[Staphylococcal Lyell's syndrome in varicella. Apropos of a case].
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Biomedical subjects
Publications and source records attributed to Y de Prost.
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Forty-two patients with alopecia areata were treated with local applications of dinitrochlorobenzene (DNCB); We used DNCB in two forms, an acetone solution applied weekly or a cream used every day, employing a wide range of DNCB concentrations. The concentration used was varied at the time of each application to produce a contact dermatitis. Seven patients experienced complete and lasting hair regrowth, 17 had poor results, and in 18 patients the treatment was a failure. Acquired tolerance to DNCB was observed in six patients; in five it was abolished by the administration of cimetidine. Certain factors such as the delay in appearance and the intensity of the sensitization reaction influence the hair regrowth. Poor prognostic criteria for treatment effect included a history of previous systemic corticosteroid therapy, atopy, and the presence of alopecia areata in close relatives.
Two cases of bullous dermatosis with dysglobulinemia are described. The first one was associated with renal and neuromuscular amyloidosis and production of a monoclonal lambda IgG. Optical and electron microscopy showed amyloid deposits beneath basal lamina of the dermis. Results for direct and indirect immunofluorescence (IF) were negative. This bullous dermatosis is not an epidermolysis bullosa acquisita (EBA), in the strict sense, because the amorphous material deposited is amyloid. In the second case, associated with Waldenström's disease, there was no cutaneous or systemic amyloidosis. Direct IF was positive; linear IgM deposits were seen along the basal membrane of the bulla and the healthy skin. Indirect IF showed the presence of circulating antibodies against basal membrane zone. This bullous dermatosis is probably an EBA, despite the absence of IgG deposits. The absence of electron microscopy does not permit the confirmation of this diagnosis.
Epidermolysis bullosa acquisita (EBA) is a rare non hereditary bullous disease. One case is described with a morphological, and immunocytochemical ultrastructural study. The plain ultrastructural morphology showed a dense amorphous deposit in the superficial dermis, under the basal lamina. Immunofluorescence showed IgG and C3 deposits. The ultrastructural study (direct immunoperoxidase technique using Fab) showed a dense labelling associating : dark dots spaced under the basal lamina and fogging less dense labelling in the clarified superficial dermis. Using the patient's antibodies an indirect reaction on normal human skin performed : the same dotted sub laminal labelling was found but with no dermal diffuse reaction product. The study shows the site and the aspect of the Ig deposit in EBA and indicates that the antibodies demonstrated in some patients are reactive with a constituent of the normal superficial dermis.
Skin lesions in systemic candidiasis are erythematous maculopapules and maculonodules which appear at the onset of the septicaemic phase. Their presence, especially when associated with diffuse myalgia, suggest the diagnosis which must be confirmed by haemocultures, histological examination of the skin with PAS stain and culture of skin fragments in Sabouraud's medium. Systemic candidiasis with skin lesions seems to be mainly due to Candida tropicalis and to occur with great frequency in patients with underlying blood disease. Of the three cases reported here, one concerned a 24-year-old man with premyelocytic acute leukaemia, the second a 45-year-old woman with drug-induced agranulocytosis and the third one a man aged 25 admitted to hospital for peritonitis secondary to Crohn's disease.
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A remarkable increase in the number of recognized clinical abnormalities of neutrophil function has occurred within the 8 years past. Of major importance in the delineation of these disorders is the establishment of appropriate methodology of their characterization. This review discusses phagocytosis and its disorders by dividing it into stages that encompass the way in which phagocytes ferret out injection, how they recognize pathogens and the intracellular events leading to engulfment and killing of micro-organisms. The pathology of chemotaxis and random mobility is described: the lazy leukocyte syndrome, the Chediak-Higashi syndrome, the familial chemotaxis pecular dermatosis, and a variety of serum abnormalities of the serum complement system associated with deficient generation of chemotactic activity. The second part of these disorders is dailing with the inherited abnormalities of the oxygen dependent killing mechanisms of phagocytes, the chronic granulomatous disease, the glucose-6-phosphate deshydrogenase deficiency, the myeloperoxydase deficiency and the glutathione peroxydase deficiency.
Detailed investigations of 9 patients with dermatitis herpetiformis are presented. In all cases cutaneous lesions were controlled by dapsone alone or by dapsone and gluten free diet. Granular IgA deposits were found in 7 patients, linear IgA deposits in one, and C3 component of complement in one. 3 patients out of 8 tested, carried the specific HLA-B8 antigen. Despite an extensive investigation, no malabsorption was detected. Jejunal biopsies were performed in 8 cases. Jejunal villous flattening was observed in one patient. It improved after a 2 months gluten free diet on subsequent jejunal biopsies. D. H. seems peculiar in France as compared with case reports from other countries: low prevalence of gluten sensitive enteropathy; rare occurrence of the specific HLA-B8 antigen; incidence of D. H. seems to be low in France. It is noticeable that french incidence of coeliac disease is low as well. This suggests a genetic difference in the investigated population (low prevalence of HLA-B8 antigen) and/or different alimentary habits, particularly a low dietary gluten amount.
The authors report five cases of streptococcal gangrene and stress the fundamental role of surgical excision. Parenteral anti-streptococcal antibiotics should not lead to this essential step being forgotten, its early application being the most important element in prognosis. It is thus essential to clearly understand the local and general criteria of rapid diagnosis in this condition, which is far from rare.
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The authors relate their experience in the study of chloremia, phosphatemia and their relationship in the diagnosis of parathormonal hypersecretion states, 31 cases of parathyroid adenoma are compared to 26 cases of hypercalcemia depending on other mechanisms. The studied relationship appears to be interesting in the diagnostic work-up of parathyroid adenoma as well as in appreciating the mechanism of hypercalcemia during the course of other neoplastic disorders.
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Cholesterol emboli have only recently been recognized as a pathological entity and their clinical picture is still not clearly defined. A case is reported of a patient presenting signs of a severe systemic affection with distal arteriolitis and also general and biological signs of a severe inflammatory process having the particular characteristic of apparent corticoid sensitivity. The patient died following ischemic necrosis of the sigmoid from perforation and peritonitis, also related to the presence of cholesterol emboli.
We have investigated 8 patients from 7 unrelated families with lamellar ichthyosis (LI) for defects in the keratinocyte transglutaminase (TGK) gene. We have characterized three novel homozygous mutations and a previously reported splice acceptor site mutation. One patient showed a C-to-T change in the binding site for the transcription factor Sp1 within the promoter region. Another patient had a Gly 143-to-Glu mutation in exon 3 and a third patient, affected with a particular form of LI sparing the four limbs, demonstrated a Val382-to-Met mutation within exon 7. These three patients exhibited drastically reduced transglutaminase activity and an absence of detectable TGK polypeptide, as assessed by immunofluorescence and immunoblotting. Northern blot analysis showed that the Sp1 site mutation was associated with profound reduction of TGK transcript levels whereas normal transcript levels were observed for the two missense mutations. We hypothesize that the Sp1 site mutation impairs transcription of the TGK gene, whereas the two missense mutations induce structural changes leading to protein instability. Linkage to TGK was excluded in another family and no evidence for TGK defect was found in 3 other patients. These results further support the involvement of TGK in some patients with LI. They identify a TGK mutation as a cause for non-generalized LI and further delineate the molecular mechanisms underlying TGK deficiency in LI.