degree of hydroxylation of lysine in the same peptides derived from different tissues varied more widely.

Adolescent↗