[Skin diseases and monoclonal gammopathies].
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Biomedical subjects
Publications and source records attributed to E Macher.
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A 55-year-old white woman received chemotherapy with DTIC after surgery for malignant melanoma (stage I, SSM IV, depth of invasion 12 mm). She died suddenly during the second treatment cycle. Autopsy revealed massive necrosis of the liver and thrombosis of the hepatic veins. The cause of the fatal outcome is attributed to the adverse toxic effects of DTIC.
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Sera from melanoma patients, healthy donors, pregnant women and patients with types of tumors other than melanoma were tested on various melanoma lines as well as on a cultured brain tumor and adult skin fibroblasts, using a microimmune adherence test. Positive reactions against all cell lines were found in serum from each group of donors. The degree of reactivity was dependent on the cell line used. Sequential absorption with AB Rh+ and pooled platelets of more than 200 donors either reduced the titer of sera or rendered a great part of the sera negative, demonstrating that antibodies against HL-A antigens and other tissue antigens were involved. The remaining positive sera were further absorbed with pooled cells from 6- to 8-week-old fetuses. This step abolished the reactivity of most sera, indicating the relatively high frequency of antibodies in males and females against fetal antigens expressed also on melanoma and other cells. In order to determine the specificity of the few remaining positive sera, absorptions with three different melanoma cell lines, a brain tumor and fibroblasts were carried out. The results showed only partial cross-reactivity between different cell lines. No evidence was obtained from this study for the existence of a common cross-reacting membrane-associated antigen on human malignant melanoma. Antigens that could be readily detected seemed mostly to be tumor-associated fetal antigens.
Widespread atrophic lesions and pigmentary disturbances of the skin distributed in a linear or whorled pattern, are seen in some patients with chondrodysplasia punctata of the Conradi-Hünermann type. Arguments are presented in favor of the hypothesis that this association of anomalies constitutes a distinct genetic disorder, which is inherited as an X-linked dominant trait lethal in hemizygous males.
In a patient with systematized verrucous epidermal nevus, marked improvement was obtained with oral administration of a new aromatic retinoid (Ro 10-9359). Mild cheilitis and thinning of scalp hair were the only side effects obser Withved. continuous treatment the good result was maintained for 10 months. Further investigation is needed to determine, whether long-term oral administration of this new drug is feasible.
We have observed non-myelomatous monoclonal hypergammaglobulinaemia in a 61-year-old man with Sézary syndrome. Similar cases have not been reported before. Sézary cells of patients studied by other investigators were found to be helper T cells. The abundant production of T-lymphocytic stimuli may possibly explain in our case the occurrence of a paraprotein.
A case of linear pigmentation of three fingernails is reported. Following extraction of the nail of the thumb the matrix displayed a 2X4 mm circumscribed pigmented area. Histologic examination revealed a junction nevus.
Melanoma cells carry membrane-bound antigens that induced both antibody production and cellular immunity. However, these antigens appear not to be tumor-specific, as the activity of human antisera can be absorbed out by fetal antigens. Nonetheless, the phenomenon of spontaneous regression, though mostly affecting only parts of a lesion, indicates that effective attack mechanisms do exist. Simultaneous tumor progression is due to heterogeneity of tumor cells, which vary widely in antigen expression. Cells that are not recognizable sneak through defense mechanisms and produce metastases.
In a 22 year-old female patient affected with focal dermal hypoplasia, multiple apocrine nevi occurred in the sternal region; these nevi may be part of the syndrome. Roentgenograms of the long bones revealed marked longitudinal striations. This anomaly represents a characteristic and hitherto underestimated feature of the syndrome.
Two cases of keratosis palmo-plantaris cum degeneratione granulosa Voerner are reported. A brief description of the characteristics of the clinical and histologic aspects of the disease is given and the mode of inheritance within the family affected is discussed.
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