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Biomedical subjects

J Ferrando

Publications and source records attributed to J Ferrando.

At least 109 records · Page 6Linked to original sources
PubMed1982

[Juvenile Duhring's disease. Apropos of 7 cases].

Chronic non hereditary subepidermal blistering diseases are in childhood and present mainly problems on its clinical and histological diagnosis. Immunopathologic, immunogenetic and ultrastructural studies play a definitive role in the classification of this group of entities. In this paper we report and comment seven children affected of dermatitis herpetiformis. The clinical diagnosis was assessed by direct immunofluorescence studies of the skin, which showed granular Ig A deposits at the MBZ. In five out of our seven patients we were able to demonstrate by clinic and laboratory malabsorption syndrome. A bibliographic review of the subject is carried out, stressing specially the differential aspects with other subepidermal blistering disease of the childhood (i. e. bullous pemphigoid and chronic benign blistering disease of the childhood) as well as some other acute and chronic infantile blistering entities.

Adolescent↗
PubMed1981

Histopathology of the saburral tongue.

Optical microscopical studies were carried out on biopsies from 22 patients affected by saburral tongue (ST). The histopathological pattern consisted of a papillomatous acanthosis covered with a very thick horny layer. Abundant colonies of banal germs were seen in the keratin. There was an alternation of suprapapillary orthokeratosis and interpapillary parakeratosis. Under the keratin layer all the keratinocytes were large and clear with a glycogen-filled cytoplasm. Two specimens, studied with a transmission electron microscope, only showed that the keratinocytes were filled with glycogen. The clear glycogen-filled epithelial cells are common either in normal lingual epithelium or in some pathological conditions. The microscopic field of ST is quite similar to that of the hairy black tongue; but these peculiar clinical aspects of the tongue do not evolve one through the other.

Adult↗
PubMed1981

[Neviform hyperkeratosis of the mammary areola].

A case of neviform hyperkeratosis of the areola is reported. This case may be identified as type III of the Lévy-Franckel classification: not associated to other cutaneous alterations. A review of the literature is made. The authors emphasize the importance of the hormonal factors in the development of these lesions.

Adult↗
PubMed1981

[Trichorhinophalangeal syndrome (Giedion)].

Three cases of the Tricho-rhyno-phalangeal syndrome (TRF) are described. Two cases were in the same family with some affected membres (Camacho et al., 1978). The third case, of sporadic presentation, showed radiological signs of Perthes' disease. All cases presented the main clinic and radiologic alterations of the TRF syndrome: hypotrichia, pear-shaped nose and cone shaped epiphyses. Some of them presented other characteristic features such as: darwism, prominent nose, elonged philtrum, thin upper lip, baldness of the external 1/2 eyebrows, prominent ears of low implantation, shortening of some fingers (of metacarpals and metatarsal), onicodystrophy, etc. The differential diagnosis must be stablished with: a) congenital processes which present facial abnormalities, specially of the nose, b) congenital atrichias o hypotrichias associated to osteoarticular troubles of the growth and c) congenital syndromes with epiphyses in cone (peripheric dysostosis). Finally, we find, by scanning electron microscope studies, an increase of the cuticular cells in detachment, an inespecific sign that denotes a special lability of these patients' hair to the action of the outside agents on it.

Adolescent↗
PubMed1980

[Postovulation dermatitis (dermatitis caused by progesterone)].

Postovulation Dermatitis is a frequent clinical picture although it is not well known. Clinically a polymorphous eruption appears between than 8 or 10 day before menses. The Authors report three cases in which an complete immunological investigation was performed. They conclude that there is no objective evidence of the autoimmune pathogeny of this picture in spite of the clinical relationship between the dermatitis and the ovulation. Therefore they suggest that diseases should be described as "Postovulation Dermatitis" instead "Autoimmune Progesterone Dermatitis".

Adult↗
PubMed1980

[Pili canaliculi (uncombable hair syndrome or spun glass hair syndrome). A scanning electron microscope study of ten new cases (author's transl)].

"Uncombable hair" is the denomination applied to a new abnormality of the hair, being characterized by its arranged in bundles disposed in different directions and which are irreductible on combing. It may have a familial trait and starts at the age of 3 months onwards. It appears in children of both sexes, whose hair becomes dry, tightly curled, shiny, lighter and impossible to comb. There are no accompanying abnormalities in the development. Neither have any biological changes been registered. The clinical and ultrastructural aspects of 10 cases of children affected with "uncombable hair" are presented. At the scanning electron microscope we have observed longitudinally disposed canalicular formations on the cuticular surface of 50 p. 100 of hair taken from each patient. The canal is, in some zones, more excavated than in others, occasionaly has an irregular outline and sometimes is uniformly located nearly along the whole length of the hair. The transversal section shows triangular aspects, kidney-shaped or completely irregular. We agree with Dupré to use the term pili canaliculi for the defect shown by these patients.

Adolescent↗
PubMed1979

Ultrastructural aspects of normolipidemic xanthomatosis.

Electron microscopic aspects in ten cases of normolipidemic cutaneous xanthomatosis have been investigated. Two additional types IV and V hyperlipoproteinaemic xanthomatosis have also been included. Ultrastructural findings in all cases were similar. Abundant histiocytic cells with numerous intracytoplasmic lipid vacuoles, lysosomes, and myelin-figures, were the striking features. Moreover, in older lesions microfilaments and lipid vacuoles were found in some fibroblastic cells, as well as long space collagen around them. In some specimens we observed: giant multinucleated histiocytic cells, crystalline cleft-like spaces in histiocytes and some mastocytes with lipidic crystals in the extracellular space, as well as lipid vacuoles in Schwann cells, endothelial cells and pericytes. Rod-shaped tubulated bodies were found in some endothelial cells, with multiple basal vascular laminae. In xantelasma palpebrarum and in disseminate plane xanthoma the histiocytary foamy cells adopted a perivascular arrangement, as in hyperlipoproteinemic xanthomatosis. We concluded that ultrastructural aspects of different xanthomatosis are fairly similar as a consequence of the large amount of intracytoplasmic lipids accumulated in xanthomatosus cells. In xanthelasma palpebrarum and in disseminated plane xanthoma this cell phase is reached by similar pathways to those for hyperlipoproteinemic xanthomatosis, whilst in xanthoma disseminatum and juvenile xanthogranuloma the pathways seem to be different. A classification of normolipidemic xanthomatosis is also provided.

Histiocytes↗
PubMed1977

[Solitary juvenile xanthogranuloma. Apropos of 3 cases].

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.

Child, Preschool↗
PubMed1977

[Desmoplastic melanoma].

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.

Aged↗
PubMed1977

[Lipid composition of cutaneous lesions in different types of xanthomatosis].

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.

Chromatography, Thin Layer↗
PubMed1977

[Uncombable hair syndrome].

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.

Child↗
PubMed1976

Necropsy and ultrastructural findings in histiocytic medullary reticulosis.

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.

Adrenal Glands↗
PubMed1976

[Lipid composition of the normal skin].

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.

Adolescent↗
PubMed1975

[Disseminated plane xanthoma with photosensitization].

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.

B-Lymphocytes↗
PubMed1975

[Hepato-erythrocytic porphyria. A new type of porphyria].

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.

Child↗