PubMed HealthSearch

Biomedical subjects

S A Büchner

Publications and source records attributed to S A Büchner.

12 recordsLinked to original sources

Focal dermal hypoplasia syndrome in a male patient. Report of a case and histologic and immunohistochemical studies.

BACKGROUND: Focal dermal hypoplasia (FDH) is a rare ectomesodermal dysplasia syndrome characterized by cutaneous, skeletal, dental, ocular, and soft-tissue defects. An X-linked dominance with death of male subjects has been assumed as the mode of inheritance. Only a few cases of FDH have been reported in male subjects. A 68-year-old man had typical skin lesions of FDH. Clinical, histologic, and immunohistochemical features are presented. OBSERVATIONS: The cutaneous abnormalities consisted of atrophic hyperpigmented and hypopigmented macules and erythematous, slightly raised lesions showing a highly characteristic linear distribution. Other abnormalities, including syndactyly, apocrine hidrocystoma of eyelids, and bilateral cholesteatoma were observed. Only one case of FDH in association with an apocrine hidrocystoma has been reported previously. Consistent findings microscopically in the erythematous lesions were areas of scar formation with subepidermal bulla overlying the neodermis. A strongly positive immunohistochemical reaction for vimentin, fibronectin, and collagen type III was observed in the scar tissue. No collagen type IV was detected in the basement membrane zone of the epidermis covering the lesion. CONCLUSION: It has been proposed that fibroblastic abnormalities may lead to an alteration of collagen synthesis in FDH, although confirmation of this hypothesis was unavailable. Our findings suggest that production of collagen type IV may be delayed in FDH resulting from a fibroblastic defect.

Aged

Follicular mucinosis associated with mycosis fungoides.

We report a patient who developed erythematous indurated plaques with alopecia on the face and multiple well-demarcated infiltrated scaling lesions on the trunk and extremities. Skin biopsy showed a mucinous degeneration of the follicular outer root sheaths and sebaceous glands consistent with the diagnosis of follicular mucinosis. A histologic examination of a specimen from the left forearm showed microscopic changes of mycosis fungoides. The present case confirms the need of multiple skin biopsies in the evaluation of patients with follicular mucinosis.

Aged

Blood flow response to cryosurgery on basal cell carcinomas.

Cryosurgery with liquid nitrogen is commonly used to treat benign and malignant skin tumours. One of the crucial factors which influences the freezing and thawing rate of the target cells is the response of the microcirculation to cold stimulus. In our experiment, laser Doppler flowmetry was used to monitor blood flow during cryosurgical treatment of basal cell carcinomas. Outside the frozen tissue hemisphere, blood flow, as measured by laser Doppler flowmetry, increased almost instantaneously and remained on a high level during thawing. Baseline blood flow at the contralateral anatomical site remained stable. Pharmacological or physical modification of the cutaneous microcirculation before therapy may influence freezing and thawing times, and therefore the outcome with regard to healing, cosmetic appearance and the recurrence rate of basal cell carcinomas.

Aged

[Skin manifestations in HIV infection].

A review of the spectrum of dermatologic findings related to human immunodeficiency virus infection is presented. Oral candidiasis, seborrheic dermatitis, oral hairy leukoplakia, herpes simplex infections and molluscum contagiosum were among the most frequently encountered dermatologic disorders among the HIV-seropositive individuals. The recognition of cutaneous manifestations is of particular importance for the early diagnosis and prognosis of HIV-infection.

Bacterial Infections

[Acne and its drug treatment].

The most common disease of the pilosebaceous follicle is acne vulgaris. The primary detectable pathologic defect in acne is abnormal keratinization of the follicular epithelium, resulting in a retention hyperkeratosis. The primary lesion produced by this process, the comedo, ist the precursor of most other acne lesions. These include inflammatory papules, pustules and nodules. Treatment principles are directed against known pathogenic factors. The major topical modalities that are currently being used include tretinoin, benzoyl peroxide and topical antibiotics. Benzoyl peroxide is a powerful antibacterial agent. Topical erythromycin and clindamycin appear to have equivalent efficacy. A major treatment advance has been heralded by the use of oral 13-cis retinoic acid (isotretinoin) in the management of patients with severe nodulocystic acne, acne conglobata and acne fulminans. Isotretinoin is teratogenic and should not be given to women of childbearing age.

Acne Vulgaris

[Morphaea profunda].

A 47-year-old woman with a solitary morphoea profunda on the flexor aspect of the left thigh is described. Clinically the lesion is characterized by a circumscribed, deeply indurated sclerotic plaque. The main histopathological features were sclerotic collagen changes and a striking accumulation of inflammatory cells in the deep dermis as well as in the subcutaneous tissue. Plasma cells and T- and B-lymphocytes were the predominant cellular components of the infiltrate. The patient had an elevated serum titre of antibodies to Borrelia burgdorferi. The patient was treated with penicillin, which brought about a remarkable improvement. These findings provide further support for the concept that in some cases morphoea profunda may be a manifestation of Borrelia infection.

Antibodies, Bacterial

[Morphea--a tick transmitted borreliosis of the skin? A contribution to the pathogenesis of circumscript scleroderma].

We report on a 34-year-old patient suffering from erythema chronicum migrans, who developed clinically and histologically typical morphea, which was confined to the area previously involved by the erythema migrans. The patient's serum antibody level against borrelia burgdorferi spirochetes was significantly elevated. By means of the silver impregnation technique, we were able to identify spirochetal organisms both in the lower dermis and within the septa of the subcutaneous fatty tissue. In frozen sections, spirochetes were demonstrated by the immunoperoxidase method using specific anti-spirochetal antibodies. For a better assessment of the composition of the inflammatory infiltrate in morphea, we applied a panel of monoclonal antibodies to a sensitive two-stage immunoperoxidase technique. Helper-inducer T-cells and a large number of suppressor-cytotoxic T-cells were observed both in the perivascular infiltrate and between collagen fibers in close proximity to the HLA-DR reactive fibroblasts. A large number of mast cells were seen in the dermal infiltrate. The detection of spirochetal organisms in histological sections as well as the demonstration of closely associated helper-inducer T-cells, macrophages, and activated fibroblasts in the dermis strongly suggest that a cell-mediated immune response against borrelia may be the dominant pathogenetic event in this variant of scleroderma. Activated T-lymphocytes and various factors secreted either by activated lymphocytes or mast cells may cause proliferation of fibroblasts, which can lead to increased collagen synthesis and dermal fibrosis.

Adult

[Parapsoriasis en plaques. Characterization of the cellular infiltrate using monoclonal antibodies].

We report on a 65-year old woman suffering from parapsoriasis with large plaques. The cellular infiltrate was analysed by means of monoclonal antibodies and an immunoperoxidase technique. Our findings proved that the majority of the cellular infiltrate in the upper dermis is composed of helper-inducer T-cells (Leu 4+, Leu 3a+). A notable number of Leu-2a-reactive suppressor-cytotoxic T-cells associated with hydropic degeneration of the basal cell layer were found within the dermal infiltrate and the dermoepidermal interface. Large numbers of Leu-6-reactive dendritic Langerhans' cells were noted in the lower layers of the epidermis, in particular in areas where the epidermis showed focal exocytosis of lymphoid cells. Langerhans' cells, in contrast, were present in the upper portions of epidermis lacking exocytic T-cells. HLA-DR antigens were expressed on Langerhans' cells as well as on nearly all T-lymphocytes. Positive intercellular staining for HLA-DR antigens were only found in localized areas of the epidermis. Small numbers of both Leu-11B-reactive natural killer cells and cells expressing interleukin-2 receptors were seen within the basal cell layer and at the dermo-epidermal junction. Our findings suggest that a cell-mediated immune response which is directed against antigens expressed by keratinocytes may play an important role in the pathogenesis of parapsoriasis en plaques.

Aged

Hyperkeratosis lenticularis perstans (Flegel's disease). In situ characterization of T cell subsets and Langerhans' cells.

We report a patient with hyperkeratosis lenticularis perstans (HLP) manifesting as multiple reddish-brown hyperkeratotic papules on the lower extremities. Typical histologic features of HLP include hyperkeratosis, thinning or absence of the granular layer and a band-like infiltrate in the upper dermis underlying an atrophic epidermis. In order to determine the cellular composition of the infiltrate, skin biopsy specimens were studied immunohistochemically using a series of commercially available monoclonal antibodies. The dermal infiltrate consists predominantly of helper/inducer T cells (Leu-4+, Leu-3a+). Suppressor/cytotoxic T cells (Leu-2a+) were fewer at the periphery of the infiltrate. The majority of T cells were activated as they expressed HLA-DR-antigen. Large numbers of Leu-6+ Langerhans' cells were observed at the dermo-epidermal interface. Few natural killer cells (Leu-11b+) were noted within the dermal infiltrate. These findings support the hypothesis than an active cellular immune reaction involving the epidermis is of pathogenic importance for HLP.

Antibodies, Monoclonal

[Enzyme cytochemical and immunocytological differentiation of infiltrative cells in the skin in mycosis fungoides].

In 4 patients with mycosis fungoides in the praemycotic, infiltrative and tumour stage of the disease, a differentiation and functional characterization of the infiltrate cells, obtained from cell suspensions and cutaneous smears, was carried out by means of enzyme cytochemical and immunological methods. The lymphocyte-associated acid esterase is considered to be a marker for mature T-cell populations. Apart from monocytes and histiocytes, acid esterase-positive small lymphocytoid cells with T-cell properties are found in the praenycotic stage. In the tumour stage, large lymphocytoid cells become increasingly prevalent, they show no acid esterase activity, but an intracytoplasmatic-localized reaction in the acid phosphatase activity. On the basis of the cytochemical pattern, it is assumed that these cells represent proliferating lymphoblasts.

Acid Phosphatase

[Enzyme cytochemical studies of malignant cutaneous lymphomas using simple smear preparations].

Cyto-morphological and enzyme-cytochemical investigations were carried out in five patients with lymphoreticular proliferations in the skin, using a simple smear technique. As purely morphological criteria allow only a limited differentiation of the infiltrate cells in the smears, enzyme-cytochemical methods were applied for further identification (detection of hydrolytic enzymes). With this new technique, the single cells are not damaged and keep their cell structure, so that the exact localisation of the enzyme activity is possible. Our investigations demonstrate the change of the cellular infiltration in the cutaneous smear during the clinical course of mycosis fungoides. In the premycotic stage, enzyme cytochemically positive monocytes and enzyme cytochemically negative lymphocytes with small nuclei are prevalent. In the infiltrative and tumor stages the cellular picture is dominated by polymorphonuclear lymphocytoid cells with medium sized nuclei, these cells contain ample amounts of esterases and acid phosphatase. In contrast to these findings, other lymphomas investigated by us are characterized by the lack of monocytes and an abundance of lymphocytes, which according to the stage of differentiation present a different pattern of the alpha-naphthylacetate-esterase and the acid phosphatase.

Adult

The significance of the hairpin counter-current principle in the pathogenesis of toxic kidney lesions. An investigation of the influence of antidiuretic hormone and papaverine on hydroxyquinoline nephropathy of the rat.

The influence of antidiuretic hormone (ADH) and papaverine on hydroxyquinoline-induced nephropathy in rats was tested, using histotopochemistry, enzyme activity measurement and morphometric investigation. Hydroxyquinoline causes a marked increase in renal weight, the development of wedge-shaped foci with severely dilated tubule segments, and a simultaneous reduction in dehydrogenases, alkaline phosphatase, and alpha-naphthyl esterase. Both ADH and papaverine produced a significant inhibition of renal damage. The subjective findings were quantitatively confirmed by measurement of enzyme activity, using the microscope photometer, and by morphometric studies with the Leitz-Classimat (determination on the basis of the alkaline phosphatase reaction) of the surface percentage of brush border in the proximal tubules. A disturbance of the hairpin counter-current system is to be considered as the cause of the renal lesion. This disturbance is caused by hydroxyquinoline-induced impairment of Na+/K+ transport, especially in the thick ascending limb of Henle's loop. Our results show that the hydroxyquinoline nephropathy can be favourably influenced both by stimulation of water re-absorption and possibly also transepithelial Na+ transport (ADH), and by increasing the blood flow of the arteriolae rectae with a resultant lowering of the intratubular urine concentration (papaverine). The dependency of hydroxyquinoline nephropathy on the phylogenetically determined concentration capacity of the kidney, and the effective influencing of the condition by ADH and papaverine indicate the importance of the degree of efficiency of the medullary countercurrent system in the pathogenesis of this renal lesion.

Alkaline Phosphatase