Biomedical subjects
Publications and source records attributed to G Burg.
Three cases of LEOPARD-syndrome are presented. A review of literature is given. Among the symptoms of the syndrome lentigines, ECG-abnormalities, pulmonal-stenosis, retardation of growth and deafness show the strongest penetrance and are most constantly found.
Semi-thin sections are superior to paraffin sections for differentiating lymphoid cells. To test their possible significance in the diagnosis of cutaneous T-cell lymphomas, the following investigation was made: A quantitative analysis of lymphoid cells in the cellular infiltrate of cutaneous lesions of mycosis fungoides (11 cases), Sézary's syndrome (9 cases), and parapsoriasis en plaques (14 cases) was made using semi-thin sections of epon-embedded biopsies. As controls biopsies of patch test reactions, contact dermatitis, initial psoriasis, and erythema chronicum migrans were studied. A high percentage of Lutzner (Sézary) cells was consistently found in Sézary's syndrome and occasionally in parapsoriasis en plaques only. In mycosis fungoides lymphocytoid cells with the nuclei less dense than in normal lymphocytes were predominant. In most tumorous lesions of mycosis fungoides and of Sézary's syndrome an increased percentage of immunoblasts was noted. Normal lymphocytes were the most frequent cell type in parapsoriasis en plaques as well as in the controls. These findings may prove helpful in the diagnosis of problem cases, for which the use of semi-thin sections is recommended.
Eighteen cases of low grade malignant B-cell lymphomas (confirmed through enzyme cytochemistry and immunocytology) were investigated morphologically using thin and semithin sections. Four types with a characteristic pattern of different lymphoid cells were found: (1) the lymphocytic type, predominantly consisting of small lymphocytes; (2) the lymphocytoid (centrocytic or prolymphocytic) type, in which in addition to small lymphocytes medium-sized lymphocytes (with or without cleaved nuclei) are found; (3) the lymphoplasmacytoid type, where lymphoid cells with different degrees of similarity to plasma cells are seen as well as small and medium-sized lymphocytes; and (4) the centroblastic-centrocytic type, in which centroblasts are present next to small and medium-sized lymphocytes. These findings correlate well with the morphological observations in low-grade malignant B-cell lymphomas of the lymph nodes. Therefore, the application of modern classifications of non-Hodgkin lymphomas, such as the "Kiel Classification", to cutaneous lymphomas appears justified. Thin and semithin sections are particularly useful in suspected cases of cutaneous malignant lymphoma, in which paraffin sections did not allow the diagnosis to be established.
Report of a 42 year old woman who had given up her profession as a hairdresser because of urticarial skin eruptions following contact with substances used during working. An open patch test showed after 30 min positive urticarial reactions against permanent wave solution, fixation solution, p-aminodiphenylamin, lanolin-alcohol and clioquinol.
Evaluation and classification of malignant lymphomas have changed decisevely in recent years. It was especially through immunology that these new discoveries were made. Some namely the immunocytoma and immunoblastic lymphoma. These lymphoma-entities of the non-Hodgkin-group are in the majority of cases neoplastic analogues of transformed B-lymphocytes. Clinico-pathologic correlations and the enzymecytochemical characterisation of the tumor cells are also described in this paper.
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A further case of pagetoid reticulosis (PR) was studied, and the findings were compared with 18 previous case reports in the literature. There seem to be two variants: The generalized type (Ketron-Goodman) with disseminated lesions and a poor prognosis, and the localized type (Woringer-Kolopp), with a single lesion usually located on the extremities with a good prognosis (if the lesion can be totally cleared by excision or highdose-radiation). The basic criterion of diagnosis is a very strong cellular infiltrate within the epidermis, but usually weak in the upper dermis. The etiology of PR remains unclear: In some cases a T-cell lymphoma seems a likely interpretation. In others, epidermal infiltrating cells are not typically lymphoid, but remain morphologically unclassifiable.
Report of a 59 year old man who suffered from spontaneously healing multiple keratoakanthomas of the face and neck for five years. Multiple keratoakanthomas, multiple primary squamous-cell carcinomas with spontaneous healing and tumor-like keratoses probably are variants of the same disease. The etiology of multiple keratoakanthomas is unknown. Clinically as well as histologically they are similar to solitary keratoakanthomas and show spontaneous healing. Thorough clinical control of the patients is necessary because of the possible transformation into squamous cell carcinomas.
Skin infiltrates and tumors characteristic of mycosis fungoides, together with other clinical features typical of Sézary's syndrome (such as erythroderma, itching and Sézary cells in the peripheral blood), were found in a 76-year-old female. Histologically, there was a shift from a well-differentiated to a poorly-differentiated lymphoblastic infiltration indicating the transformation from a low-grade to a high-grade malignant (T-cell) lymphoma.
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Enzyme cytochemical (hydrolytic enzymes for cell differentiation), immunocytologic (B and T lymphocyte differentiation), and electron microscopic studies of skin infiltrates facilitate the proper diagnosis of myelomonocytic and lymphoreticular proliferations. With use of these methods, the original clinical diagnosis of malignant reticulosis of the skin was corrected to monocytic leukemia in a 65-year-old woman. Because primary involvement of the skin preceded monocytosis of the blood, it was concluded that the cutaneous infiltrates in our patient resulted from proliferation of tumor cells in the skin rather than from homing of the cells to, or settling of the cells in, the skin.
It was the purpose of this study to characterize the proliferating cells in skin lesion of Sézary's syndrome and of mycosis fungoides by means of their surface markers and their response to Phytohemagglutinine mitogen stimulation. Viable infiltrating cells were freed from skin biopsy specimens by means of a disaggregating homogenizer and the cells yielded were tested with heterologius polyvalent anti-human Ig and with anti-human T-cell globulin, as well as for spontaneous rosette formation with sheep red blood cells (SRBC) and for their response to stimulation with Phytohemagglutinine. Most of the infiltrating cells in skin lesions of mycosis fungoides and Sézary's syndrome lack receptors for anti-human Ig but form spontaneous rosettes with SRBC and have receptors for anti-T-cell globulin, indicating the T-lymphocyte nature of the infiltrating cells; however, their response to Phytohemagglutinine is weak. The results indicate the atypical, presumably neoplastic, nature of T-lymphocytes proliferating in skin lesions of mycosis fungoides and Sézary's syndrome.
During recent years there has been much progress in interpreting the histo- and cytomorphology of lymphoreticular proliferations by means of enzyme cytochemical (cell typing by hydrolytic enzymes) and immunological (B- and T-cell differentiation using surface markers and functional tests) methods. Applying these methods in skin biopsies from 101 patients clinically suspected of having cutaneous lymphomas, patterns of lymphoreticular infiltrations in the skin have been elaborated. Based primarily on the 'Kiel' classification, low-grade and high-grade malignant lymphomas, pseudolymphomas and 'histiocytic lymphomas' can be differentiated in the skin; however, there still remain some hitherto unclassifiable lymphoreticular proliferations. In the low-grade malignant lymphomas of the skin, mycosis fungoides, Sézary's syndrome and Pagetoid reticulosis histologically display a pattern which is typical for T-cell infiltrations in the skin, whereas most of the 'malignant reticuloses', including immunocytoma, show a B-cell pattern. Erythrocyte antibody complement rosette fixation on cryostat sections is positive only in cutaneous pseudolymphomas whereas no fixation is seen in malignant B-cell lymphomas of the skin.
A 76 year old female developed an acrodermatitis-chronica-atrophicans-(ACA)-like lesion on her foot. Histological examination and quantitative determination of immunoglobulins in homogenates from the skin lesion revealed an immunocytoma of the IgM type. Malignant lymphoma originating in ACA lesions or concomitting ACA are well known. The case described here indicates that primary malignant lymphoma of the skin clinically can simulate ACA and creates diagnostic, therapeutic and prognostic problems.
A case of acquired digital fibrokeratoma is presented. The asymptomatic cylindrical tumor preferably occurs during the third to sixth decade of life and affects mainly the fingers. Histologically one finds a cylindriform connective tissue proliferation surrounded by an akantholytic band of epidermis with papillomatous changes and hyperkeratosis. For differential diagnosis rudimentary supernumerary digit, cutaneous horn and granuloma pyogenicum are to be considered. Therapy consists of excision of this benign tumor of hitherto unknown origin.
8-Methoxypsoralen (8-MOP) and long-wave ultra-violet light (UVA) were administered to 16 patients with mycosis fungoides and to three with other cutaneous lymphomas. Total body clearing was achieved in eleven patients, while temporary recurrences were observed in three, maintenance treatment arresting the recurrences. In the others all treatment failed. Histological and enzyme-cytochemical studies indicate that the histological features under 8-MOP-UVA treatment correspond to those induced by other forms of treatment: a lose enzyme-cytochemically negative lymphocytoid infiltrate remaining around blood vessels, after regression of the skin disease. Oral 8-MOV-UVA treatment (12 patients) proved superior to topical treatment (seven patients), because cutaneous lymphoma is a systemic disease. In mycosis fungoides the mean number of exposures causing clearance of skin lesions and the mean UVA dose (single treatment) were generally less than those for psoriasis vulgaris.
In an ultrastructural study supplementing previous histological, cytochemical and immunocytological investigations of lymphadenosis benigna cutis, the tumor is shown to consist mainly of two cell types: Lymphocytes and macrophagocytic (reticulum?) cells. The tendency to spontaneous regression is manifested on the ultrastructural level by pronounced degenerative alterations.
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