Congenital ichthyosiform erythroderma: particulate staining pattern of TGK.

A case of late onset non-bullous congenital ichthyosiform erythroderma (CIE) was studied. This patient was not born as a collodion baby and did not have skin abnormalities until 9-10 years of age. She gradually developed erythroderma and fine scales, callosities of her feet, and a mild ectropion. Since recent work has revealed that in the majority of CIE patients, transglutaminase (TGK) is distributed in the cytoplasm of granular cells and horny cells (11), TGK was studied in our case. It was found that TGK was distributed along the cell periphery of horny cells and also in the cytoplasm of granular cells. In the control skins, TGK was stained along the cell periphery of horny cells and granular cells. The marginal band formation was normal. Involucrine and loricrin, the building materials of the marginal band whose-cross-linking is mediated by TGK, were normally stained in the upper epidermis. Cytoplasmic TGK of granular cells and normal development of the marginal band may serve as a helpful diagnostic marker of CIE, particularly because the often confusing collodion baby of lamellar ichthyosis may lack TGK staining and the marginal band altogether.

Sudoriferous acrosyringeal acantholytic disease. A subset of Grover's disease.

Three selected cases of transient acantholytic dermatosis were studied because of their definitive correlation with sweating due to fever and/ or bed-ridden situations. Biopsy specimens were serially sectioned and acantholysis was found in the acrosyringium or traced to connect to the acrosyringium in all biopsy specimens. Carcinoembryonic antigen (CEA) and eccrine gland-specific monoclonal antibody, IKH-4, were positive in acantholytic cells. Electron microscopy revealed electron dense material filling the lumen of intraepidermal eccrine ducts. This material leaked into lateral intercellular spaces of the luminal cells, passing tight junctions. Marked edema and numerous lysosomes were reminiscent of those found when eccrine acrosyringium is formed in the embryo; this suggested that an occluded and damaged eccrine intraepidermal duct was being rebuilt via lysosomal digestion.

Linear focal elastosis in a young black man: a new presentation.

We report the sixth case of linear focal elastosis. Our patient is a thin 29-year-old black man who was first seen with rows of papules with a yellow hue distributed symmetrically on the lumbar region of the back. The patient had been aware of these lesions since early childhood, and similar lesions reportedly had occurred in his late father. Light microscopic examination demonstrated dermal thickening but no change in the epidermis. The elastic tissue stain revealed fragmented elastic fibers throughout the dermis. This case meets previously defined diagnostic criteria for linear focal elastosis, which has been reported as isolated cases in elderly white or Japanese men. Our patient is the first young black man with a possible familial association.

Levels of [3H]glucosamine incorporation into hyaluronic acid by fibroblasts is modulated by culture conditions.

Tissue culture conditions can modulate apparent levels of incorporation of the radiolabeled precursor [3H]glucosamine into hyaluronic acid in cells. A careful study was made on the effects of culture conditions on human skin fibroblasts. A newly described technique to measure hyaluronic acid was utilized based on incorporation of [3H]glucosamine into cetylpyridinium chloride-precipitable hyaluronidase-digestible material. The precipitate was collected on glass fiber filters using a manifold suction apparatus. A six-fold greater level of incorporation occurred in rapidly growing preconfluent than in confluent fibroblasts. Ascorbic acid stimulated incorporation with a maximum at 25 micrograms/ml. The same ascorbic acid optimum was observed for collagen prolylhydroxylation. When beta-hydroxybutyrate was used as an energy source instead of D-glucose, a 3.5-fold increase in levels was observed. All tissue-culture media examined supported comparable levels of incorporation, except for Roswell Park Memorial Institute Media-1640, in which cells had only half the level. Fetal calf serum supported high levels of incorporation in a dose-dependent manner, while newborn calf and calf sera supported much lower levels of incorporation. Under serum-free conditions, lactalbumin hydrolysate was best able to support incorporation of hyaluronic acid. In the search for mechanisms that modulate hyaluronic acid, it is critical to consider the tissue culture conditions under which incorporation of radiolabeled precursors are being examined.

Hyaluronic acid-stimulating activity in sera from the bovine fetus and from breast cancer patients.

The sine qua non of malignancy is the ability of tumor cells to migrate and invade surrounding tissue. There are many substances that have been described that enhance cell motility and hyaluronic acid is prominent among these. Hyaluronic acid is a high molecular weight alternating disaccharide polymer found in abundance in extracellular matrices whenever rapid cell proliferation or tissue regeneration and repair occur. It creates a permissive environment for cell motility during embryogenesis, and high levels of hyaluronic acid also correlate with increased tumor cell invasion and aggressiveness. Little is known about the regulation of hyaluronic acid production, either in normal tissue or in malignancy. In this study, we characterize a hyaluronic acid-stimulating activity in fetal calf serum and describe a similar activity in the sera of breast cancer patients. The stimulating activity was measured by placing aliquots of test substance on fibrosarcoma cells. These indicator cells, which synthesize copious quantities of hyaluronic acid, respond to stimulation in a time- and dose-dependent fashion. The fetal calf serum hyaluronic acid-stimulating activity is maximum early in gestation and then falls rapidly to essentially no activity at term. This activity was partially purified from 120-day fetal calf serum by concanavalin A-Sepharose affinity and ion exchange chromatography and is accounted for by a glycoprotein with a molecular weight of 150,000 on gel filtration under native conditions. The sera of breast cancer patients with measurable burden of disease also contained hyaluronic acid-stimulating activity, which was not present in normal serum donors or in breast cancer patients without evidence of disease. The production of this stimulating activity may contribute to the development of the malignant phenotype by inducing hyaluronic acid-rich microenvironments that are permissive to tumor cell invasion and metastases.