Atypical Kaposi's sarcoma in a patient with vitiligo and pernicious anemia.
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Biomedical subjects
Publications and source records attributed to T Ruzicka.
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We report the case of a 15-year-old girl with a uneventful family history. Her skin condition was clinically, histologically and ultrastructurally compatible with the diagnosis of ichthyosis congenita. She suffered from neurosensory deafness and oligophrenia. Further findings included dental aplasia, brachydactyly, clinodactyly and accessory cervical ribs. At the age of 14, a thyroid carcinoma was diagnosed. Therapy with a retinoid derivative (Ro 10-9359) resulted in a marked improvement of the ichthyosis. We assume a genetic syndrome with autosomal-recessive inheritance.
Dapsone 100 mg daily led to a rapid and complete clearing of extensive vasculitic urticarial lesions in a female patient with systemic lupus erythematosus. Complement determinations revealed an intensive activation of the classical pathway before dapsone therapy. Normalization of CH50, C1 and C2 occurred during the treatment, whereas C3 and C4 remained lowered.
In the last five years, a distinct increase of allergic reactions to surfen, a constituent of various insulin preparations, was observed. The allergic reactions were tuberculoid granulomas of the delayed type (type IV of Gell and Coombs). Intracutaneous tests with surfen or surfen-containing insulin preparations confirmed the diagnosis. Dermal infiltrates and mild erythema developed 24 to 96 hours after the injection of insulin. In some patients a post-inflammatory pigmentation was noted. Histological examination of the lesions and the intracutaneous test sites revealed a granulomatous inflammation without signs of foreign body reaction. At present, the cause of the increased incidence of allergic reactions to surfen remains hypothetical.
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The influence of systemic application of chemically unrelated drugs and xenobiotics (phenobarbital (PB), rifampicin (Rifa), pregnenolone-16 alpha-carbonitrile (PCN) and 3-methylcholanthrene (MC) on drug metabolizing enzymes was investigated in the skin of rats of both sexes. The results were compared with those obtained in the liver. PB, Rifa and PCN induced the aryl hydrocarbon hydroxylase (AHH) in the skin though to a lesser extent than MC. The basal enzymic activity as well as the inducibility were considerably lower in the skin than in the liver. The activity of cytochrome c reductase was similar in all rats independently from the pretreatment. Only slight sex differences were noted: generally, the activity was higher in males. Cytochrome P-450 content of the skin could not be detected as well as various dealkylation activities.
Both single intravenous and repeated intracutaneous injections of bacillus Calmette-Guérin (BCG) resulted in alteration of the hepatic microsomal drug-metabolizing enzymes of the rat liver. The cytochrome P-450 content was not significantly altered; its activity (ethoxycoumarin O-dealkylation) was inhibited in both the intravenous and intracutaneous group. The arylhydrocarbon-hydroxylase and aminopyrine-demethylase activities were also diminished; decrease of cytochrome-c-reductase activity was noted after intravenous application only. Comparison of the results for the intravenous and intracutaneous application routes respectively showed qualitative and quantitative differences. The inhibitory effects of BCG treatment on the drug-metabolizing enzymes of the rat liver can only partly be explained by changes in the cytochrome P-450 system. These findings might lead to reconsideration of dosage of drugs in cancer (malignant melanoma) patients treated by combined chemoimmunotherapy.
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The occurrence of two primary malignant melanomas (superficial spreading melanoma, superficial spreading melanoma with nodular growth) in a patient with diffuse plasmacytoma treated with chemotherapy and radiation is described. Such an association does not appear to have been reported previously.
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The clinical evolution of Lyell' syndrome (LS), its complications, the histological findings, the nosological position and the differentiation from erythema exsudativum multiforme are dealt with. There are at least two etiological forms of LS: the LS caused by staphylococci, and the LS caused by drugs. The former mainly occurs in children, and is caused by staphylococci of the phagous group II, while the latter is mainly caused by sulfonamides, pyrazolones, penicillines, barbiturates and salicylates. The causative responsibility of a certain drug can be proved by three criteria: 1. Relapse of LS after exposure to the same drug. 2. Allergy to the drug taken before the onset of LS. 3. Positive allergy tests. The hypothesis concerning the pathogenesis of LS caused by drugs, the differential diagnosis and therapeutical guidelines are dealt with.
The Lyell-syndrome and the importance to ophthalmology is demonstrated. The nosological position - as staphylococcal disease (almost only in childhood) and as allergic extreme variant of bullous mucocutaneous disease is described. The differential diagnosis of similar (identical?) syndromes is discussed: Fuchs-syndrome, Baader-syndrome, Fiessinger-Rendu-syndrome. About 15 personal patients and 40 in the literature with LS with participation of the eyes is reported. The clinical results, development of the disease and the histological pictures of the cornea are made for the first time discussed. The therapy by lamellar or penetrating keratoplasty is reported.
We describe a 56-year-old Caucasian man with history of multiple regressed basal cell carcinomas. During the last 20 years approximately 200 histologically proven basal cell carcinomas preferentially localized on the face were surgically treated. Several large skin grafts were necessary to cover the extensive tissue defects on the face and scalp. Although all excised tissues were histologically proven to be basal cell carcinomas with tumor-free margins, new tumors developed in proximity to the skin graft margins. The dissemination of the new tumors made it difficult to perform additional invasive operation procedures without influencing the cosmetic result. Thus, we used photodynamic diagnosis to improve detection and demarcation of the neoplastic tissues. This procedure facilitated surgical planning and enabled primary in toto excisions. Surgical trauma and a number of interventions were thus minimized with the consequence of improved cosmetic and functional results.
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Cellular responses to platelet-derived growth factor, which affects all phases of the wound healing process, are dependent on the interaction of the growth factor with its cell surface receptors. Recently, we have shown that the platelet-derived growth factor-receptor was not expressed in uninjured human skin. In acute human wounds healing by secondary intention, both platelet-derived growth factor-receptor subunits were coordinately expressed, whereas no expression was found after reepithelialization at day 47. Even though impaired wound healing may be due to uncoordinated expression or the failure to express platelet-derived growth factor-receptor subunits, little is known regarding their expression in chronic ulcers. We studied the localization of platelet-derived growth factor-receptor expression in chronic venous leg ulcers of 15 patients with a median age of 73 years. Cryostat sections of biopsy specimens were immunostained with the use of antibodies against the alpha- and the beta-platelet-derived growth factor subunits. RNA was extracted from biopsy specimens and subjected to Northern blot analysis with the use of oligolabeled complementary DNA for the platelet-derived growth factor-receptor. Platelet-derived growth factor-receptor alpha- and beta-subunit expression was found in fibroblast-like cells within the wound bed and in cells beneath the epidermis of the wound edge. Platelet-derived growth factor-receptor beta-subunit expression was detected in endothelial cells of the vessels, in the granulation tissue, and the wound edge, whereas platelet-derived growth factor-receptor alpha-subunit was not expressed in endothelial cells of the uninjured skin. This finding suggests that the platelet-derived growth factor alpha-subunit may be involved in vessel formation during tissue repair. Both platelet-derived growth factor-receptor subunits were expressed at the messenger RNA level indicating that the synthesis is at least partly regulated at a pretranslational level. As the cellular responsiveness to growth factors depends on their specific receptors, our finding that both platelet-derived growth factor-receptor subunits are expressed in chronic venous ulcers substantiates the concept of therapeutic trials with recombinant platelet-derived growth factor.