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Biomedical subjects

J Wechsler

Publications and source records attributed to J Wechsler.

At least 253 records · Page 14Linked to original sources
PubMed1982

["Deep scleroderma" or diffuse fibromatous scleroderma. A case-report of uncertain nosologic classification (author's transl)].

An unusual form of scleroderma, seen in one patient, is described. Clinical and histological examinations demonstrated no dermal changes. Both the subcutaneous tissue and the fascias were involved. This case can be distinguished from Shulman fasciitis, on both histological and biological grounds, as well as from scleroderma, since Raynaud phenomena, sclerodactylia, and visceral involvement were lacking.

Adult↗
PubMed1982

Bullous dermatosis associated with dysglobulinemia (two cases). Relationships with epidermolysis bullosa acquisita.

Two cases of bullous dermatosis with dysglobulinemia are described. The first one was associated with renal and neuromuscular amyloidosis and production of a monoclonal lambda IgG. Optical and electron microscopy showed amyloid deposits beneath basal lamina of the dermis. Results for direct and indirect immunofluorescence (IF) were negative. This bullous dermatosis is not an epidermolysis bullosa acquisita (EBA), in the strict sense, because the amorphous material deposited is amyloid. In the second case, associated with Waldenström's disease, there was no cutaneous or systemic amyloidosis. Direct IF was positive; linear IgM deposits were seen along the basal membrane of the bulla and the healthy skin. Indirect IF showed the presence of circulating antibodies against basal membrane zone. This bullous dermatosis is probably an EBA, despite the absence of IgG deposits. The absence of electron microscopy does not permit the confirmation of this diagnosis.

Aged↗
PubMed1982

[Epidermolysis bullosa acquisita (EBA). Morphological, immunocytochemical, optical and ultrastructural study about one case (author's transl)].

Epidermolysis bullosa acquisita (EBA) is a rare non hereditary bullous disease. One case is described with a morphological, and immunocytochemical ultrastructural study. The plain ultrastructural morphology showed a dense amorphous deposit in the superficial dermis, under the basal lamina. Immunofluorescence showed IgG and C3 deposits. The ultrastructural study (direct immunoperoxidase technique using Fab) showed a dense labelling associating : dark dots spaced under the basal lamina and fogging less dense labelling in the clarified superficial dermis. Using the patient's antibodies an indirect reaction on normal human skin performed : the same dotted sub laminal labelling was found but with no dermal diffuse reaction product. The study shows the site and the aspect of the Ig deposit in EBA and indicates that the antibodies demonstrated in some patients are reactive with a constituent of the normal superficial dermis.

Epidermolysis Bullosa↗
PubMed1981

[Pulmonary interstitial electron dense deposits in lupus erythematosus. Interest of the transbronchial biopsy (author's transl)].

The case reported concerned a patient 44 years old, affected by a systemic lupus erythematosus for 18 years and admitted for an acute respiratory distress due to a bronchopulmonary infection. After this episode an interstitial chronic lung disease is diagnosed (reticulonodular pattern on the chest X-ray, functional restrictive syndrome). The study of the transbronchial lung biopsy by electron microscopy revealed interstitial electron dense deposits. This observation focussed on the interest of this technic of morphological study mainly in chronic lung interstitial diseases.

Adult↗
PubMed1981

[Lupus, insulin-resistant diabetes and acanthosis nigricans (author's transl)].

The authors report the case of a 22-year-old Guatemalan in whom lupus was diagnosed 8 months after a second pregnancy. The diagnosis of lupus met the criteria of the ARA: Raynaud's syndrome, alopecia, arthralgia, thrombophlebitis, facial erythema, antinuclear factor at 1/100, Farr at 75 p. 100 and immunofluorescent demonstration of IgM binding in healthy skin. Two months after the beginning of the lupus, there was onset of insulin-resistant ketosic diabetes without overweight. The serum insulin was 1.140 mu U/ml. Acanthosis nigricans was noted and confirmed by a biopsy. Insulin-resistance can be attributed to anti-membrane receptor antibodies titrating at 1/200 (R. Khan). The short-term progress of the disease was favorable on corticosteroid treatment. Insulin could be stopped, but high insulin serum levels persisted. This case meets criteria for type B as defined by Flier, Khan and Roth, and is the first European case of lupus with a complete presentation. Short-term progress was favorable, and there is no evidence to affirm that there will be a tardive progression towards hypoglycemia which is, however, possible due to the persistence of elevated serum insulin levels.

Acanthosis Nigricans↗
PubMed1979

[Eosinophilic fasciitis (Shulman syndrome) associated with insulin-dependent diabete. 1 case (author's transl)].

A patient with eosinophilic fasciitis and insulin-dependent diabete mellitus is recorded. Diabete and blood hypereosinophilia occurred four months before acute onset of severe proximal sclerodermic features. The deep seated level of fibrosis, mainly localized in subcutaneous fat and fascia was documented by histologic examination. Prednisone therapy (0,75 mg/kg/day) resulted in cutaneous improvement and disparition of eosinophilia. Survey of pertinent literature lead us to consider Shulman's syndrome as a distinct entity amid the broad scleroderma spectrum. Features of esosinophilic fasciitis are very close to, and sometimes undistinguable from generalized morphea. Simultaneous occurence of diabete mellitus in this case is not fortuitous. The same association has already been recoreded with various sclerodermic syndromes.

Adult↗