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Biomedical subjects

K Wolff

Publications and source records attributed to K Wolff.

At least 271 records · Page 15Linked to original sources

Histiocytosis X cells in eosinophilic granuloma express Ia and T6 antigens.

Morphologic similarities between histiocytosis X (HX) cells and epidermal Langerhans cells (LC) have led to the hypothesis that HX represents a proliferative disorder of LC. In order to prove the validity of this assumption, we tested single cell suspensions isolated from an eosinophilic granuloma type HX lesion for the presence of various antigenic determinants defined by monoclonal antibodies using an immunoelectron microscopic technique. An anti-Ia reagent reacted with essentially all histiocytic cells and a small portion of lymphocytes whereas plasma cells and eosinophils were negative. T6 antigen, in contrast, was disclosed exclusively on HX cells either with or without Birbeck granules. Pan-T cell-reagent OKT3 reacted only with small lymphocytes. The finding that HX cells from eosinophilic granuloma lesions are the only cells that have the identical surface marker equipment as epidermal LC (Ia antigens, T6 antigen, Fc-IgG, and C3 receptors) strongly supports the concept that these cells are derived from the LC lineage.

Adult↗

Coated Langerhans cell granules in histiocytosis X cells.

Histiocytosis X cells (HXC) and epidermal Langerhans cells (LC) are thought to be closely related because they share morphologic features and immunologic markers. One of these common markers, the LC granule, is an acknowledged morphologic criterion for the identification of these cell types, but its function and, thus, significance are as yet unknown. In this study, we report the presence of a fuzzy coat radiating from the cytoplasmic face of the LC granule membrane in HXC. This bristly coat is indistinguishable from that of coated vesicles and pits and, thus, represents a strong morphologic clue to the function of LC granules because coated structures have been shown to be involved in the selective transport of molecules in eukaryotic cells.

Adult↗

The Langerhans cell.

Langerhans cells are the bone-marrow-derived immune cells of the epidermis; they express Ia antigens and receptors for the Fc portion of IgG and complement components and are required for epidermal-cell-induced antigen-specific, syngeneic and allogeneic T-cell activitation and the generation of epidermal-cell-induced cytotoxic T cells. Their presence within the epidermis and functional integrity determine whether topical application of haptens leads to specific sensitization or unresponsiveness, and in skin grafts of only I region disparate donors, they represent the cells responsible for the critical allosensitizing signal. UV radiation abrogates most of Langerhans cell functions in vitro; under certain conditions in vivo, it prevents contact sensitization favoring the development of specific unresponsiveness. UV radiation abrogates antigen-presenting capacities of epidermal cells by interfering both with the processing of antigen by Langerhans cells and the production of the epidermal-cell-derived thymocyte activating factor required for optimal T-cell responses.

Animals↗

The syndrome of milker's nodules in burn injury: evidence for indirect viral transmission.

Four patients with first- to second-degree burns developed multiple unusual nodular lesions confined to the burned areas 2 to 3 weeks after the accident. Electron microscopy disclosed viral particles within epidermal cells. These were identified as subgroup II poxvirus. Viral culture established the diagnosis of paravaccinia (milker's nodule) infection. Since none of the patients had had direct contact with infected animals, but had been in contact with contaminated objects, an indirect viral transmission, previously not reported for milker's nodules, appears the most likely mode of infection.

Adult↗

Systemic lupus erythematosus in hereditary deficiency of the fourth component of complement.

Three patients from two families with complete hereditary deficiency of the fourth component of complement (C4) and systemic lupus erythematosus are described. The syndrome presented by these patients is characterized by early onset in life; exquisite sensitivity to sunlight and to cold exposure, the latter resulting Raynaud's phenomenon; and skin lesions involving not only exposed areas of the body but also palms and soles and presenting as butterfly rashes, maculopapular eruptions, and lesions similar to those of chronic discoid lupus erythematosus, with marked scaling, atrophy, and scarring. Lupus erythematosus (LE) cell tests were negative and antinuclear antibody (ANA) titers low or negative. The male patient of our series died at the age of 31/2 years from septicemia, whereas the two girls, aged 18 and 11 years, respectively, were alive at the time of writing. The C4-deficient gene is associated with HLA-Aw32, Bw38, and Bf S in one family and with HLA-A30, B18, DR7, and Bf S1 in the other family; the latter is the second family in which this HLA haplotype has been found to be associated with hereditary C4 deficiency.

Adolescent↗

In vitro complement binding on cytoplasmic structures in normal human skin: immunoelectronmicroscopic studies.

We have previously provided evidence that suggests that exposure of cryostat skin sections to normal human serum (NHS) results in the antibody-independent Clq binding to cytoplasmic structures of various cell types, leading to classical complement pathway activation as evidenced by cytoplasmic C3 deposition. In the present study, we have employed immunoelectronmicroscopic methods to clarify the exact nature of cytoplasmic C3 binding structures. Incubation of cryostat skin sections with NHS followed by peroxidase-labeled rabbit anti-human C3 serum (HRP-R/Hu C3) revealed that intracytoplasmic binding of C3 occurred in suprabasal keratinocytes, melanocytes, fibroblasts, smooth muscle cells, endothelial cells, pericytes, Schwann cells, and nerve axons, but not in basal keratinocytes, Langerhans cells, and other cellular constituents of the skin. C3 binding, as revealed by the deposition of HRP reaction product, was exclusively confined to intermediate-sized filaments (ISF), which can therefore be considered to represent the subcellular site for classical complement pathway activation. Under experimental conditions that do not allow classical complement pathway activation, ISF were not decorated. Our observation that ISF of ontogenetically different cell types share the capacity of complement fixation is in accordance with the recent finding that different ISF types, despite their biochemical and antigenic heterogeneity, have common alpha-helical domains and may provide a clue to the mechanism and site of interaction between complement components and ISF.

Axons↗

Serum levels of 8-methoxypsoralen in two different drug preparations: correlation with photosensitivity and UV-A dose requirements for photochemotherapy.

In a quantitative study we have compared the serum levels, the time course and the photosensitizing capacity of a conventional crystalline 8-methoxypsoralen brand and an investigational liquid formula. Evidence is presented showing that the liquid preparation is superior to the crystalline form: it peaks earlier after ingestion, it produces higher and more constant degree of photosensitization, it is eliminated more rapidly from the blood, and it requires a lower UV-A dose for eliciting photosensitivity reactions aiming at a reduction of the total cumulative UV-A dose required for clearing psoriasis.

Adult↗

Selective accumulation of lipid within melanocytes during photochemotherapy (PUVA) of psoriasis.

In patients undergoing photochemotherapy (PUVA) a selective accumulation of lipid droplets was observed within the cytoplasm of melanocytes. Keratinocytes did not develop lipid droplets. Within 2 months after clearing of psoriasis the droplets gradually vanished and did not reappear even during maintenance PUVA therapy. Increased intracellular lipid deposits are usually taken to herald cell degeneration. Since the maximum lipid accumulation coincided with the end of the clearing phase when melanin production was at its height, the intramelanocytic lipid may have been a morphological sign of over-stimulation of melanocytes, which could eventually result in melanocyte destruction. Since excess melanin may be cytotoxic for melanocytes this may explain the irreversible hypopigmentation which develops in some patients treated with PUVA.

Adolescent↗

Generalized atrophic benign epidermolysis bullosa.

Eight cases of a new variant of hereditary epidermolysis bullosa (EB), generalized atrophic benign EB, are reported. This is a junctional form of EB that, in contrast to EB letalis of Herlitz, has a good prognosis. It is inherited as an autosomal recessive trait, and the clinical picture is monotonously similar in all patients observed so far, with generalized blister formation, atrophic alopecia, and dystrophic nail changes. Blisters on the skin and mucous membranes heal without scarring or dystrophy but often result in notable atrophy. There is a definite tendency for amelioration of symptoms as the patients age, but therapy has, so far, been ineffective.

Adolescent↗

[Stevens-Johnson syndrome and toxic epidermal necrolysis following intake of sulfonamides].

Sulfonamides, particularly in combination with trimethoprim, are among the most commonly prescribed antimicrobial chemotherapeutic agents since they are generally well tolerated and have a broad antibacterial spectrum. However, they are the most common cause of adverse cutaneous drug reactions, the most serious of which are the major variant of erythema multiforme, i.e. Stevens-Johnson-syndrome, and toxic epidermal necrolysis. In eight of 11 patients with Stevens-Johnson syndrome or toxic epidermal necrolysis seen in our department over a period of 4 years, sulfonamides were the most probable causative agent. The present report describes these eight patients and discusses the potential risks of sulfonamide therapy.

Aged↗