Biomedical subjects
Publications and source records attributed to J Ferrando.
Three cases of solitary Juvenile Xanthogranulome of the skin are reported, one of them with tumoral appearance. Histologycal pictures were characteristic, except for the tumoral form. Other few cases of this atypical form of Juvenile Xanthogranuloma are reported in the literature.
Conley et al., in 1971, described a special type of melanoma characterized by a superficial melanic lesion at the onset; repeated local relapses as subcutaneous tumorations with an histological picture closely resembling an atypical fibroxantoma or fibrosarcoma. After a review of all the published material the autors presents a personal case with the clinical, histological and evolutive characteristics of this disease. The most interesting findings of the published case are the following: The special stains for the melanocytes (silver stain, Dopa, tyrosinase and cholinesterase) were all negative. There was an intense positivity for the lisosomal enzymes (non specific sterases, and acid phosphatases). The ultrastructural study of the tumoral tissues as well as the cells of cultures showed abundant cells with tumoral aspects, with prominent nucleoli somewhat dilated granular endoplasmic reticulum, myelin-like figures, lipidic vacuoles and abundant lisosomes. No melanosomes or premelanosomes were observed. Beside these tumoral cells abundant typical fibroblastic elements were found. There was a great amount of collagen fibers with periodicity superior to the normal. The conclusion is that the desmoplastic melanoma must be considered as a tumor of mesenchimatous origin intervening in its development multiple local and general factors.
Twenty one specimens of cutaneous xanthomas from different types of hyperlipoproteinaemic and normolipidemic xanthomatosis were obtained and analyzed by thin layer chromatography. After separation and development, the areas were determinated by densitometry, and the results are given in percentages for each development. Acording to the results, the following data are of interest: -- Recents xanthomas have greater amount of lipids. -- Cholesterol esters (EC) are prevalent in xanthomas and in normal skin the triglycerides fraction. -- Monoenoic esters (EM) are prevalent on EC chromatography, like in normal skin. -- Finally, there are not significant variations between lipid composition of xanthomas in the different Xanthomatosis.
Two cases of new hair defect syndrome, recently described by French dermatologists, are reported. They are characterized by clinically dry, bright and uncombable hair. Pathologic and scanning electron microscope studies, show: -- Angulation of the hair bulb with adherence between the inner root sheath and the hair shaft. -- Canalicular formations longitudinally disposed on the cuticular surface with irregular stratification of the external sheath. A malformation of the hair bulb of unknown aetiology is postulated. This malformation, probably familial, is not associated with any other abnormalities. This defect would result in hair shaft surface abnormality.
In a case of histiocytic medullary reticulosis with protracted course and necrotic skin lesions, histological study revealed infiltration of the fat tissue by atypical reticulohistiocytic cells, areas of associated fat necrosis and vascular lesions. Autophagocytosis (phagocytosis of erythrocytes, erythroblasts, platelets, lymphoid cells, and nuclear debris) was found in biopsy specimens of the subcutaneous infiltrates, lymph nodes, and bone marrow, and in necropsy specimens of skin, iliac mesenteric lymph nodes, bone marrow, liver, spleen, and suprarenal glands. Electron microscopy of various viscera showed abnormal histiocytic cells containing multiple phagocytosed erythrocytes and other blood cells in different stages of destruction and digestion. Multi-membranous bodies also were present. Defective lysosomal degradation of intracytoplasmatic ceroids in this condition is postulated.
Thirty six specimens of normal human skin were obtained from persons of both seces, diferent ages and localizations. Each lipid extract (Ex L) was analyzed by thin layer chromatography. After separation and development, the areas were determined by densitometry. The results are given in percentages for each development. Statistical studies were carried out for each variant (sex, age, and localization) and for each lipid fraction. According to results, the following data are of interest: 1) Underlined results on table V, are significative. 2) The variant: sex, does not change the results. 3) Squalene (Esc) is absent on plasma. 4) Cholesterol esters (EC) are age and localization dependant. 5) The total amount resulting from the addition of monoenoics (EM) and dienoics (ED) esters, is constant (approximatively the 75 % of EC). 6) EM are prevalent in skin, and the ED in plasma. 7) Phospholipids (FL) are not constant. 8) The results depend particularly on the localization of each specimen. Thin layer chromatography is a good qualitative method, but the densitometry is not an exact method, as a 5-10 % of error is usually accepted. Our results gave only an idea of the lipid components of normal human skin, but this data can be applied as a tentative pattern por subsequent studies in pathological cases.
A case of diffuse normolipemic plane xanthome showing no association with systemic disease (multiple myeloma, leukemia, reticulosis, or dysglobulinaemia), in a 50 year old female, is reported. The patient presented hypersensitivity to the erythrogenic spectrum with pathological response and lowered MED. The B lymphocytes in the peripherical blood was raised. The authors postulated that B lymphocytes probably play an important role in the pathogenesis of the skin light-induced reactions.
Explore the source record for details and available documents.
A case of porphyria with symptomatology beginning in early infancy with peculiar biochemical features is described. There were crops of blisters, an extraordinary hypertrichosis and scarring of the exposed skin. The porphyrin examination showed an abnormal excretion pattern of urinary porphyrins with large amounts of 5-COOH carboxyle porphyrins and the protoporphyrin level of the red cells was raised. There was fluorescence of the erythrocytes and erythroblasts. The porphyrin excretion pattern of the stools was similar to those of the PCT. This case is closely similar to another published by the authors in 1969 and to 2 published by Schneider and Simon. The authors consider that could represent a new type of porphyria.
A case of atypical fibroxanthoma of the skin is related. Ulstrastructural findings include: fibroblast-like cells, histiocytic-like cells, xanthomatous cells, and multinucleated cells. Strikingly similar findings were found by Fu and cols. (1975) in four cases of malignant fibrous histiocytoma. The authors postulated that both tumours could represent different aspects of a single proliferating process.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.