Biomedical subjects
Publications and source records attributed to S Dalac.
We report a case of familial porphyria cutanea tarda (FPCT) in a 7-year-old girl. The condition was exacerbated by low dose of hydroxychloroquin, but controlled by venesection. The favorable prognosis of FPCT should be further established by long-term prospective studies.
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Haematoporphyrin is a synthetic porphyrin the photosensitizing properties of which are used for the detection and treatment of some neoplasias. Three cases of phototoxicity from Hemedonine, an "anti-asthenia" drug composed of pure haematoporphyrin, are reported. Following exposure to the sun, painful urticarial plaques developed at the sites of intramuscular injections, and spread to the lumbar and abdominal regions, leaving a brownish pigmentation which persisted for years. Photobiological exploration was performed in one of these patients, demonstrating photosensitivity to Hemedonine in vivo and showing that long UVA rays were essentially responsible for the lesions. Histological examination elicited the initial vascular changes of a phototoxic reaction and suggested a new mechanism of photosensitization: "photo-induced immune vasculitis". The phototoxic effects of haematoporphyrin are primarily due to the destruction of intracellular organelles consecutive to oxidative denaturation of membrane systems. The initial phase of the phototoxic reaction might consist of complement activation and degranulation of dermal mast cells.
The authors present a case suggesting either bullous systemic lupus erythematosus of dermatitis herpetiformis. An immunoelectron microscopic study located the immunoglobulin deposits beneath the cutaneous basement membrane zone. The nosological problem of possible relationship between systemic lupus erythematosus and epidermolysis bullosa acquisita is discussed.
Between 1975 and 1983, 46 patients with mycosis fungoides (MF) were treated with radiotherapy. The patients were classified according to the Stanford system (table II) and to the TNMB system (table III). The superficial lesions were treated with total skin electron-therapy (TSE) (7), and the visceral and lymph node lesions received additional whole-body photon therapy (WBP) (7). The results are summarized in table I. Initial complete regression was obtained in 21 of the 46 patients; 15 had partial regression. At the moment, 11 are alive without any apparent lesion. There were 9 complete remissions in each of the stage I and stage II groups. In stages III and IVa groups the course of the disease was improved by the two types of irradiation combined. In stage IVb patients the leukaemia was not improved. The survival curves (fig. 1 to 6) offer a simplified representation of the factors responsible for a favourable course.
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The authors report the case of a 54-year old woman presenting with right cardiac failure in whom the diagnosis of contemporaneous cutaneous-systemic mast cell disease was made. The cardiac symptoms regressed concomitantly with the vasomotor manifestations of the mast cell disease. Following a review of the principal mast cell mediators, the cardiovascular manifestations of the disease are studied and pathogenetic hypotheses are being put forward.
Extramedullary plasmocytomas are ubiquitous plasmocytic tumours which are principally located in bones and mucosae but rarely found on the skin. Clinically, they present as purplish-blue cutaneous nodules or, less frequently, as papular or urticarial eruptions. The diagnosis rests on histology and immunostaining. Cutaneous plasmocytomas are usually divided into two types: (1) primary plamocytomas which occur in the absence of myeloma, present as solitary or multiple skin tumours, accompanied or not by monoclonal gammopathy, and have an imperfectly known course and prognosis; (2) secondary plasmocytomas--a case of which is reported here--which appear in the course of a large tumoral mass myeloma and have a very poor prognosis.
CONTEXT: Since 1992, sentinel lymph node (SLN) biopsy was generally applied to melanoma for tumor staging. As the literature points out, an increasing number of nodes are being removed for each procedure, driving up the cost for this procedure and wandering away from the defining concept of sentinel lymph node. AIMS: The objective of the current study was to show that the number of sentinel lymph node s removed can be minimized without influencing the reliability of tumor staging. MATERIALS AND METHODS: We conducted a single-arm prospective study in patients with stage I melanoma. For each patient, the sentinel lymph node was identified using the hand-held gamma probe technique. We removed only the hottest nodes as well as the nodes with radioactivity greater than 70% compared to the hottest. We analyzed the characteristics of each melanoma, the success rate of this procedure, how many nodes were removed and how many had micro metastases. STATISTICAL ANALYSIS: The results were compared to those of the literature, previously published Porter study using the chi-square test. RESULTS: We included 90 patients. The success rate of this technique was 100%. We dissected 1.3 sentinel lymph nodes for each patient, with 22% positive SLN. Statistical analyses point out a better selectivity of our study for a similar rate of pathological positivity and recurrence compared to the literature. CONCLUSIONS: Our technique for decreasing the number of sentinel lymph nodes removed is reliable. The removal of minimal number of nodes doesn't compromise the sensitivity of tumor staging, while it does reduce the cost of the procedure.
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BACKGROUND: Cross-sensitivity between angiotensin-converting enzyme inhibitor-induced angioedema and cough, and angiotensin II receptor antagonist has been reported in the literature. Eczema-like skin reactions have never been documented. We report the first two cases. CASE REPORTS: Two patients, aged 79 and 88 years, with a history of hypertension, were treated with angiotensin-converting enzyme inhibitors, which had been discontinued because of an eczematiform rash. In spite of substitution with an angiotensin II receptor antagonist, the patients had developed the same eruption. The outcome was favourable after discontinuation of the angiotensin II receptor antagonist. The pharmacologic study suggested the possibility of a cross-sensitivity reaction between these two drugs. CONCLUSION: We report the first two cases of a cross-sensitivity between angiotensin-converting enzyme inhibitors and angiotensin II receptor antagonist presenting as an eczematiform rash. The exact mechanism is unknown, but clinicians must be aware that angiotensin II receptor antagonist is not a safe alternative in patients who have a history of eczematiform rash secondary to angiotensin-converting enzyme inhibitors, as has been always reported with angioedema.
Cryosurgery is a controlled refrigeration technique used to destroy some tumoral tissues that are more sensitive to congelation than healthy tissues. The authors present its principle and describe the apparatus: a source of liquid nitrogen, a flexible tube and a probe with a skin-suitable nozzle. The main advantages of the method are its reliability and reproductibility. Among the numerous indications of cryosurgery are all benign tumours of the skin, especially when they are multiple. Concerning malignant lesions, the most usually treated are the multiple forms of basal cell carcinoma, notably the superficial type. Although cryosurgery acts blindly, its indications are increasingly better defined.
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