[Comment on the contribution by N. Tsankov and S. Tonev: Ghirlandajo's painting "Grandfather and Grandchild" from the viewpoint of the dermatologist].
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Biomedical subjects
Publications and source records attributed to G W Korting.
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Skin lesions, especially at areas exposed to sunlight, prove to be a major form of manifestation of diseases related to Ro-antibodies and neonatal-, 'ANA-negative-', and cutaneous types of lupus erythematosus. A monoclonal Ro-antibody established by our group reacts with a 60 kD polypeptide in extracts from human spleen, whereas in extracts from human epidermis the monoclonal Ro-antibody and a purified Ro-antibody from a monospecific serum of a patient with subacute cutaneous lupus erythematosus reacted with a 60 kD and a 48 kD protein. Performing immunofluorescence microscopy on HEp2-cells both antibodies showed a nuclear speckled staining pattern and a reaction with cytokeratin filaments. On skin biopsies obtained from the patient, predominantly the basal layers of the proliferative stratum germinativum demonstrated a high rate of immunofluorescence; antigen-expression seems to depend on sunlight exposure. From these data we assume that, especially in subacute cutaneous lupus erythematosus, cross-reaction of the Ro-antibody with one of the lower molecular keratins happens in that area of human epidermis which histologically shows cellular destruction.
We investigated polymorphonuclear leukocyte (PMN) function in a 50-year-old woman with primary Sjögren's syndrome (SS). The respiratory burst of PMN was monitored by luminol-enhanced chemiluminescence using zymosan, opsonized zymosan, zymosan-activated serum, and phorbol-myristate-acetate, as well as serial dilutions of aggregated immunoglobulin (aggIg) as Fc-receptor (FcR) stimulus. The effects of serum on the chemiluminescent response as well as the binding of aggIg to PMN were also compared. We found the patient's PMN not to respond to stimulation by aggIg, only the highest concentration (greater than 180 micrograms/ml) induced a marginal chemiluminescent response in the patient. By contrast, incubation of the patient's PMN with other stimuli resulted in responses similar to those in a healthy control. Binding of aggIg to PMN was higher in the patient (3.6% vs 1.5% of the radioactivity added in the control). Sera of patient and control induced similar chemiluminescence on PMN as did that of another human serum. Our data indicate a selective functional FcR defect of PMN despite unimpaired binding of aggIg in a patient with SS.
A de novo carcinoma of only a few weeks' duration is reported in a 34-year-old man. Histological findings revealed a well-differentiated squamous cell carcinoma with unexpected early spread deep in the dermis and subcutis and a striking affinity to the neural structures.
A patient with Sjögren's syndrome exhibited markedly impaired stimulation of her granulocytes via Fc-receptors (FcR), four other stimuli elicited a normal chemiluminescent response. Unexpectedly, granulocytes of the control bore 39,000, of the patient, however, 86,000 FcR/granulocyte.
We report on two cases of coexisting lupus erythematosus and hepatic porphyria and give a review of the corresponding literature. It is not clear yet, if the coincidence is accidental or if there exist any unknown relationship. 29 patients with these two conditions were found in the literature.
Cutaneous angiosarcoma of the head is a rare tumor of the elderly and can occur in an undifferentiated form without any clinical or histological signs of the vascular origin of this tumor. In these cases, the tumor can be identified by using endothelial cell markers, such as factor-VIII-related antigen and ulex europaeus agglutinin I, in an immunofluorescence technique or a peroxidase-antiperoxidase method. A 78-year-old patient is described who died within 18 months from such a tumor, which was diagnosed using the endothelial cell marker, ulex europaeus agglutinin I.
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The occurrence of polyneuropathy in connection with systemic sclerosis is extremely rare. Proliferative changes leading to mechanical compression of the nerves have been ascribed to an etiological involvement. In the case reported here, polyneuropathy could be verified morphologically (nervebiopsy). The elevated IgG in the cerebrospinal fluid suggests inflammatory changes of the nerves to be the cause of polyneuropathy.
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Side effects of bleomycin therapy are most frequent in the skin and as pulmonary fibrosis because of the nearly selective concentration in these organs. The cutaneous manifestations include a variety of clinical symptoms, most frequently erythema and infiltrations with marked hyperpigmentation. A 38-year-old patient is reported who received 615 mg bleomycin for therapy of a malignant testicular teratoma and in the following time developed numerous morphoea-like lesions, acrocyanosis, Raynaud's phenomenon, acrosclerosis, and cutaneous calcinosis. Histologically, the alterations were similar to those of scleroderma. In contrast to progressive systemic sclerosis the symptomatic character of the lesions is evident, for the skin changes resolved several months after the drug was discontinued.
A patient with constant intumescences of the forehead due to bilateral hypertrophy of mimic muscles is reported.
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Human melanomas were investigated for the presence of high-affinity estrogen-, gestagen-, and glucocorticoid-binding proteins. A statistically significant difference was found for mean estrogen receptor (ER) concentrations in melanomas of male versus female origin: female origin 37.6 (0-107) fmol/mg protein, male origin 3.9 (0-8.3) fmol/mg protein. No significant difference between sexes was found for gestragen receptors: 41.5 (0-194) fmol/mg protein for melanomas of female origin versus 99 (0-362) fmol/mg protein for male. Sucrose density gradient analyses revealed specific binding for both receptor types in the 4-5 S region as well as in the 8 S region. The binding affinities were in the same order of magnitude as reported for receptors found in typical steroid target organs. No significant difference in receptor values depending on sex was found for the glucocorticoid receptor: 19.2 (0-43) fmol/mg protein.