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Biomedical subjects

J Wechsler

Publications and source records attributed to J Wechsler.

At least 217 records · Page 12Linked to original sources
PubMed1987

Toxic epidermal necrolysis. Clinical findings and prognosis factors in 87 patients.

Eighty-seven patients with toxic epidermal necrolysis were observed at Hôpital Henri Mondor in Créteil, France, over the last 12 years. The mean percentage of body surface area involved was 39%. Erosive mucous membrane lesions, identical to those of Stevens-Johnson syndrome, were present in all but three cases. Necrolysis was sometimes generalized within 24 hours but usually spread progressively after a Stevens-Johnson syndrome-like aspect at the onset. Mortality was 25%. Infection, mainly with Staphylococus aureus and Pseudomonas aeruginosa, was the first cause of death, clearly responsible in ten of 20 cases. Age, extension of necrolysis, idiopathic nature of toxic epidermal necrolysis, ingestion of many drugs, elevation of urea, creatinine, and glucose levels, neutropenia, lymphopenia, and thrombocytopenia were statistically linked to a bad prognosis. A multivariant analysis showed that three of these prognosis factors are of paramount importance, namely: age, area of necrolysis, and serum urea level. Pigmentary changes and sicca syndrome were frequently observed sequelae in survivors.

Adolescent↗
PubMed1987

High-grade malignant cutaneous plasmacytoma metastatic to the central nervous system. A case report with electron microscopy, immunohistological, and neuropathological studies.

A case of high-grade malignant extramedullary plasmacytoma (EMP), unusual in its initial cutaneous localization and major involvement in the central nervous system rapidly resulting in death, is reported. Light- and electron-microscopic studies of a skin nodule revealed a high proportion of immature and mature plasmacytes. Immunohistological study revealed that both cells synthesized different amounts of a monotypic cytoplasmic kappa light chain. Neuropathological study revealed extensive infiltration of the leptomeninges and perivascular spaces as far as the deep parenchyma. In several places, effraction of vessels resulted in parenchymatous, tumor-like, plasmacytic infiltration. The findings of this case were compared with those of the few previously reported cases of EMP with initial cutaneous or intracranial localization, and with those of immunoblastic sarcoma occurring in some cases of myeloma.

Brain Neoplasms↗
PubMed1987

[Diffuse disease of elastic tissue and hyperphosphoremia].

Pseudoxanthoma elasticum (PXE) is exceptionally associated with abnormal phosphate-calcium metabolism. On the other hand, significant hyperphosphataemia is present in tumoral calcinosis, with an occasional elastopathy resembling that of PXE. We report here the first case of diffuse elastic tissue disease associated with hyperphosphataemia in the absence of tumoral calcinosis. The patient, a 29-year old Gabonese, had severe arteritis of the lower limbs with linear calcifications of the aorta and major arteries of the limbs. Angioid streaks and calcifications were present in the retinas. The skin appeared to be normal, but histological examination of specimens from flexures showed abnormalities of the elastic tissue with many fibres being broken into small pieces and curled up. In a morphometric study of cutaneous elastic tissue these abnormalities were compared with those observed in a PXE patient and with normal elastic tissue. The abnormalities in our patient and in the PXE patient were similar. Compared to normal elastic tissue, there was a slight decrease in the total number of oxytalan and elaunin fibres. The elastic fibres were distinctly wider, longer and separated from each other by smaller spaces, but not more numerous than normally. The histopathological appearance of an artery removed during bypass surgery was similar to that of a PXE artery. The hyperphosphataemia was due to an increase in tubular absorption of phosphates without abnormalities of parathyroid hormone, or resistance to its phosphaturic effect or any other tubular disorder. It was little altered by aluminium carbonate, and it partially decreased after addition of calcitonin.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1987

[Lymph node histopathologic aspects in cutaneous lymphomas of the mycosis fungoides or Sézary syndrome type. Retrospective study of 98 biopsies].

The results of a retrospective study of 98 lymph nodes in 89 patients with documented mycosis fungoides (M.F.) are reported. According to the histopathological aspect, three groups are determined: group I of 20 lymph nodes with lesions of dermopathic lymphadenopathy; group II of 27 lymph nodes, presenting variable-sized clusters of atypical cerebriform lymphocytes located in paracortex without homogenization; group III of 51 lymph nodes with partial or total homogenization; according to the different cytologic types, four histopathologic forms are described in group III: small, large, mixed and immunoblastic cellularly type. The paracortical changes of dermopathic lymphadenopathy observed in group I cannot be distinguished from those in patients with unrelated diseases. All histopathological aspects of group III correspond to a specific MF involvement. The most difficulties are caused by lymph nodes from group II; in order to detect an early MF involvement, the histopathologist has to find a partial infiltration of paracortex by atypical large and numerous lymphocytes. Morphometric and immunohistochemical methods previously reported are useful but they are expensive and they cannot be usually carried out. Because of the heterogeneity of the lesions a perfect routine paraffin-embedded with serial sections seems to be the most helpful way for recognition of early MF involvement in lymph nodes. In our series, as in literature, correlations with clinical course show that lymph node involvement classified in group III is pejorative. But it seems that histo-prognostic evaluation based on a suspicion of early involvement has to be carefully established because of the lack of significant difference of clinical behaviour between group I and group II.

Humans↗
PubMed1986

[Erdheim-Chester disease. The multiviceral form presenting as exophthalmos].

Erdheim-Chester disease is a rare visceral xantho-granulomatosis, the 17th case of which is reported here. The initial symptom, bilateral exophthalmos, was uncommon. The picture was completed by a retroperitoneal xanthogranuloma and by bilateral and symmetrical osteosclerosis of the long bones. The lack of X-bodies at electron microscopy differentiated the disease from Hand-Schüller-Christian disease. The patient's condition improved with chemotherapy (vinblastine and doxorubicin) combined with corticosteroid therapy. After a 5-month remission period, he died of an intercurrent infection. No autopsy was performed.

Diagnosis, Differential↗
PubMed1985

Sjögren-like syndrome after drug-induced toxic epidermal necrolysis.

7 of 9 patients recovering from a drug-induced toxic epidermal necrolysis (TEN) which they had had 2 months to 4 years before had xerostomia, or keratoconjunctivitis sicca, or both. 5 patients had symptoms; 2 had severe visual impairment. In 5 cases there was lymphocytic infiltration of small salivary glands; in 2 patients this was identical to that of Sjögren syndrome. None of the patients had antinuclear antibody. TEN may occur during acute graft-versus-host disease (GVHD) and these observations are reminiscent of the sicca syndromes reported during chronic GVHD. They suggest that autoimmune mechanisms may be involved in drug-induced TEN and that Sjögren syndrome may occur as the result of a drug reaction.

Adult↗
PubMed1985

[Angiolymphoid hyperplasia with eosinophilia. Optical and histo-immunological study of 5 cases with an electron microscopy study of one of the cases].

5 cases of angiolymphoid hyperplasia with eosinophilia of the skin are reported. This rare condition was initially described in 1969 by Wells and Whimster. The microscopical pattern is characterised by an atypical vascular proliferation associated with an eosinophilic and lymphoplasmocytic granuloma. In our series, immunohistological study demonstrated the polyclonality of the plasmacytoid component. Electron microscopy performed in one case shows endothelial features even in cells forming clusters without well-defined lumen. These results support the endothelial origin of angiolymphoid hyperplasia with eosinophilia.

Adult↗
PubMed1985

[Diagnostic procedure in chronic cervicofacial adenopathies].

Results of clinical examination are fundamental for establishment of an etiologic diagnosis, but the additional data provided by paraclinical investigations are essential elements. Adenectomy should be performed when etiology is uncertain.

Chancre↗
PubMed1985

[Ocular sequelae and sicca syndrome following Lyell's syndrome].

The mucous lesions are customary in toxic epidermal necrolysis. The acute ocular lesions have led to definitive sequelae. 22 out of 32 patients surviving to a Lyell's syndrome lend themselves to an ophthalmological supervision. 13 of these 22 patients (59 p. 100) presented a sicca syndrome with a decrease of the lacrimal secretion (12 cases) and/or salivary secretion (8 cases). The sicca syndrome appeared sometimes from the beginning of the acute phase of the Lyell's syndrome or more often a few weeks later. This decrease of the lacrimal flux went along with objective corneal lesions among 11 patients, and 6 of them had a change of the visual function. A biopsy of the accessory salivary glands was performed on 7 of these patients having a reduction of the salivary flux. 5 of these 7 biopsies showed lymphocytic infiltrates giving in 2 cases a nodular aspect, grade III of Chisholm's classification, held to be pathognomonic of Sjögren syndrome. The 13 patients having presented a sicca syndrome differed from the other 9 patients (without sicca syndrome) by a higher severity of their Lyell's syndrome and by a higher age. None of our patients with dry syndrome had antinuclear antibody detected by immunofluorescence on sections of rat's livers. Sicca syndromes with sometimes lymphocytic infiltrate similar to those of Sjögren's syndrome were occasionally imputed to drug reactions. The occurrence of "Sjögren like's syndrome" after a toxic epidermal necrolysis suggests the intervening of autoimmune phenomena in the pathogenesis of this toxic epidermal necrolysis.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗