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Biomedical subjects

M Lessana-Leibowitch

Publications and source records attributed to M Lessana-Leibowitch.

54 records · Page 3Linked to original sources

[Menkes' disease (new skin and hair ultrastructural abnormalities) (author's transl)].

The authors report the sixth case of Menkes' kinky hair disease. This boy has been observed for as long as 16 months, and he his still alive at the time of publication. This genetic, X linked disorder of copper metabolism is always fatal in childhood. Diagnosis is evoked when is noted the conjunction of progressive cerebral degeneration, seizures, with pili torti and monilethrix. It can be asserted with the very low copper and cerulo-plasmin blood levels. Recognition of the disease in utero might be possible. New findings in skin' electron microscopy and hair' scanning electron microscopy are reported here. And two RX scanner of the brain have been performed.

Brain Diseases, Metabolic↗

[Cutaneous manifestations of angio-immunoblastic lymphadenopathy].

The authors report four cases of angio-immunoblastic lymphadenopathy, presenting with cutaneous lesions: these have a tumoral presentation in two cases while in the other two, the polymorphic aspect of the cutaneous lesions is reminiscent of a toxidermic eruption. In contrast to the relative clinical polymorphism, the pathological findings are monomorphic, i.e., in all cases, there was a vascular proliferation together with a more or less dense cellular infiltrate consisting of cells indistinguishable from those involving the abnormal lymph nodes.

Female↗

[Lymphomatoid granulomatosis: cyclic nodular lymphomatoid panniculitis with immunologic deficiency].

The case of a 66 years old woman having presented an unusual cyclic dermatosis of 11 months duration is reported. The cutaneous elements were successively located in a subcutaneous, dermal and epidermal situation, and presented a spontaneous healing with sometimes deep retractive residual scars after an ulceration or not. Pathologically, the lesions consisted in dense mononuclear infiltrates and vascular lesions. This disease was associated with an immuno-deficiency state characterized by extremely low levels of circulating IgM. This deficit was found to be persistant as it was still present two years later. However during this lapse of observation no cutaneous lesions recurred. The possible connexions of such a case with those of lymphomatous granulomatosis are discussed. In the absence of pulmonary lesion in the case reported here, no identification to the syndrome isolated by Liebow seems permitted. The authors offer a new denomination for this unusual entity.

Aged↗

[Subcutaneous and tendinous nodules in scleroderma. Apropos of 4 anatomo-clinical cases].

The authors have observed 3 cases of pure scleroderma and one case of scleroderma in which tendinous or subcutaneous nodular structures were present. To the author's knowledge, such nodules have never been described previously in cases of scleroderma. Their site and their histology identifies them as "rheumatoid" nodules and at the same time provides other valuable information. Scleroderma is thus, together with rheumatoid polyarthritis and lupus erythematosus, one of the possible causes of nodule formation, and the authors put forward the hypothesis that these nodules might represent one of the first lesions of the Thibierge and Weissenbach syndrome.

Adult↗

[Migrant eruptions in lupus erythematosus. Apropos of a case of lupus erythematosis gyratus repens].

Rare cases of migrant circinated eruptions occurring during the course of lupus erythematosus have been published so far. They have been classified in two categories: 1) supposed toxicodermias due to antimalarials; 2) real cases of erythema annulare centrifugum, mostly inaugurating the L. E. In an exceptional case, a migrating eruption looking like an erythema annulare with a complicated pattern on the trunk, neck and limbs, took place between two rashes of typical L.E. The exact nature of this eruption, clinically and histologically atypical, and its relation with the L.E. itself, have been discussed.

Aged↗

[Fabry's disease without cutaneous angiokeratoma : diagnosis by electron microscope study of skin biopsy (author's transl)].

A case of Fabry's disease without typical skin lesion is reported. Electron microscope study of normally appearing skin (at clinical and even histological levels) has shown typical inclusions within cytoplasm of various dermic cells (endothelial cells and pericytes, myocytes, fibroblasts) and adipocytes. These inclusions are made up of stacks of membranes with parallel array and periodicity about 5 nanometers. Such a case emphasizes usefulness of electron microscopy of skin, even normally appearing, for the diagnosis of Fabry disease, as it has been previously shown for other dyslipidoses.

Adult↗