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Biomedical subjects

B Hazaz

Publications and source records attributed to B Hazaz.

At least 19 recordsLinked to original sources
PubMed1992

A study of keratin expression in benign familial chronic pemphigus.

We studied keratin expression in the involved and uninvolved skin of six benign familial chronic pemphigus (BFCP) patients, using monoclonal antibodies specific for various keratin polypeptides and immunohistopathologic techniques. Normal and psoriatic (i.e., hyperproliferative) skin specimens served as controls. The uninvolved BFCP epidermis showed keratin profiles identical to that of normal epidermis. In the acantholytic epidermal segments of the involved BFCP skin, some of the lower suprabasal acantholytic cells failed to express keratin polypeptides 10 and 11 (Moll's catalog). This delay in expression of suprabasal keratins was not accompanied by an expression of hyperproliferative keratin polypeptide 16. Also, some of the lower suprabasal acantholytic cells of the involved BFCP epidermis retained staining by the antikeratin KS-1A3 antibody, which in the normal, psoriatic, and uninvolved epidermis was limited to the basal cell layer. Staining for keratin polypeptide 18 was negative in the epidermis of all four types of specimens. We believe that the delay in suprabasal keratin expression in the involved BFCP epidermis was more likely secondary to the acantholysis (i.e., "arrest of differentiation" due to acantholysis) rather than due to a primary defect in keratin expression.

Adult↗
PubMed1992

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↗
PubMed1991

Esophageal involvement in pemphigus vulgaris: a clinical, histologic, and immunopathologic study.

Eleven newly diagnosed patients and one patient with pemphigus vulgaris who relapsed underwent endoscopy of the upper gastrointestinal tract. Three patients had blisters or erosions and two had longitudinal lines of erythema in the esophageal mucosa. In four patients histopathologic examination showed findings of pemphigus vulgaris but direct immunofluorescence was positive in all patients. This study demonstrates that the immunopathologic disturbance in pemphigus vulgaris involves the entire length of the esophagus, although only some patients have clinical or histologic involvement. To the best of our knowledge this is the first histopathologic and direct immunofluorescence study of esophageal involvement in patients with untreated pemphigus vulgaris.

Adult↗
PubMed1991

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↗
PubMed1990

Conjunctival involvement in pemphigus vulgaris: a clinical, histopathological and immunofluorescence study.

Eleven patients with pemphigus vulgaris and with eye complaints had histopathological and direct immunofluorescence (DIF) studies on biopsies of their conjunctivae. In eight of the patients the ocular symptoms preceded the other manifestations of pemphigus. None of the 11 patients had any detectable conjunctival blisters or erosions. The conjunctivitis in three of the patients proved on histopathology and DIF to be a manifestation of pemphigus vulgaris. In the remaining patients, conjunctival hyperaemia with or without a mucoid discharge was observed, but there were no specific histopathological features, although one of them had a positive DIF.

Adult↗
PubMed1990

An immunofluorescence study of primary anetoderma.

Primary anetoderma (PA) has occasionally been described in association with lupus erythematosus (LE). The present study was performed to elucidate a possible causal link between PA and LE by the use of direct and indirect immunofluorescence (IF) methods. Two patients with PA were studied. Biopsy specimens were obtained from early inflammatory and atrophic anetoderma lesions and from the exposed and unexposed uninvolved skin of each patient. The pattern of immune deposits observed in one patient was indistinguishable from that which is often seen in systemic LE, and in the other patient from that which may be observed in chronic cutaneous LE. The direct IF study also showed fibrillar immune deposits in the dermis that resembled elastic fibres morphologically. The indirect IF study, however, failed to demonstrate anti-elastic fibre antibodies in the patients' sera. The results of this study and a review of the literature suggest that some cases of PA have direct IF findings similar to those of either chronic cutaneous or systemic LE. However, these findings, along with the serological findings, are insufficient to establish a diagnosis of LE in most of these PA cases.

Adult↗
PubMed1990

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↗
PubMed1989

Characterization of T-lymphocyte subpopulations infiltrating primary breast cancer.

Characterization of T-lymphocyte subpopulations adjacent to and infiltrating the primary tumor of breast cancer was carried out using a direct immunofluorescence procedure with the antibodies anti-(Leu-2a) for suppressor/cytotoxic (CD8+) and anti-(Leu-3a) for helper/inducer (CD4+) T-lymphocytes. Fifty-six primary malignant tumors with lymphoid infiltration were studied. The majority (58.9%) were infiltrating duct carcinoma. There were metastases to axillary lymph nodes in 6.67% of the patients. Massive lymphoid infiltration (greater than 40 lymphocytes per x 400 microscopic field) was found in 19.6% of the tumors and moderate infiltration (20-40 lymphocytes per field) in 51.8%. In all the tumors studied there was a reversed CD4+/CD8+ ratio as compared to that found in normal peripheral blood. In 66.1% the CD4+/CD8+ ratio (helper/suppressor) was less than 1.0. The reversed ratio was due to a significant decrease in the number of helper cells (P less than 0.0005). The most significant drop was in the stroma area (P less than 0.0001) as well as in the tumor tissue (P = 0.001). Of particular interest was the significant positive correlation between the age of the patients and an increased number of CD4+lymphocytes in the stroma (P = 0.02). Significant negative correlations were found between a reduced number of CD4+ lymphocytes or CD4+/CD8+ ratio and several histological parameters: tumor diameter, pleomorphism, nucleus/cytoplasm ratio. There was also a significant positive correlation between the total number of CD8+ lymphocytes infiltrating the tumor tissue and the number of axillary lymph nodes with metastatic disease (P = 0.03). It is suggested that the reversed ratio of CD4+/CD8+ lymphocytes may significantly affect the host/tumor immune surveillance.

Adult↗
PubMed1989

The usefulness of immunofluorescent tests in pemphigus patients in clinical remission.

Direct and indirect immunofluorescent studies (DIF, IIF) were performed on 24 pemphigus vulgaris patients who were in a state of clinical remission. The tests were repeated after an interval of 6 months. All the patients were on maintenance therapy with oral prednisone. The DIF in eight patients showed negative results among whom seven remained negative. Six patients out of 24 showed weakly positive fluorescence and ten patients showed strong positive fluorescence. The IIF was negative in 17 patients and positive in seven patients who also showed positive DIF. During a follow-up period of 20 months, one of eight patients with negative DIF relapsed compared with two of six patients with weak positive DIF and five of 10 patients with strong DIF. Five patients with strong DIF for IgG also had C3, of whom three relapsed, compared with five of 19 patients who were negative for C3. Four of seven patients with positive IIF relapsed compared with four of 17 with negative IIF. It is suggested that repeated DIF tests in pemphigus patients, who are in clinical remission, may serve as an indicator for the immunological activity and be of help in the management of these cases.

Adult↗
PubMed1989

Determination of IgG subclasses in patients with pemphigus with active disease and in remission.

IgG subclasses were determined in perilesional skin of 13 patients with pemphigus with active disease and of 14 patients in a state of clinical remission. Using indirect immunofluorescence technique, frozen sections were incubated with mouse monoclonal antihuman IgG1, IgG2, IgG3, and IgG4 followed by a second incubation with fluorescein isothiocyanate-conjugated goat antimouse IgG. The results showed that among patients with active disease, IgG1 was found in all of them and IgG4 in 85%, while IgG2 and IgG3 were found in 54% and 77%, respectively. For patients in remission the most common subclass was IgG4 in 79% of patients, and in a decreased order IgG1, 50%; IgG3, 29%; and IgG2, 14%. It appears that IgG1 and IgG4 are predominant among patients with active lesions. IgG1 seems to be the most sensitive indicator for activity of the disease. IgG4, normally found in the lowest concentration in human serum, is the most common subclass in patients who are in remission. IgG3 and C3 may have a predictive value for remission.

Adult↗
PubMed1988

Hormonal evaluation and autoimmune background in pruritic urticarial papules and plaques of pregnancy.

There is little insight into the pathogenesis of most of the dermatoses specifically associated with pregnancy. We evaluated the hormonal profile and the autoimmune background in 11 pregnant women with pruritic urticarial papules and plaques of pregnancy. No statistically significant difference was found between the serum levels of the beta-subunit of human chorionic gonadotropin, estradiol, cortisol, and urinary estriol of the patients and gestational age-matched control subjects. No autoantibodies were found in the patients' group. We conclude that patients with pruritic urticarial papules and plaques of pregnancy have no hormonal alterations when compared with normal pregnant women and that no known major autoimmune background plays a part in the pathogenesis of the disease.

Adult↗
PubMed1988

Bullous pemphigoid mimicking bullous erythema multiforme: an untoward side effect of penicillins.

Three young and middle-aged patients developed severe bullous eruption after receiving various penicillins. The clinical findings included high fever, prostration, widespread bullous eruption mainly on the head, face, and extremities, targetlike lesions on the palms and soles, and severe erosions in almost all the mucous membranes. Direct and indirect immunofluorescence studies were those of bullous pemphigoid. Complete clearing of the eruption occurred with prednisone therapy. We conclude that drug-induced bullous pemphigoid is a different entity from the classic bullous pemphigoid.

Adolescent↗
PubMed1988

Linear IgM dermatosis of pregnancy.

An intensely pruritic dermatosis that occurred in a woman during the third trimester of pregnancy is described. The clinical manifestations included red follicular papules symmetrically distributed on the skin of the forearms, abdomen, thighs, and legs. The histopathologic findings were not specific. However, immunopathologic examination revealed dense linear deposition of IgM in the dermoepidermal junction. The eruption and the immunopathologic findings disappeared at the end of the puerperium. This dermatosis differs from previously described specific dermatoses of pregnancy because of the clinical appearance and the immunopathologic findings. Thus we propose the term linear IgM dermatosis of pregnancy.

Adult↗
PubMed1987

Possible role of fibrinogen in the aggregation of white blood cells.

In order to verify whether leukocyte aggregation correlated with aggregation of other cellular elements during inflammation, we examined the state of leukocyte adhesiveness/aggregation (LAA) in the peripheral blood and red cell aggregation. Correlation was found to be significant as was the correlation between LAA and fibrinogen, and with the fibrin/fibrinogen degradation products concentration during various inflammatory states. In vitro leukocyte aggregation was decreased when the cells were suspended in autologous heat defibrinogenated plasma as compared to cells suspended in autologous native plasma. Heat aggregated fibrinogen but not native fibrinogen caused leukocyte aggregation in vitro. Finally, Arvin defibrinogenation in rabbits reduced the state of LAA in endotoxinemic rabbits. Integrating all this information, we assume that fibrinogen participates not only in the aggregation phenomena of red cells and platelets, but also in those of leukocytes.

Animals↗
PubMed1987

Diffuse, progressive hyperpigmentation: an unusual skin manifestation of mycosis fungoides.

Pigmentary changes in mycosis fungoides usually occur in association with poikiloderma atrophicans vasculare or following therapy and regression of lesions. Several cases of hypopigmented mycosis fungoides have also been reported. We present the case report of a patient who developed pruritic, diffuse macular hyperpigmentation of the skin. Biopsy specimens from hyperpigmented skin revealed histologic and ultrastructural features typical of mycosis fungoides. Giant melanin granules were found in the tumor cells, as well as in keratinocytes and Langerhans cells. As far as we know, this is the first report of cutaneous hyperpigmentation as a single presenting sign of mycosis fungoides.

Aged↗
PubMed1987

Pruritic urticarial papules and plaques of pregnancy. A review of 21 cases.

Twenty-one patients with pruritic urticarial papules and plaques of pregnancy (PUPPP) were reviewed. Direct immunofluorescence (DIF) examination was negative in all patients. DIF in patients with PUPPP is a useful and reliable laboratory aid in distinguishing this peculiar dermatosis from herpes gestationis.

Adult↗