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Biomedical subjects

G Burg

Publications and source records attributed to G Burg.

At least 253 records · Page 14Linked to original sources

Report from the president (V) 1992-1994.

During the past 3 years, from 1992 to 1994, the Society has accomplished many of its goals of being an international society. However, activities must continue especially in developing dermatopathology in less and least developed countries. There is no space here to mention the many scientific, oral, and publication activities of our members who have contributed to the symposia, colloquia, and other events sponsored by the ISDP, and who deserve many thanks for their contributions to the realization of the intellectual mood of our Society.

Congresses as Topic↗

Primary cutaneous T-cell-rich B-cell lymphoma. A case report with a 13-year follow-up.

Cutaneous B-cell lymphomas constitute approximately 20% of primary cutaneous lymphomas. Most histologic subtypes of nodal B-cell lymphomas also occur primarily in the skin. The recently described T-cell-rich B-cell lymphomas (TCRBCLs) manifest mainly in the lymph nodes. This article presents a case of TCRBCL arising primarily in the skin, the origin of which could be traced back 13 years. The patient is a 59-year-old man. Plaque-like and nodular skin infiltrates had first appeared in the left preauricular region. Repeated examinations never found any extracutaneous involvement. A skin biopsy and a retrospectively studied 10-year-old skin specimen showed identical histologic features. Immunohistochemistry identified the TCRBCL previously considered as cutaneous Hodgkin's disease or a diffuse centroblastic centrocytic non-Hodgkin's lymphoma. A clonal B-cell population was detected by polymerase chain reaction, showing a rearrangement of IgH gene. The case of this patient shows that primary cutaneous TCRBCLs, similarly to other B-cell lymphomas in the skin, may have a good prognosis, in contrast to their nodal counterparts.

Biopsy↗

Relapsing eosinophilic perimyositis.

Relapsing eosinophilic perimyositis is a rare entity. Only 11 cases have been reported in the literature. We describe a patient with relapsing myopathy, peripheral blood eosinophilia and cutaneous manifestations, who had histopathological features of eosinophilic perimysial inflammation. His disease responded to moderate doses of glucocorticosteroids, and pursued an apparently benign course. Serum levels of eosinophilic cationic protein correlate with disease activity, and may be helpful in monitoring treatment. The features of this case are compared with those of other hypereosinophilic syndromes. It appears to be part of the spectrum of eosinophilic myositis.

Anti-Inflammatory Agents↗

Necrotizing herpes zoster mimicking relapse of vasculitis in angioimmunoblastic lymphadenopathy with dysproteinaemia.

An 81-year-old man presented with a generalized maculopapular rash, lymphadenopathy, conjunctivitis and arthritis. Vasculitis was confirmed by skin biopsy and by direct immunofluorescence, which showed perivascular C3 and granular IgM accumulation. Histology of an inguinal lymph node was diagnostic for angioimmunoblastic lymphadenopathy with dysproteinaemia (AILD), and this was confirmed by the finding of hypergammaglobulinaemia and elevated IgE levels. Immunohistology on a lymph node biopsy showed a T-helper cell (CD4) infiltrate expressing the interleukin (IL)-2 receptor alpha and beta chains. While receiving prednisone 100 mg/day, the patient developed new lesions, mimicking a relapse of vasculitis, which were subsequently shown to be necrotizing herpes zoster. Serum IL-2 and IL-6 levels were elevated. To our knowledge, this is the first report of simultaneous elevation of IL-2 and IL-6 in AILD: IL-2 may be involved in proliferation of the malignant cell clone, and IL-6 in the pathogenesis of both the vasculitis (via endothelial cell activation) and the hypergammaglobulinaemia.

Aged↗

Staging of metastatic melanoma by whole-body positron emission tomography using 2-fluorine-18-fluoro-2-deoxy-D-glucose.

Metastatic melanoma was staged in 15 patients using whole-body positron emission tomography (PET) and the radiopharmaceutical 2-fluorine-18-fluoro-2-deoxy-D-glucose (FDG). PET correctly demonstrated 30 metastases in lung, brain, pancreas, nasal cavity, skin and subcutaneous tissue, and lymph nodes. It detected 97% of all metastases exceeding its spatial resolution (> 5 mm). Two cutaneous metastases (approximately 3 mm) did not show increased FDG uptake; the overall detection sensitivity was 91%. Two false-positive lesions in one patient were due to severe wound infection. PET correctly excluded malignancy in four cases where suspicious lesions were found with conventional cross-sectional imaging modalities but later ruled out by fine-needle biopsy. PET therefore proved to be an excellent method for staging of metastatic melanoma. Due to its high sensitivity for malignant lesions and the possibility of covering the whole body in one examination, it can replace staging techniques employing multiple imaging modalities: chest X-ray, ultrasonography and computed tomography. Furthermore, it provides information on the malignant potential of the detected lesion.

Adult↗

Scalp dermatitis, distinctive hair abnormalities and atopic disease in the ectrodactyly-ectodermal dysplasia-clefting syndrome.

We report a 2-year-old girl with a combination of the ectodermal dysplasia, ectrodactyly, cleft lip/palate (EEC) syndrome, distinctive hair abnormalities, scalp dermatitis and atopic disease. To our knowledge, this is the first report of scalp dermatitis in the EEC syndrome. Distinctive structural hair abnormalities shared by the syndromes of ectodermal dysplasia and clefting are helicotrichia, pili torti et canaliculi, and cuticle defects, all of which were observed in the present case. A high incidence of atopic disease in certain subsets of ectodermal dysplasia has been reported. In our patient this manifested as food-induced exacerbation of atopic dermatitis, associated with positive prick tests and significant levels of circulating specific IgE.

Abnormalities, Multiple↗

Assessment of skin irritancy by 2 short tests compared to acute irritation induced by sodium lauryl sulfate.

Irritant contact dermatitis is a very common disease that is preventable by protective measures. The development of screening methods to identify subjects with increased susceptibility to irritants is essential to reduce the incidence of this disorder in the workplace. On the outlook for such methods, 2 quick non-invasive tests for irritability of the skin were compared to reliable, but time-consuming, patch testing with sodium lauryl sulfate (SLS). In 20 healthy volunteers, 0.5% SLS was applied on the medial 1.3 of the forearm for 23 h in order to induce experimental irritant contact dermatitis. On the same part of the forearm, 3 concentrations of dimethylsulfoxide (DMSO) and a solution of 0.2 mol/l NaOH were applied for 5 min. Assessment of skin irritability was made by visual scoring and measurement of transepidermal water loss. No correlation between the "quick tests" and SLS patch testing was found, indicating that these tests assess different mechanisms of irritation.

Acute Disease↗

CD44v6 is a marker for systemic spread in cutaneous T-cell lymphomas. A comparative study between nodal and cutaneous lymphomas.

Adhesion molecules are involved in leukocyte recruitment, lymphocyte recirculation, and in several aspects of tumour biology. Recent discoveries of surface proteins on tumour cells involved in tumour metastasis may explain the invasive behaviour, the migration involving reversible adhesive contacts, the release into the circulation and the extravasation of tumour cells. CD44 is a family of glycoproteins involved in cell-cell and cell-matrix interactions. The v6 (variant exon v6) form of CD44 confers a metastatic potential onto some carcinoma cells. In the present study, the expression of CD44v6 on skin biopsies of 10 inflammatory skin diseases, 30 cutaneous lymphomas (CL), 11 reactive lymph nodes, 10 primary nodal non-Hodgkin's lymphomas (NHL) and 5 secondary nodal NHL was investigated immunohistochemically. None of the 10 nodal NHL were CD44v6 positive for the neoplastic B- or T-cells, whereas 11/12 CL with systemic spread showed a distinct CD44v6 expression in the skin. CD44v6 was not expressed on the tumour cells of skin biopsies of patients without systemic spread (18 cases of CL). In conclusion, CD44v6 expression is connected to an aggressive behaviour of CL.

Adult↗

Malignant melanoma: staging with whole-body positron emission tomography and 2-[F-18]-fluoro-2-deoxy-D-glucose.

PURPOSE: To evaluate whole-body positron emission tomography (PET) with 2-[fluorine-18]-fluoro-2-deoxy-D-glucose (FDG) in the detection of metastasis from melanoma. MATERIALS AND METHODS: Whole-body PET was performed in 33 patients with either known metastatic or newly diagnosed melanoma. Patients with suspected metastases also underwent computed tomography, magnetic resonance imaging, or both. Diagnoses were confirmed with histologic examination or with at least one imaging modality in addition to PET. Blinded interpretations of PET scans were performed. RESULTS: Forty of 53 lesions evaluated proved to be melanoma metastases. Whole-body PET correctly depicted 37 sites of metastases. Three cutaneous metastases (< 3 mm) were missed. PET correctly excluded malignancy in 10 cases where suspicious lesions were found with conventional cross-sectional imaging modalities but later ruled out with fine-needle biopsy. In six patients, PET depicted new metastases. The sensitivity for the detection of malignant lesions was 92%; the specificity for reading the PET images without clinical information was 77% and with clinical information was 100%. CONCLUSIONS: These results suggest that whole-body FDG PET is an effective imaging modality to screen for metastases from malignant melanoma.

Adult↗

Anti-melanoma monoclonal antibody 225.28S immunoscintigraphy in metastatic melanoma.

BACKGROUND: Accurate staging and early detection of metastases are crucial to melanoma management. It would thus be of great value to have a widely available, cost-effective method that allows to examine the whole body and is more specific than current imaging modalities. OBJECTIVE: The purpose of the study was to assess the value of immunoscintigraphy with 99mTc-radiolabeled F(ab')2 fragments of the 225.28S monoclonal antibody in the staging of melanoma. METHODS: 29 patients with known or suspected metastases were enrolled in the study and examined by monoclonal antibody fragment scintigraphy. The results were compared to physical examination, other imaging modalities and, where available, histology. RESULTS: Antibody scintigraphy detected 9/34 metastases. There were 2 false-positive lesions. Diagnostic accuracy was 41%, sensitivity 26% and specificity 83%. CONCLUSION: 99mTc-radiolabeled F(ab')2 monoclonal antibody-fragment scintigraphy cannot be recommended for staging of melanoma patients. The search for more sensitive radiopharmaceuticals for scintigraphy should thus be encouraged.

Adult↗

Dermatofibroma: an abortive immunoreactive process mediated by dermal dendritic cells?

Dermatofibromas are very common tumors of the skin, but little is known about their etiology and pathogenesis. Current concepts of disease pathogenesis are discussed, with special emphasis on an immunoreactive origin. There is recent evidence, that high numbers of cells with dendritic morphology and positive staining for factor XIIIa are concentrated at the periphery of the lesions. Furthermore, they express MHC class II molecules and costimulatory molecules such as B7-1 and B7-2 on their surface. Thus, there are similarities to professional antigen-presenting cells of the dendritic cell family, so-called dermal dendritic cells (DDCs), which have recently been identified in the human dermis. A concept is developed which explains DF as an abortive immunoreactive process, featuring DDCs as initiators of the disease.

Acquired Immunodeficiency Syndrome↗

Lymphomatoid papulosis--treatment with recombinant interferon alfa-2a and etretinate.

Lymphomatoid papulosis is a rare cutaneous lymphoproliferative disorder with nodular, papulonecrotic or plaque-like lesions. Although it is clinically benign, the histology shows large, atypical lymphoid cells that display antigenic markers of activated T-helper lymphocytes and express CD30. There is a close relationship to Hodgkin's disease and to Ki-1-positive anaplastic large-cell lymphoma of the skin. For therapy, various modalities such as PUVA, steroids and acyclovir have been used. We report on a patient with a 10-year history of disease. Treatment with interferon alfa-2a, 3 MU 3 times/week for 4 weeks, and etretinate, 50 mg/day for 5 months, was initially successful, but lesions further relapsed 5 months after cessation of the therapy.

Adult↗

Expression of MxA protein in inflammatory dermatoses.

The human MxA protein can be detected in the cytoplasm of IFN-alpha/beta-treated cells, whereas other cytokines, including IFN-gamma, are poor inducers. Because IFN-alpha/beta is predominantly synthesized in response to viral infections, MxA protein should be detectable in virally infected tissue. Biopsy specimens (n = 64) of 12 different dermatoses were therefore screened with an MxA-specific monoclonal antibody on formalin-fixed, paraffin-embedded and microwave-treated tissue sections. As expected, high amounts of MxA protein were found in acute viral skin lesions (chickenpox, Herpes zoster, and Herpes labialis). In addition, MxA protein was also detected in some inflammatory skin lesions of unknown etiology (lupus erythematosus, lichen planus, Schoenlein-Hennoch's anaphylactoid purpura and psoriasis). MxA protein was not found in non-viral infections (bacterial, mycotic, and parasitic) and was also not detectable in various other dermatoses (eczema, scleroderma, urticaria, granulomatous and bullous disorders). MxA staining proved a reliable, sensitive histochemical viral marker for infectious dermatoses. The positive results in non-infectious inflammatory dermatoses might implicate viral involvement or activation of the IFN system by thus far unknown mechanisms.

Antibodies, Monoclonal↗

[Skin symptoms in neurological diseases].

The considerable variety of diseases involving both the skin and the peripheral or central nervous system is due to the developmental relationship between skin and nervous system. Diseases of the nervous system resulting in skin changes comprise disorders of nails, hair, sweat and sebaceous glands as well as neuropathic ulcers in various manifestations. Neurocutaneous syndromes (phacomatoses) are nevoid systemic diseases comprising neurofibromatoses, tuberose sclerosis, ataxia telangiectasia, nevoid angiomatoses and other neurocutaneous disorders. Polytopic disorders of the skin and nervous system may be due to infectious diseases, diseases of the connective tissue, fat, inflammatory vascular and granulomatous disorders.

Ataxia Telangiectasia↗