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Biomedical subjects

O Shamai-Lubovitz

Publications and source records attributed to O Shamai-Lubovitz.

4 recordsLinked to original sources

Immunologic abnormalities associated with primary anetoderma.

BACKGROUND AND DESIGN: Primary anetoderma is a rare cutaneous elastolytic disorder, the etiopathogenesis of which has not yet been established. Six patients with primary anetoderma were studied in an attempt to assess the role of the immunologic system in the elastolytic process. The investigation included the medical history, physical examination, routine blood tests, specific tests for collagen diseases, prothrombin time, activated partial thromboplastin time, thyroxine, indirect immunofluorescence test, and skin biopsies for histopathologic study and direct immunofluorescence. RESULTS: Two of the patients presented with autoimmune disorders: the first had Graves' disease, lupus anticoagulant, and autoimmune hemolysis, and the second had systemic scleroderma. There were positive direct immunofluorescence findings in most of the patients. Furthermore, all of them were found to have serologic immunologic abnormalities, of which the most common was a positive antinuclear factor. CONCLUSIONS: These findings indicate that there is an immunologic involvement in primary anetoderma.

Adult

Primary anetoderma associated with a wide spectrum of autoimmune abnormalities.

Although the underlying pathologic mechanisms of primary anetoderma have not yet been identified, data suggest the participation of an immunologic mechanism in some cases. In a woman with clinical and histopathologic features of primary anetoderma (Jadassohn-Pellizzari type) of 30 years' duration, laboratory investigation disclosed positive antinuclear factor, hypocomplementemia, hypergammaglobulinemia, granular deposits of immunoreactants along the dermoepidermal junction, and fibrillar deposits in the papillary dermis. In addition, she was found to have autoimmune hemolysis and circulating lupus anticoagulant associated with recurrent deep-vein thrombosis and a history of Graves' disease (starting 5 years after onset of primary anetoderma). To our knowledge, none of the latter three autoimmune conditions has been previously associated with primary anetoderma.

Antibodies, Antinuclear

The clinical and histopathological spectrum of IgA-pemphigus--report of two cases.

Two new cases of neutrophilic bullous disease exhibiting bound and circulating intercellular IgA in vivo in both direct and indirect immunofluorescence (IF), for which the term IgA-pemphigus was recently proposed, are presented. The first case showed a unique constellation of clinical and histopathological features not previously described for IgA-pemphigus, resembling both dermatitis herpetiformis and pemphigus, for which the designation IgA-herpetiform pemphigus seems most appropriate. The second case showed clinical and histopathological features mimicking subcorneal pustular dermatosis for which the previously used term IgA-pemphigus foliaceus seems most appropriate. The previously reported 11 cases showing similar direct IF findings, as well as our two patients, illustrate the main differences between IgA-pemphigus and classic forms of this condition: (a) different clinical manifestations with the absence of Nikolsky's sign; (b) scanty acantholysis; an abundance of neutrophils, with occasional neutrophilic spongiosis; (c) absence of C3 on direct IF; (d) the lower sensitivity of indirect IF and low levels of circulating antibodies; (e) the relatively benign course of the disease; (f) the apparent responsiveness to sulphones in many cases. It is suggested that IgA pemphigus should be considered as an entity separate from, but closely related to, classic pemphigus.

Diagnosis, Differential