Biomedical subjects
Publications and source records attributed to T Estrach.
Two adult males with cutaneous granular cell myoblastoma, localized on the leg and pubic area, are described. Atypical localizations of Abrikossoff's tumor are reviewed pointing out that the upper trunk is the most frequent of these localizations. Histopathogenesis of that tumour is also considered.
Cases of two 48 and 55 year-old males affected of facial granuloma with eosinophilia are presented. In both cases direct immunofluorescence studies revealed the presence of IgG, IgA and C3 along the basement membrane. A brief comments on clinical and histological appearance, previous immunofluorescence studies, pathogenesis and treatment are made.
A 77 year old man, who presented cutaneous T cell lymphoma, was treated with polychemotherapy and cyclosporine. After one year, erythemato-papulonodules were seen on the face, neck and trunk. Histologic examination showed specific infiltration myelomonocytic, bone marrow examination revealed the same characteristics. The patient died one month after the diagnosis. We comment the association between these two hemoproliferative processus.
A case of a primary cutaneous lymphoplasmacytic lymphoma synthesizing IgG lambda chains is reported. Immunohistochemical studies were done with the use of antihuman immunoglobulin antisera and monoclonal antibodies against T cell antigens. These studies reveal an unusual pattern of immunoglobulin production (IgG lambda chains) and an important population of accessory cells (T cells and Langerhans cells).
Explore the source record for details and available documents.
Explore the source record for details and available documents.
A case of myelofibrosis with papulonodular cutaneous lesions in a 49 year old man is reported. The histopathological studies showed the presence of the three hematopoietic lines in the skin lesions, which is a uncommon feature.
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.
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.
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.
Explore the source record for details and available documents.
In Hodgkin's disease (HD) specific cutaneous involvement is rare. When it occurs, retrograde lymphatic spread, direct extension from the underlying lymph nodes, and hematogenous dissemination are the mechanisms usually implicated. Among 349 patients with HD diagnosed and followed-up at our institution from 1969 to 1990, three (0.85 per cent) presented specific cutaneous involvement. In two cases with mixed cellularity subtype, skin lesions displayed histologic features similar to those found in the lymph node. Treatment with combination chemotherapy resulted in rapid disappearance of the lesions in two patients but was ineffective in the third. Previous reports of cutaneous involvement in HD, its differential diagnosis, prognostic significance, and treatment are briefly reviewed.
Trichothiodystrophy (TTD) is a hair abnormality that may be associated with a large number of alterations affecting the skin phenotype and skin appendages, nervous system, eyes, bones, and immune, gonadal, and endocrine systems. We report the first case of TTD associated with a urologic malformation and primary hypercalciuria. Our patient had congenital ichthyosis, physical and mental retardation, and a dysmorphic facies associated with a complex urologic malformation and primary hypercalciuria. Characteristic features of his hair under microscopic examination (trichoschisis, alternating light and dark bands under polarizing microscopy, and a severely defective cuticle) and low sulfur content by radiographic microanalysis confirmed the diagnosis. We discuss the meaning of this new association in the spectrum of abnormalities related to TTD.
The occurrence of Hodgkin's disease (HD) and non-Hodgkin's lymphoma (NHL) in the same patient is well known. The most frequent observation has been the development of large B cell lymphoma in patients affected with the nodular form of lymphocytic predominant HD. A less common situation is the development of NHL among patients successfully treated for HD. In such patients the second lymphoma has been thought to be related to the previous therapy or the immunodeficiency state that can accompany HD. Histologically, these NHL lymphomas often are intermediate to high grade and frequently extranodal. We report two patients successfully treated for HD who also developed NHL of the skin. Both patients presented with strikingly similar findings regarding to sex, age and subtype of HD. Clinical, histopathological and immunophenotypical findings were consistent with cutaneous low-grade B cell lymphoma of the marginal zone type. Both cases remain in complete remission of HD after standard therapy. In both patients the cutaneous lymphoma followed an indolent clinical course after a long follow-up period. This observation expands the spectrum of alterations possibly related to HD.