Search PubMed⌕ Search

Biomedical subjects

B Zelger

Publications and source records attributed to B Zelger.

At least 73 records · Page 4Linked to original sources
PubMed1996

An immunocytochemical assessment of 19 cases of cutaneous angiosarcoma.

Four endothelial cell markers, two selective cytokeratin markers and a monoclonal smooth muscle antibody (SMA) were employed in the assessment of 19 cases of cutaneous angiosarcoma classified according to their degree of tumour differentiation. No labelling was seen for SMA or with cytokeratin markers MNF116 and CBL170. Expression of factor VIII-related antigen was seen in two tumours and positivity for CD34 (QBend 10 antibody) was found in four tumours. By contrast the pan-endothelial cell marker Ulex europeaus agglutinin 1 (UEA-1) and the CD31 marker JC70A labelled all cases of cutaneous angiosarcoma with the exception of one poorly differentiated tumour. These data confirm the endothelial cell origin of angiosarcoma, they demonstrate that CD31 and UEA1 are reliable markers in routinely processed tissue, and they suggest a lymphatic derivation for the tumour. This finding is in marked contrast to Kaposi's sarcoma where CD34 is the most reliable marker.

Antibodies, Monoclonal↗
PubMed1996

Differentiation of primary and secondary cutaneous B-cell lymphoma by Southern blot analysis.

Malignant B-cell lymphomas represent a heterogenous group of lymphoreticular disorders that involve the skin in about 20% of reported cases. Skin involvement may be primary or secondary (ie, the result of hematogenous spread). Primary cutaneous B-cell lymphomas (PCBCLs) are thought to take a comparatively favorable course, respond readily to nonaggressive treatment, and lack evidence of extracutaneous spread. Nine primary B-cell lymphomas (7 centrocytic or centroblastic follicular, 1 immunoblastic, 1 centroblastic), three secondary (follicular) cutaneous B-cell lymphomas (SCBCLs) and two pseudolymphomas were studied. Staging revealed that bone marrow was involved only in SCBCLs. Centrocytes were detected in blood smear preparations of all SCBCLs. All lymphomas were treated with local irradiation. Patients with primary centroblastic and immunoblastic cutaneous lymphomas and those with secondary lymphomas received additional chemotherapy. Pseudolymphomas were treated by simple excision. Patients were monitored on average for 55 months. During this period, no patients with PCBCLs exhibited cutaneous relapses or hematogenous spread. In contrast, all patients with SCBCLs experienced cutaneous relapses. Peripheral blood, bone marrow, and skin samples from all patients were subjected to Southern blot analysis using a JH probe. Clonal rearrangement was found in all skin samples investigated except specimens from pseudolymphomas. Peripheral blood and bone marrow samples were positive in SCBCLs (the rearrangement pattern was different from that of the skin samples for two of the three patients), whereas it was negative in all PCBCLs and pseudolymphomas. In conclusion, Southern blot analysis of peripheral blood may be useful in differential diagnosis of PCBCLs and SCBCLs and a prognostic marker. Furthermore, these data confirm the comparatively favorable clinical course of PCBCLs and suggest that in these cases, local irradiation can be considered adequate treatment, whereas SCBCLs require additional systemic therapy.

Adult↗
PubMed1996

[Indications and concepts for plastic surgery therapy in chronic varicose ulcer].

In the treatment of extensive leg ulcers, surgical therapy is superior to conservative methods in many respects. Radical resection of sclerotic tissue around the ulcer and its causing veins is essential, followed by defect cover with mesh-skin grafts. Surgery leads to prompt healing with a shortened hospitalization and therefore decreases costs; it leads to a prompt relief of pain and a low rate of recurrence. Especially in the aged patient the indication for operation must be well-considered and discussed interdisciplinarily. Even though operative technique seems to be simple, it should be reserved for the plastic surgeon experienced in the treatment of these lesions.

Adult↗
PubMed1996

Indeterminate cell histiocytosis--a clinicopathological entity with features of both X- and non-X histiocytosis.

An otherwise healthy 50-year-old woman presented with a 6-month history of having developed more than 100 generalized, non-confluent, reddish-brown, partially yellow-coloured papules. A non-epidemotropic, monomorphous infiltrate of vacuolated mononuclear, and occasionally multinuclear, histiocytes, positive for factor XIIIa and macrophage markers HAM56 and KiM1p, was consistent with the clinical impression of generalized eruptive histiocytomas. However, the additional reactivity for S100 protein, in the absence of features of histiocytosis X, suggested a diagnosis of indeterminate cell histiocytosis (ICH). Further immunohistochemical studies, performed on snap-frozen material, characterized the lesions as being diffusely positive with LN3 (HLA-DR), Leu4 (CD3) and Leu3 (CD4), the infiltrate in the upper dermis as reactive for OKT6 (CD1) and IOT6c (CD1c), and the infiltrate in the lower dermis as reactive for a variety of macrophage markers. Ultrastructural studies showed various non-specific features of histocytic disorders, but no Birbeck granules. Our findings confirm those of previous reports suggesting that ICH is a distinct histiocytic entity, characterized by immunophenotypic features of both X- and non-X histiocytoses. Generalized eruptive histiocytoma seems to be an early indeterminate stage of various non-X histiocytic syndromes including ICH, multicentric reticulohistiocytosis, xanthogranuloma and xanthoma disseminatum. The distribution pattern of the various X/non-X histiocytic markers suggests dermal arrest of antigen-presenting cells during their physiological trafficking from the skin to the lymph nodes.

Female↗
PubMed1996

The non-Langerhans' cell histiocytoses in childhood.

The cutaneous histiocytoses are best divided in the Langerhans' cell histiocytoses and non-Langerhans' cell histiocytoses. In the former group, the cells react with S100 and CD1a antibodies, while in the latter group they express a variety of macrophage markers. Xanthogranuloma is a frequent childhood tumor and the only common non-Langerhans' cell histiocytosis. Xanthogranulomas contain a mixture of several different types of histiocytes that also appear in more pure forms as both solitary tumors and disseminated processes. The varying histiocyte morphology provides a unifying concept for non-Langerhans' cell histiocytoses.

Adult↗
PubMed1995

Sneddon's syndrome--an inflammatory disorder of small arteries followed by smooth muscle proliferation. Immunohistochemical and ultrastructural evidence.

Sneddon's syndrome is a rare, but potentially severe, arterioocclusive disorder characterized by generalized livedo racemosa of the skin and various central nervous symptoms due to occlusion of medium-sized arteries of unknown cause. We have recently shown that, in skin, small to medium-sized arteries of the dermis-subcutis boundary are affected in a stage-specific sequence. An initial phase (stage I), characterized by the attachment of lymphohistiocytic cells and detachment of endothelial cells (endothelitis), is followed by an early phase (stage II), which displays partial or complete occlusion of the lumen by a plug of lymphohistiocytic cells and fibrin. In an intermediate phase (stage III), the occluding plug is replaced by proliferating subendothelial cells accompanied by the occurrence of dilated capillaries in the adventitia of the occluded vessel. The late phase (stage IV) shows fibrosis and shrinkage of the affected vessels. We investigated sections of paraffin-embedded specimens of 18 patients by immunohistochemistry using a panel of antibodies to detect endothelial cells, macrophages, T cells, smooth muscle-specific actin, and intermediate filaments (vimentin, desmin). We found that the cells involved in subendothelial proliferation were vimentin and actin positive (smooth-muscle-specific), but desmin negative and thus displayed the phenotype characteristic of smooth muscle cells, which was confirmed by ultrastructural studies. CD3+, UCHL-1+, and HLA-DR+ cells constituted a significant proportion of the inflammatory infiltrate in the early stages. The endothelial cells in the dilated capillaries of the adventitia were strongly HLA-DR positive. In later stages, endothelial cells and leukocytes were scarce. The data confirm the hypothesis that Sneddon's syndrome starts as an inflammatory and possibly immunologically mediated disorder, leading to a migration and proliferation of smooth cells of small arteries, resulting in a partial or complete narrowing of the vessel lumen.

Arterial Occlusive Diseases↗
PubMed1995

Solitary giant xanthogranuloma and benign cephalic histiocytosis--variants of juvenile xanthogranuloma.

Sequential biopsies taken from a patient with a solitary giant xanthogranuloma, an exaggerated macronodular (> 5 cm in diameter) variant of juvenile xanthogranuloma, and from a patient with benign cephalic histiocytosis, revealed a characteristic time sequence of histopathological findings. Early stages of the diseases showed a monomorphous infiltrate of mononuclear vacuolated histiocytes positive for KiM1p, HAM56 and factor XIIIa and were characterized by clusters of comma-shaped bodies. This was followed by a polymorphous mixture of various mononuclear and multinucleate histiocytes additionally labelling with KP1 (CD68) and, in occasional cells, for the adherence of peanut agglutinin. A variety of ultrastructural changes were found, including dense and regularly laminated bodies or lipid droplets. Our findings indicate that both entities are variants of a xanthogranulomatous reaction.

Antibodies↗
PubMed1995

Immunostaining for CD31 and CD34 in Kaposi sarcoma.

AIMS: To evaluate antibodies directed against CD31 (JC70/A) and CD34 (QBEND/10 and anti-HPCA-1) more extensively in Kaposi sarcoma; to assess their value in routine diagnosis; and to compare them with the traditional endothelial cell markers Ulex europaeus agglutinin 1 (UEA-1) and factor VIII related antigen. METHODS: Twenty four cases of Kaposi sarcoma were studied retrospectively. All specimens had been fixed in formalin and embedded in paraffin wax. The antibodies were applied using the Streptavidin biotin technique in all cases except for UEA-1, for which an indirect two stage method was used involving peroxidase conjugated anti-ulex as the secondary antibody. RESULTS: Tumours were classified into those showing angiomatoid or lymphangiomatoid elements and spindle cell lesions. Universal labelling of all lesions and virtually all elements within lesions was seen with the anti-CD34 antibodies QBEND/10 and HPCA-1. Labelling of spindle cells was less consistent with JC70/A but both markers were superior to the traditional endothelial cell markers UEA-1 and factor VIII related antigen. CONCLUSIONS: These data confirm that Kaposi sarcoma is a tumour of endothelial cell origin. They shed further light on the histogenesis of this complex tumour and demonstrate that immunostaining for CD34 and CD31 can be used as an aid to diagnosis in routinely processed tissue.

Antigens, CD34↗
PubMed1994

Reticulohistiocytoma and multicentric reticulohistiocytosis. Histopathologic and immunophenotypic distinct entities.

The clinicopathological and immunohistochemical features of four patients with systemic multicentric reticulohistiocytosis (MR) were compared with five cases of solitary and one case of multiple reticulohistiocytoma (RH), which were confined to the skin only. The MR cases mostly affected the limbs of older women, while RH affected young male adults without preference to site. Characteristically, both entities consisted of oncocytic mononuclear histiocytes (with granular eosinophilic cytoplasm similar to oncocytic thyroid cells) and multinucleated histiocytes with a ground-glass appearance, which appeared to be much larger (> 200 microns) and bizarre in cases of RH compared with cases of MR (50-100 microns). In RH a variable number of vacuolated, spindle-shaped, and xanthomatized mononuclear histiocytes were also present. Immunohistochemical profiles showed positivity of mononuclear histiocytes with HHF35, factor XIIIa, and LN3 (HLA-DR), with a variable number of multinucleated histiocytes in RH showing binding with peanut agglutinin. In mono- and multinucleated histiocytes in both entities macrophage markers KP1 (CD68), KiM1P, HAM56, lysozyme, and alpha 1-antitrypsin were positive. However, macrophage markers MAC387 (L1 antigen) and Leu-M1 (CD15) were negative. Vimentin was universally positive in both conditions, with all other markers (S100, desmin, smooth muscle-specific actin, and QBEnd 10 [CD34]) negative. This study shows that histology supplemented by immunocytochemistry delineates MR from RH and immunohistochemical profiles indicate a cell lineage relationship between RH and adult xanthogranuloma.

Adolescent↗
PubMed1994

Juvenile and adult xanthogranuloma. A histological and immunohistochemical comparison.

Thirteen cases of juvenile xanthogranuloma (JXG) and 13 cases of adult-type xanthogranuloma (AXG) were compared at the light and immunohistochemical levels. Histologically, four main cell types (vacuolated, xanthomatized, spindle-shaped, and "oncocytic") were seen in variable proportions (from monomorphous to mixed variants) with different types of giant cells (nonspecific, foreign body, Touton, and "ground-glass"). Giant cells were more prominent in AXG than in JXG; oncocytic cells (characterized by an eosinophilic, slightly granular cytoplasm similar to thyroid oncocytic cells) and mostly periodic acid-Schiff (PAS) negative giant cells with a ground-glass appearance (6 of 26) were not observed in classic JXG (i.e., occurring in children < 2 years old). Immunohistochemically, JXG and AXG gave similar results: most xanthogranuloma cells labeled strongly with KiM1P and vimentin, while HHF35 and HAM56 stained less intensively. Factor-XIIIa (FXIIIa), KP1 (CD68), and HAM56 stained mostly in the periphery of the lesions. Some markers gave variable results: peanut agglutinin (PA), 60%; alpha-1-antitrypsin, 50%; lysozyme, 25%; LN3 (HLA-DR), < 10% of cells positive. Others were negative: S-100, MAC387 (L1 antigen), LeuM1 (CD15), desmin, smooth muscle-specific actin, and QBEND10 (CD34). This profile helps to delineate xanthogranuloma from histological stimulants such as dermatofibroma (which is FXIIIa+, LN3+, KP1-, and PA-) and multicentric reticulohistiocytosis (which is FXIIIa-, KP1+, PA-, and HHF35-).

Adolescent↗
PubMed1994

Deep penetrating dermatofibroma versus dermatofibrosarcoma protuberans. A clinicopathologic comparison.

A study of the clinical, histological, and immunohistochemical features of 20 cases of deep penetrating dermatofibroma (DPDF) and eight cases with 14 specimens (eight primary, one reexcision, five secondary tumors) of dermatofibrosarcoma protuberans (DFSP) showed distinct entities. Clinically, DPDF usually appeared as a nodule (approximately 2 cm) of the (lower) limbs, whereas DFSP affected the trunk (shoulder) with irregularly arranged plaques or nodules (> 5 cm). Histologically, DPDF showed a regular silhouette with a smooth, nodular (four of 20) or scalloped (16 of 20) lower margin and variable sclerosis (nine of 20); DFSP, irregularly infiltrated fatty tissue in a lacelike/honeycomb (eight of 14), multilayered (three of 14), or mixed pattern (three of 14), but without sclerosis. Immunohistochemically, DPDF was mostly negative with QBEnd 10 (CD34; 18 of 20) but positive for factor XIIIa (17 of 20), actin (HHF35; 10 of 20), and metallothionein (MT; 12 of 20). DFSP was positive for CD34 (13 of 14), yet with some sparing of central tumor parts, highly cellular tumor nodules, and myxoid areas; factor XIIIa and MT were consistently negative, as was HHF35 in 11 of 14 cases. In a multivariate analysis of histologic and immunohistochemical criteria, the combination of sclerosis and labeling with MT was most valid (p = 0.0001) for diagnosis: all DPDF showed either labeling with MT in "early" (metabolically active) lesions or sclerosis in "late" lesions, not present in DFSP.

Adult↗
PubMed1994

Syringotropic cutaneous T-cell lymphoma: a variant of mycosis fungoides?

We report two patients with the typical clinical (patches/plaques studded with brownish-red papules) and histopathological (hyperplastic eccrine ducts and glands surrounded and infiltrated by lymphocytes) features of syringotropic cutaneous T-cell lymphoma. Immunohistochemical studies confirmed the T-cell character of the infiltrate, and gene rearrangement studies its monoclonality (in one case). Our patients did not present with visceral involvement.

Adult↗
PubMed1993

Sneddon's syndrome. A long-term follow-up of 21 patients.

BACKGROUND AND DESIGN: Twenty-one patients with histologically proven Sneddon's syndrome were followed up in a retrospective study. We report on their detailed clinical courses and extensive follow-up examinations. RESULTS: Incidence is estimated at four cases per million population per year. Nonspecific prodromal symptoms (headache, dizziness) frequently (80%) precede livedo racemosa for 3.5 and (multi)focal neurological symptoms of fully developed disease for 9 years followed by progressive cognitive impairment (60%) 10 years later. Involvement of fundi, peripheral nerves, heart, and kidneys is frequent (50% to 70%) yet usually asymptomatic. Some symptoms prove irreversible (livedo racemosa, multifocal cerebral lesions on imaging, or creatinine clearance), whereas other symptoms tend to resolve after days to years (many focal neurological symptoms, some electrocardiographic changes, or hypertension). Mortality is calculated at 9.5% within an average observation time of 6.2 years. Laboratory findings, including antiphospholipid antibodies, are normal except for elevated erythrocyte sedimentation rates and complement consumption at times of disease progression and increased cholesterol levels parallel to disease extent. Skin biopsy specimens reveal inflammatory findings ("endothelitis") of small- to medium-sized arteries followed by subendothelial proliferation and fibrosis. Hypertension is the only risk factor significantly associated with a more severe course of the disease; no medication proved effective. CONCLUSIONS: Sneddon's syndrome is an often unrecognized, slowly progressive, systemic disease with evidence of vasculitic origin.

Adult↗
PubMed1993

Synovial metaplasia of the skin.

We present two female patients, aged 15 and 45 years, with synovial metaplasia of the skin. Both lesions, localized in the knee and hand respectively, arose after preceding local trauma. Case 1 had intradermal irregular cystic spaces in the adjacent myxoid stroma of which large polygonal eosinophilic cells were found. In contrast, case 2 was characterized by a longitudinal space within the lower dermis and subcutis which was lined by a membrane similar to hyperplastic synovium. The cells of the membrane showed an eosinophilic spindle shaped cytoplasm with processes towards the lumen. In both cases the eosinophilic cells, strongly suggestive of fibroblasts, showed staining for vimentin only, whereas no reactivity could be obtained with antibodies to actin, desmin, S-100 protein, Factor VIII related antigen, cytokeratin, epithelial membrane antigen, carcinoembryonic antigen and CD 68. The absence of CD 68 positivity differs from that seen in normal and hyperplastic synovium. Delayed wound healing around a nidus seems the most likely cause of the change.

Adolescent↗