Biomedical subjects
Publications and source records attributed to J Ferrando.
Three cases of angiolymphoid hyperplasia have been treated with retinoic acid (Ro-10.9359). These three cases with a characteristic histological picture had been resistant to other previous treatment. The lesions improved markedly during the first month of treatment and finally 50 to 75% reduction of tumor sizes were obtained.
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Polypoid pseudosarcoma (PP) is a rare type of squamous-cell carcinoma of mouth, fauces and larynx with sarcoma-like reaction. Clinically PP is characterized by large pedunculated or sessile masses of rapid growing. This tumour recurs easily but rarely provokes metastasis. A case of polypoid pseudosarcoma of the tongue is reported. Differential diagnosis with spindle-cell carcinoma can be made following the criteria: a) the frequency and the sarcomatous appearance of the metastasis in the spindle-cell carcinoma, while PP rarely provokes metastasis which have a squamous-cell pattern; b) the gradual transition within the epithelial and the sarcomatous aspects in the spindle-cell carcinoma, not present in PP; and c) the presence of tonofilaments and desmosomes in the sarcomatous cells of the spindle-cell carcinoma, which lack in the sarcoma-like masses of PP.
Five cases of Woolly hair, three generalised, and two localised are reported. On the first two, we carried out trichogrammes which were studied with polarized light, and on all five, SEM (Scanning Electron Microscopy). The data obtained coincided with previous descriptions (Pseudomonilethrix images, Pili torti, Trichorrhexis nodosa, reduced hair diameter, oval section following transversal cut, and a wavy-line disposition), although we emphasize the smaller number of layers of cuticle cells following the transversal cut, and the presence of Trichonodosis, not previously refered.
Patients affected by Waldenström's macroglobulinaemia may rarely present specific cutaneous manifestations. The violaceous plaques or tumours infiltrated by lymphoplasmocytoid cells, and the pink, translucent, shiny papules composed of deposits of hyaline monoclonal IgM possess definite clinico-pathological characteristics that may permit the diagnosis before any other data were available. The immunopathological and ultrastructural features of these lesions are described.
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Two cases of cutaneous self-healing histiocytosis X in a 6 and a 16 months-old children, are reported. Clinically, the lesions were characterized by few, small, translucent and confluent papules, sometimes purpuric. The scalp lesions were seborrheic eczema-like; the elements on the groins were erosive. Systemic examinations, laboratory data and general development were completely normal. The histological pattern, in both cases, were identical of that in histiocytosis X (atypic mononuclear cells with glassy eosinophilic cytoplasm and excentric kidney-shaped nucleus). Electron microscopy of the second case showed a proliferation of histiocytes containing Langerhans bodies. Ten per cent of these cells showed multivesicular, myelin and dense laminar bodies. The latest had been specially described in other selfhealing histiocytic syndromes: congenital reticulohistiocytosis (Hashimoto-Pritzker) and generalized eruptive histiocytoma. Since the clinical behaviour and prognosis of the histiocytic proliferations can not be assumed on clinical and histological data, an attempt, of classification and understanding of these processes is presented. The possibility that histiocytosis represent a proliferation of the mononuclear-phagocytic system at different levels of cellular maturation, is considered. So it would be possible to consider: 1) Acute malignant processes: xantholeukaemia, malignant histiocytosis syndromes (histiocytic medullary reticulosis . . .), and Letterer-Siwe disease; 2) Chronic and severe processus: xanthoma disseminatum, necrobiotic xanthogranuloma, disseminated plane xanthoma, multicentric reticulohistiocytosis, localized reticulohistiocytoma of Crosti and Hand-Schüller-Christian disease; and 3) Benign processus, some of them being involutive: juvenile xanthogranuloma, regressing atypical histiocytosis, eosinophilic granuloma of the bone, Illig-Fanconi disease, Hashimoto-Pritzker disease, benign cephalic histiocytosis and generalized eruptive histiocytoma. These groups include classic X-histiocytosis, self-healing X-histiocytosis, non-X malignant histiocytosis and non-X benign or auto involutive-histiocytosis (some with intracellular lipid storage: xanthohistiocytosis) (see table II). The ultrastructural cytoplasmic markers of histiocytosis (table I) are consistent features in the accuracy of diagnosis of these conditions.
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.
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.
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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.
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.
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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".
"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.
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.