Perplexing parlance of paraneoplastic pemphigus.
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Autoantibodies↗
Biomedical subjects
R Valenzuela
Publications and source records attributed to R Valenzuela.
Continuous dermoepidermal junction IgM detected by direct immunofluorescence: a report of nine cases.
BACKGROUND: Although a linear IgM dermatosis of pregnancy has been reported, other authors have not found evidence for a distinct linear IgM dermatosis. OBJECTIVE: We set out to determine whether or not continuous dermoepidermal junction IgM deposition detected by direct immunofluorescence was indicative of a specific disease. METHODS: We collected nine cases during a 9-year period in which biopsy specimens for direct immunofluorescence revealed continuous linear IgM at the dermoepidermal junction in the absence of other immunoglobulins. In all of these cases the medical record was available for review. RESULTS: Clinical correlation revealed that these patients had a heterogeneous group of diseases with urticaria and leukocytoclastic vasculitis being most common. No diagnostic specificity could be ascribed to the linear IgM pattern, although six of our nine cases could be considered manifestations of a hypersensitivity response (e.g., urticaria, leukocytoclastic vasculitis, pigmented purpuric dermatosis, or hypersensitivity dermatitis). CONCLUSION: Pathologists and dermatologists should be aware that linear IgM can be seen in a variety of clinical settings. The etiopathogenic significance of this finding, if any, remains to be determined. At present, the finding must be viewed as nonspecific.
Adult↗
Relapsing polychondritis: a case diagnosed by direct immunofluorescence and coexisting with pseudocyst of the auricle.
A case of relapsing polychondritis with perichondrial IgG and C3, dermoepidermal IgG, and a coexisting pseudocyst of the auricle is reported. The use of direct immunofluorescence testing in establishing a diagnosis of relapsing polychondritis and the expected findings are reviewed. In some cases direct immunofluorescence may allow a diagnosis of relapsing polychondritis before clinical criteria or a routine histologic evaluation suggests a definite diagnosis.
Aged↗
Paraneoplastic pemphigus: a report of three cases including one long-term survivor.
BACKGROUND: Paraneoplastic pemphigus is a newly described autoimmune disease characterized by painful mucosal ulcerations and polymorphous skin lesions in association with an underlying neoplasm. All reported patients with an associated malignant neoplasm have had a poor prognosis. OBJECTIVE: We present three new cases of paraneoplastic pemphigus associated with a malignant neoplasm and further characterize this disease. METHODS: We used clinical criteria, histologic and immunopathologic examinations, and immunophenotyping to characterize this disease. In addition, we performed immunoprecipitation studies with extracts of radiolabeled human keratinocytes to characterize the antigens to which patient serum binds. RESULTS: All three patients had clinical, histologic, and immunopathologic findings that were strongly suggestive of paraneoplastic pemphigus. Their sera immunoprecipitated a complex of four polypeptides from human keratinocyte extracts with molecular weights of 250, 230, 210, and 190 kd, confirming the diagnosis of paraneoplastic pemphigus. The 250, 230, and 210 kd antigens comigrated with desmoplakin I, the 230 kd bullous pemphigoid antigen, and desmoplakin II, respectively. Lymphocyte immunophenotyping revealed large populations of monoclonal CD19+, CD5+ B cells in two cases. Although two of the patients died, one patient is alive and well 2 years after the diagnosis of paraneoplastic pemphigus. CONCLUSION: We report three cases of paraneoplastic pemphigus. One patient is alive and well 2 years after diagnosis, which suggests that a subgroup of patients may have a more benign course.
Aged↗
Oral and cutaneous lichen planus pemphigoides.
Lichen planus pemphigoides is a rare bullous disorder characterized by tense bullae on lichen planus lesions and on clinically uninvolved skin. A diagnosis of lichen planus pemphigoides is made on the basis of clinical, histologic, and immunopathologic evaluation. We describe a patient who had lichen planus pemphigoides of the skin and oral mucosa and briefly review the literature on this uncommon entity.
Aged↗
Glandular basement membrane zone immunofluorescence in cicatricial pemphigoid.
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Aged↗
Cell cycle analysis by flow cytometry of non-exposed, sun-exposed, and tretinoin-treated skin.
The percentage of keratinocytes in the proliferative phase of the cell cycle (S + G2 + M) was measured by DNA flow cytometry in sun-exposed, non-exposed, and tretinoin-treated skin. Before tretinoin treatment, the percentage of keratinocytes actively cycling was higher in sun-exposed than in non-sun-exposed skin (p = .002) and was correlated with clinically assessed photodamage (p = .007). Subsequently, tretinoin-treated sun-exposed skin was compared to the pre-treatment sun-exposed skin. Overall, there was no statistically significant change. However, there was a trend toward a decrease in the percentage of keratinocytes in the S + G2 + M phases immediately after four months of tretinoin use that was limited to the most severely damaged patients. This effect was no longer evident two months after discontinuing treatment. This is the first study, to our knowledge, utilizing flow cytometry to investigate the effects of tretinoin in patients with varying degrees of photodamage.
Adult↗
Delayed cyanide induced dystonia.
A 16 year old man ingested 1 g potassium cyanide in 1969. A few days after an apparently full recovery he developed a severe dystonia syndrome. He had a positive response to an apomorphine test and showed improvement with levodopa treatment. A 21 year follow up showed minimal neurological sequelae; CT showed bilateral putaminal lucencies. Visual and brain stem auditory evoked potentials were normal.
Adolescent↗
Immunoelectrophoretic and immunohistochemical characterizations of fibrinogen derivatives in atherosclerotic aortic intimas and vascular prosthesis pseudo-intimas.
Cadaveric aortic intimas with uncomplicated atherosclerosis were examined to determine the distribution and polypeptide chain composition of fibrinogen-related protein. Immunohistochemical staining showed deposits rich in fibrinopeptides A and B. The deposits were usually disseminated throughout intimas of moderate thickness < 0.7 mm, but were distributed focally in elongate patches bounded both lumenally and medially by deposit-free tissue in thick atheromas. Saline extracts generally showed undegraded monomers and dimers by electrophoresis. The residual protein contained A alpha and gamma-chains that were cross-linked predominantly (>80%) into unresolved high M(r) (>200 kd) derivatives, whereas B beta-chains were left non-cross-linked, as occurs in late stages of cross-linking by transglutaminases. The resolved components had electrophoretic mobilities corresponding to characteristic products of both factor XIIIa and tissue-transglutaminase. A greater incorporation of alpha- rather than gamma-chains into cross-linked products implicated tissue-transglutaminase as contributing heavily. By contrast, vascular graft pseudo-intimas and a cadaveric clot were rich in degraded fibrin devoid of fibrinopeptide A, and cross-linked in patterns typical of XIIIa with gamma 2 dimers constituting the principal product. The findings indicate that the fibrinogen in the aortic intima is comparatively well protected from thrombin and plasmin, and that much of it is deposited through direct cross-linking by tissue-transglutaminase without being converted to fibrin.
Antibodies, Monoclonal↗
Nitrogen mustard as induction therapy for rheumatoid arthritis: clinical and immunologic effects.
We treated 5 patients diagnosed with rheumatoid arthritis (RA) with nitrogen mustard (HN2) and monitored clinical and immunologic variables. HN2, 0.3 mg/kg ideal body weight was given over 7 days. Disease activity and immune function were monitored during and after treatment. Duration of morning stiffness (p = 0.0044), joint count (p = 0.0140), and assessment of pain (p = 0.0264) and function (p = 0.0057) improved by Day 6. T lymphocytes (p = 0.0060), especially T memory cells (CD4CD29; p = 0.0017) fell dramatically. HN2 is effective for rapidly gaining control of active RA. This effect is T cell specific.
Aged↗
Pseudo-gamma heavy chain (IgG4 lambda) deposition disease.
Two patients with Ig deposition disease presented with acute renal failure, moderate proteinuria, and hematuria. A plasmacytoid lymphocytic infiltrate was identified in bone marrow that produced IgG4 lambda and free lambda light chains. One patient developed an anaplastic plasmacytoma (secreting only lambda light chains) 1 yr after renal biopsy. Renal biopsy in both patients demonstrated a nodular intercapillary glomerulopathy and electron dense granular deposits, associated with a linear pattern of IgG4 heavy chain deposition in vascular, tubular, and glomerular basement membranes (VBM, TBM, and GBM). In one patient this entrapped IgG4 was unassociated with detectable kappa or lambda light chains. In the second patient, lambda light chains (1+) were detected only in the GBM, but IgG4 (4+) was identified in GBM/TBM. Neither circulating (peripheral blood and bone marrow serum) nor cellular free gamma chains were present. We propose the term "pseudo-gamma heavy chain deposition disease" for the process.
Aged↗
Pharmacological characterization of the inhibitory activity of beta h-endorphin (beta h-EP), [Arg9,19,24,28,29]-beta h-EP, [Gln8,Gly31]-beta h-EP-Gly-Gly-NH2, in the neuroeffector junction of the mouse vas deferens.
The inhibitory opioid activities of beta h-endorphin (beta h-EP), its structurally related peptide analogues [Gln8,Gly31]-beta h-EP-Gly-Gly-NH2 (Gly-Gly-beta h-EP), [Arg9,19,24,28,29]-beta h-EP (Arg-beta h-EP) and methionine enkephalin have been examined in the electrically stimulated mouse vas deferens bioassay. All four peptides behaved as full agonists; methionine enkephalin was the most potent followed by Arg-beta h-EP, beta h-EP and Gly-Gly-beta h-EP. Neither Gly-Gly-beta h-EP nor Arg-beta h-EP antagonized the inhibitory action of beta h-EP or methionine enkephalin. An hour of tissue exposure to 30 nM beta-funaltrexamine followed by thorough washing, displaced to the right, in a parallel fashion, the concentration-response curves of beta h-EP and analogues. Whereas the displacement of the concentration response curves was 8 to 10-fold for beta h-EP and Arg-beta h-EP, it was only about 3-fold for Gly-Gly-beta h-EP and methionine enkephalin. Naltrindole was the most potent antagonist of methionine enkephalin with an apparent pA2 of 9.4; its potency as an antagonist of beta h-EP and related analogues was approximately one-tenth of this with pA2 values approximately 8.5. Norbinaltorphimine also antagonized the action of the opioid peptides with pA2 values close to 7.8.
Animals↗
[Auditory evoked potentials in Fisher's syndrome. A clinical case].
A 15 year old patient developed ataxia 2 weeks after an upper respiratory infection. Absent reflexes, external ophthalmoplegia, bilateral ptosis, isochoric mydriasis and week photomotor reflexes (Bell's phenomenon) were noted. A slight increase in protein but not cell content of the CSF was observed. Miller-Fisher syndrome was diagnosed on clinical grounds and visual and auditory evoked potentials were explored and found to be normal. This is in complete agreement to the literature and indicates indemnity of the central nervous system in Miller-Fisher's syndrome. Peripheral nervous system abnormalities that have been identified by pathologic and radiologic studies may explain all of the alterations observed in this syndrome.
Adolescent↗
Electrophoretic characterizations of cross-linked fibrinogen derivatives in blood and vascular tissue by zonal immobilization on glyoxyl agarose.
Direct immunoprobing of electrophoregrams of plasma and intimal protein on glyoxyl agarose and composite-gels with polyacrylamide have uncovered novel modes of cross-linking of fibrinogen that differ from those previously characterized. These modes of cross-linking involve the A alpha-chains of fibrinogen and hybrid cross-linking of alpha- and gamma-chains.
Electrophoresis, Agar Gel↗
In vivo complement C3 binding to the intercellular substance and cytoplasm of epidermal basal cells in a patient with scleroderma and inclusion body myositis.
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Complement C3↗
Response of immunoregulatory lymphocyte subsets to methotrexate in rheumatoid arthritis.
In an attempt to define the immunoregulatory mechanisms operating in rheumatoid arthritis, the authors examined peripheral blood functional lymphocyte subsets in 15 patients with active rheumatoid arthritis who were not receiving remittive therapy, as well as 33 healthy controls. The percentage and absolute numbers of total T cells (CD3), T-helper/inducer cells (CD4), and T-suppressor/cytotoxic cells (CD8) did not differ among the groups, nor did the CD4:CD8 ratio or the numbers of T cells coexpressing CD4 and the activation markers Ia or IL-2R. However, rheumatoid arthritis patients did have reduced percentages and numbers of CD4+ cells coexpressing the 2H4 antigen (CD45R-naive T cells) (P less than .0003) and CD8+ cells coexpressing the Leu-15 (CD11b) marker (suppressor/effectors) (P less than .0005). Twelve patients then received oral methotrexate, 7.5 mg weekly. Most showed clinical improvement by 4 weeks and all did by 8 weeks. Although changes in the T-cell subsets were not statistically significant, several tended toward normalization. These findings may help explain the immunoregulatory defect in rheumatoid arthritis and the effectiveness of methotrexate in modifying disease activity.
Arthritis, Rheumatoid↗
Pseudohypoproteinemia and multiple myeloma.
Paraproteinemia is an important diagnostic feature of multiple myeloma. The M-protein level reflects tumor burden and helps to determine the response to chemotherapy. A case is described that illustrates the phenomenon of reversible in vitro gelification of an M-protein. Paraprotein IgG1-kappa formed a concentration- and temperature-dependent gel, which was reversed by agitation. Measurement of paraprotein without previous vortexing of the specimen can erroneously lower the apparent M-protein level as well as reduce serum viscosity levels. This phenomenon can downstage the disease at diagnosis, produce inappropriate assessment of treatment response, or lead to premature withdrawal of chemotherapy. In addition, misdiagnosis of hyperviscosity syndrome can occur with serious clinical consequences.
Aged↗
Fibrinogen A alpha and gamma-chain dimers as potential differential indicators of atherosclerotic and thrombotic vascular disease.
Cross-linked fibrin(ogen) dimers are known to be elevated in the plasma of subjects with occlusive vascular disease, and are thought to be fibrin dimers. Immunoelectrophoretic analyses of the dimers, however, indicate that (1) they are predominantly fibrinogen rather than fibrin dimers, and (2) they contain cross-linked A alpha-chains (A alpha-dyads) instead of the gamma-chain dyads that are rapidly formed by factor XIII during blood coagulation. Furthermore, the mobilities of the A alpha-dyads differ from the cross-linked alpha-chain products that accompany the gamma-chain cross-linking by factor XIII. Instead, the mobilities coincide with the distinct A alpha-dyads that are produced by tissue transglutaminase, an intracellular enzyme not normally present in plasma. The intimal fibrinogen deposits in atherosclerotic aortas also possess fibrinopeptide A and cross-linked A alpha-chains. Thus, both the plasma fibrinogen dimers and the intimal fibrinogen deposits appear to derive from the action of released tissue transglutaminase more so than factor XIII. It is proposed that, in the absence of other indications of cytolytic processes, the levels of A alpha-dyads in plasma reflect ongoing cellular injury accompanying atherogenesis. The extent to which gamma-dyads accompany the A alpha-dyads may signal progression of the disease to advanced stages in which ulcerations and occlusive lesions trigger thrombotic complications.
Arteriosclerosis↗