Biomedical subjects
Publications and source records attributed to B Hazaz.
The case history of a baby with neonatal pemphigus vulgaris is presented. This is the 13th case of pemphigus vulgaris during pregnancy reported in the literature. The correlations between the clinical, histologic, and immunofluorescent findings are discussed and a review of all previously reported cases is presented.
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Seven of 60 human monoclonal anti-DNA autoantibodies derived from 3 patients with SLE were shown to bind to Raji cells by radioimmunoassay. The binding of the lupus autoantibodies to the Raji cells in solution was not affected by prior incubation of the cells with DNase. Preincubation of the monoclonal autoantibodies with polynucleotides and cardiolipin, resulted in significant inhibition which correlated with the direct binding characteristics of these antibodies. Previous results with mouse IgG monoclonal anti-DNA antibodies supported by our results with human IgM anti-DNA autoantibodies suggest caution in interpreting analyses of immune complexes of sera containing anti-DNA antibodies entailing Raji cells.
Two cases of elastosis perforans serpiginosa were studied by direct immunofluorescence. In one case, homogeneous deposits of IgM, C3 and C4 on the abnormal elastic fibres in the papillary dermis were demonstrated. In addition, in both cases, along the basement membrane zone, coarse linear deposits of fibrinogen and properdin were observed inside and near the transepidermal channels. Groups of cytoid bodies stained for IgM and occasional weak linear deposits of IgG, IgA, IgM and C3 were also observed. The immune deposits on the abnormal elastic fibres might indicate antigenic alteration and activation of humoral immunological mechanisms in the process of their elimination. The other findings may reflect a non-specific binding by damaged tissue structures.
Seminal fluid from 170 men was examined for acrosomal abnormalities of sperm (Papanicolaou procedure X 1000) and for y-positive cells (quinacrine-stained smears). No correlation was found between these parameters, suggesting that acrosomal abnormalities are similarly distributed among x- and y-bearing spermatozoa. The proportion of y-positive sperm was found to be low (23.6 + 10.7% (SD) for the oligozoospermic specimens and 23.7 +/- 9.8% for specimens with sperm counts above 40 million/ml). Three degrees of fluorescence intensity were observed--weak, moderate, and strong--the strong fluorescence being associated with the highest percentage of y-bodies (30.6 +/- 12.7%, 36.6 +/- 5.7%) and the weak fluorescence with the lowest (16.7 +/- 7.0%, 15.0 +/- 9.5%). It is suggested that structural abnormalities and/or metabolic alterations in either the DNA molecule or the chromosomal proteins may be responsible for variability in the ability to bind quinacrine compounds.
Since high-molecular-weight levan is known to reduce capillary permeability to large molecules, an experiment was designed to investigate whether this agent may attenuate the glomerulonephritis associated with acute serum sickness in rabbits. The study, in fact, demonstrated an enhancing effect of levan, which caused increased glomerular proliferative changes and leucocyte infiltration and, possibly, increased IgG deposition in this experimental model. In addition, rabbits injected only with levan also demonstrated mild glomerulitis and C3 deposition. In one of four rabbits examined, this was accompanied by a marked fall in the serum level of total haemolysing complement. Levan was demonstrated to cause activation of complement when incubated with normal rabbit serum in vitro. We believe that these findings are best explained on the basis of complement activation in situ by levan in the glomeruli, probably via the alternative pathway, with the resulting inflammatory response. In the case of BSA-injected rabbits, this response is believed to be additive to that of the classical immune complex-mediated complement activation.
A case of acute cutaneous leishmaniasis is reported in which skin biopsy of the lesion revealed deposits of IgM, fibrinogen and C3 in the blood vessel wall and granular deposits of IgG and C3 at the dermoepidermal junction. No immune complexes were detected in the healthy unaffected skin in the same patient and no circulating immune complexes were found in the blood. This case demonstrates tissue-bound immune complexes in cutaneous leishmaniasis.
The coexistence of cryoglobulinemia and C4 deficiency was found in one family. Twelve subjects were examined, most of them being asymptomatic. Cryoglobulins were found in 9 members and its type was defined in 6 of them, 3 having mixed IgM-IgG and 3 having monoclonal IgM cryoglobulins. The concentration of the third component of the complement system was normal in 10 subjects, while the concentration of the fourth one was found to be below the lower limit of normal in 9 subjects. Only C4 deficiency--and not cryoglobulinemia--was HLA-linked. There was no evidence of HBs antigenemia. The results of this study are discussed in the light of the pertinent literature.
Skin lesions of acute cutaneous leishmaniasis were found in three healthy men. A smear for Leishman-Donovan (LD) bodies as well as histopathologic studies of the lesions confirmed the diagnosis. Direct immunofluorescent studies of the lesions revealed deposits of immunoglobulins and complement in dermoepidermal junctions and fibrinogen in dermal blood vessels.
Massive proteinuria associated with chronic pyelonephritis is reported in five patients. Two of them were known to have vesicoureteral reflux, whereas three had minor predisposing factors only. None of the patients were in severe renal failure or suffered from severe hypertension at the time of massive proteinuria. Results of histological examinations were compatible with a diagnosis of chronic pyelonephritis. Electron microscopy and immunological studies did not show any primary glomerular lesions.
Renal biopsy specimens from eight patients with cryoglobulinemic nephropathy were examined by light, electron, and immunofluorescence microscopy. The light microscopic features were not diagnostic. Characteristic ultrastructural findings were observed in all except one specimen and consisted of glomerular electron-dense deposits, which had a coarse, granular appearance at low-power magnifications and revealed tubular, annular and in a few cases, also fibrillar structures at high-power magnifications. Subendothelial deposits were a constant finding. The presence of intravascular thrombi (deposits), osmiophilic bodies, and crystals within the cytoplasm of the glomerular cells are also helpful for the diagnosis of this entity. Because of these specific findings, cryoglobulinemic nephropathy should be recognized as a clinicopathologic entity.
After 3 years of treatment with azathioprine and prednisone, immunoblastic lymphoma appeared in a patient with systemic lupus erythematosus. An increased incidence of immunological aberrations and malignancies was found in her family and their relation to the pathogenesis of this patient's diseases is discussed.
A 27-year-old woman presented with skin lesions and arthritis of the right knee of two days' duration. Her medical history was noncontributory and synovial fluid aspirated from the affected knee and a biopsy of a skin lesion were negative for gonococci. However, after methylene blue staining of cervical and urethral smears, intracellular and extracellular diplococci were demonstrated. Immunofluorescence showed deposits of immunoglobulin complexes of the skin section within the wall of blood vessels and dermal-epidermal junction. Treatment with ampicillin, 4 gm a day, resulted in resolution of the lesions and arthritis in two days. The need for cervical and urethral smears and for better, as yet unavailable tools for the diagnosis of the arthritis dermatitis syndrome, is emphasized.
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The skin biopsy specimens from six patients with primary mixed IgM-IgG cryoglobulinemia were examined by immunofluorescence, light and electron microscopy. The biopsy taken from the involved skin of one patient with leg ulcers revealed small blood vessel occlusions by cryoglobulin aggregates. Since a similar finding was not observed in the biopsy material taken from the other five patients who had no ulcerative skin lesions, it seems that the cryoglobulin aggregates play a role in the development of the skin ulcerations in primary mixed IgM-IgG cryoglobulinemia.
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