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Biomedical subjects

S Jabłońska

Publications and source records attributed to S Jabłońska.

At least 19 recordsLinked to original sources
PubMed2005

Tumorous variant of scleromyxedema. Successful therapy with intravenous immunoglobulins.

We present an unusual tumorous variety of scleromyxedema mimicking facies leonina in lymphoma. In spite of pronounced and widespread cutaneous changes, hypergammaglobulinaemia and paraproteinaemia, the general condition of the patient was satisfactory, there was no internal involvement and no symptoms of any malignancy. Initially, melphalan and corticosteroids were applied but were not effective. High-dose intravenous immunoglobulin (IVIG) therapy had dramatic effect, and after five 5-day monthly courses the tumours almost regressed and the skin became less hard. After a further five courses in the following year there was complete clearance, which was sustained without any therapy for 1 year (until now). IVIG appears to be the therapy of choice for scleromyxedema. We stress, however, that at the start of therapy, IVIG applications should be supplemented with small doses of melphalan and/or corticosteroids.

Diagnosis, Differential↗
PubMed2000

Primary atrophic profound linear scleroderma. Report of three cases.

We present 3 unusual cases of deep linear, primary atrophic scleroderma, not preceded by inflammatory reaction and sclerosis, involving the subcutis and deeper tissues. These cases differ in the course and prognosis from typical profound scleroderma since they do not lead to disfiguration and crippling deformities. In contrast to the atrophies left after regression of morphea or linear scleroderma, they do not involve the dermis, which does not show discoloration or changes in texture. Infiltrates in the endomysium, involvement of deeper tissues and the progressive character of the disease argue for atypical primary atrophic profound scleroderma. The coexistence in one case of primary facial hemiatrophy appears to indicate also its relationship with primary linear atrophies of the limbs.

Adult↗
PubMed1999

Sympathetic skin response in scleroderma, scleroderma overlap syndromes and inflammatory myopathies.

Sympathetic skin response (SSR), a non-invasive method for evaluation of the autonomic nervous system, was studied in 57 patients with various connective tissue disorders: scleroderma, dermatomyositis, polymyositis, scleromyositis and unclassified collagenoses. The patients were divided into three main groups: scleroderma (SSc), myositis or other inflammatory myopathy (M) and scleromyositis (ScM). The aim of the study was to detect abnormalities of the SSR in the connective tissue diseases, to define the pattern for each group and to evaluate the usefulness of SSR in detection of subclinical impairment of sympathetic cholinergic function. In the myositis group, an abnormal SSR was found in 88% of patients; the main abnormality was absence of the response from the lower limbs (in 50% of patients). In scleroderma, the SSR was abnormal in 77% of patients, consisting mainly of absence of the response from the lower limbs, whereas responses from the upper limbs were normal. In scleromyositis, the SSR was abnormal in 80% of patients, the most frequent finding was an increase in latency in one limb. The SSR changes were most pronounced in connective tissue disorders with myositis or inflammatory myopathy. The SSR, although non-disease-specific, because of its sensitivity, seems to be useful in the assessment of the abnormalities of the autonomic nervous system in scleroderma and inflammatory myopathies. The study showed a very high prevalence of autonomic nervous system dysfunction in connective tissue diseases associated with myopathy or myositis, displaying no clinical symptoms of autonomic system involvement.

Adolescent↗
PubMed1998

Recombinant interferon beta gel as an adjuvant in the treatment of recurrent genital warts: results of a placebo-controlled double-blind study in 120 patients.

BACKGROUND: Pilot studies of adjuvant therapy with topically applied interferons (IFN) have shown promising results in reducing the recurrence of condylomata acuminata (CA). OBJECTIVE: The aim of this multicenter placebo-controlled, double-blind study was to confirm the efficacy and safety of topically applied IFN in a large sample of patients. METHODS: After removal of CA by CO2 laser surgery, electrocautery or cryosurgery, 120 patients applied either recombinant IFN-beta (0.15 or 1.0 x 10(6) IU/g) or placebo gel to the affected areas 5 times daily for 4 weeks. The patients were followed for a total of 24 weeks or until recurrence of CA was observed, respectively. RESULTS: In the 105 patients evaluated for efficacy after 24 weeks, recurrence rates were 75% (27/36 patients) in the placebo group, 62% (21/34) in the 1.0 x 10(6) IU/g group (n.s.) and 54% (19/35) in the 0.15 x 10(6) IU/g group (p = 0.034). Only few mild adverse events were noted, which were almost limited to the application site. CONCLUSION: Topical application of gel containing 0.15 x 10(6) IU/g recombinant IFN-beta is safe and appears to reduce the recurrence of CA after surgical treatment.

Administration, Cutaneous↗
PubMed1997

Serum levels of soluble tumor-necrosis-factor receptors in patients with benign and malignant HPV-associated anogenital lesions.

The levels of type-I and type-II soluble TNF-alpha receptors (sTNF-Rs) were evaluated in sera from patients with various human-papillomavirus-(HPV)-associated benign and malignant anogenital lesions using specific enzyme-linked immunobiological assays. In patients with benign HPV6/11-associated condylomata acuminata, the levels of sTNF-RI were significantly increased, while sTNF-RII were in normal range. Both types of sTNF-Rs were in normal range in patients with benign HPV16-associated grade-I/II and grade-III cervical intra-epithelial neoplasia. However, their levels were significantly increased in patients with HPV16/18-associated squamous cervical cancer and anogenital Bowen's carcinoma. Sera from patients with condylomata acuminata and anogenital carcinomas displayed significantly increased TNF-alpha-inhibitory activity, as revealed by L929 cell-cytotoxicity assay. Increased serum TNF-alpha-inhibitory activity correlated with higher levels of sTNF-Rs. Furthermore, this inhibitory activity could be specifically abrogated by htr9 and utr1 monoclonal antibodies recognizing TNF-RI and TNF-RII respectively. Our results strongly suggest that serum sTNF-Rs may protect tumor cells from cytotoxic/cytostatic effects of locally released TNF-alpha, and that elevated levels of circulating sTNF-Rs may facilitate the growth of HPV-associated anogenital lesions.

Adolescent↗
PubMed1995

Atrophoderma Pasini-Pierini is a primary atrophic abortive morphea.

BACKGROUND: There are divergent opinions whether atrophoderma Pasini-Pierini (APP) is a nosologic entity or a primary atrophic morphea. OBJECTIVE: Since usually single cases are reported without a long-term follow-up the present study was performed in order to elucidate the natural history of the disorder. METHODS: We followed a large series of 139 patients, 91 adults and 48 children, for 4-30 years (mean over 10 years). RESULTS: APP was found to be 6 times more frequent in females and not uncommon in children (10% of our series of localized scleroderma). At some time during the follow-up period, indurations appeared in the central parts of the lesions in 17% of the patients, and in 22% they coexisted with morphea plaques outside the atrophies. The histological pattern was similar to morphea at the stage of atrophy. No case developed full-blown morphea. CONCLUSION: APP appears to be an abortive morphea, in which the indurations failed to develop. The differentiation from morphea is of practical importance because of different management and prognosis.

Adult↗
PubMed1995

Epidermodysplasia verruciformis as a model of human papillomavirus-induced genetic cancer of the skin.

BACKGROUND: Epidermodysplasia verruciformis is a rare lifelong disease that has raised an enormous interest since it is a model of cutaneous genetic cancer induced by specific human papillomaviruses. OBSERVATIONS: The interacting immunogenetic and environmental factors, especially UV irradiation, result in the inability of the patients' immune system to respond to epidermodysplasia verruciformis-specific human papillomaviruses. The local immunosuppression is an effect, at least in part, of the overproduction of tumor necrosis factor alpha and transforming growth factor beta1 and of the excessive formation of cis-urocanic acid. CONCLUSIONS: Epidermodysplasia verruciformis is a model not only of cutaneous viral oncogenesis but also of local defense mechanisms in the progression of human papillomavirus-associated cancers.

Epidermodysplasia Verruciformis↗
PubMed1994

Unusual acantholytic bullous dermatosis associated with neoplasia and IgG and IgA antibodies against bovine desmocollins I and II.

There are unusual cases of pemphigus that have antibodies nonreactive with either pemphigus vulgaris or pemphigus foliaceus antigens. We describe a patient with an acantholytic bullous dermatosis and lung cancer with intercellular IgG and IgA antibodies that differed in specificity from those of pemphigus vulgaris and pemphigus foliaceus and reacted with bovine desmocollins I and II and recombinant desmocollin protein.

Adult↗
PubMed1994

[Diagnostic yield of electrophysiological and immunological studies in inflammatory myopathies].

The aim of the study was to estimate the value of the immune markers in defining distinct subsets of inflammatory myopathies. The series of 76 patients was divided, on the basis of the clinical data, into 5 groups (polymyositis (PM), dermatomyositis (DM), scleromyositis (Scm), mixed connective tissue disease (MCTD), unclassified). In all cases detailed clinical, electrophysiological (concentric needle electromyography (CNEMG) and skin sympathetic response (SSR)) were performed as well as immunologic studies: the anti Mi-2, anti RNP, PM-Scl, Jo-1. The findings indicate that immune markers have a diagnostic value in differentiating the defined subsets of patients different in respect of course, prognosis and therapeutic indications. The authors stressed the value of the Jo-1 antibody in detecting the subset of polymyositis with coexisting interstitial lung disease. Electrophysiological data do not differentiate the groups of patients with different clinical syndrome and different immune markers. EMG results seem however to be useful in monitoring the course of the disease and response to the therapy.

Adult↗
PubMed1991

Childhood scleromyositis: an overlap syndrome associated with PM-Scl antibody.

Fourteen patients with childhood scleromyositis followed from 1 to more than 10 years experienced concomitant sclerodermoid and dermatomyositis features, variably expressed at one time or another during the course of the disease. The most characteristic features were myalgia-myositis, arthralgia-arthritis, puffy, atrophic, sclerotic fingers, and Raynaud's phenomenon. This overlap syndrome was the most frequent sclerodermoid condition in children, differing from both systemic scleroderma and dermatomyositis. The course of the disease was protracted and rather benign, and PM-Scl antibody was an important diagnostic and prognostic marker. We present criteria for diagnosis of scleromyositis and its differentiation from systemic scleroderma, dermatomyositis, and Sharp overlap syndrome.

Autoantibodies↗
PubMed1991

Immunologic markers of systemic scleroderma in children.

This study was performed on seven children with systemic scleroderma, three with the diffuse and four with the limited type. All three patients with diffuse scleroderma had high titers of clumpy pattern antinucleolar antibody on HEp-2 cells. The course of the disease was severe, and two children died. Four children with limited scleroderma had mild disease, and Scl-70 antibody, an immunologic marker that in adults is associated mostly with diffuse scleroderma. In one child Scl-70 antibody and anticentromere antibody coexisted, although previously the two were believed to be mutually exclusive. This study shows that limited scleroderma of childhood with slight cutaneous involvement may be associated with the Scl-70 marker. The findings in 10 adults in whom Raynaud's phenomenon developed in childhood and indurations appeared some years later, point to the significance of careful observation of these children, with repeated testing for immunologic markers of SSc. An important new finding is the association of different types of systemic sclerodermas with specific immunologic markers.

Adolescent↗
PubMed1991

Sympathetic skin response in scleroderma.

Examination of the sympathetic skin response (SSR) was performed in 32 patients with systemic sclerosis, morphea and mixed connective tissue disease displaying scleroderma-like features. The control group consisted of 26 healthy subjects and 12 patients with other skin diseases and asymmetrical cutaneous changes. Right and left median and tibial nerves were stimulated successively and the responses were recorded from the palms and soles simultaneously. SSR abnormalities (delayed latency, decrease and/or asymmetry of amplitude, absent response) were observed in 68.8% of the patients, most frequently in linear scleroderma. An amplitude asymmetry of the responses from upper extremities was the most characteristic pattern of abnormalities. There was no correlation between the SSR and the localization, degree and character (inclurated oedema, atrophy, sclerosis) of skin changes, the duration of the disease, symptoms of the disorder of the autonomic nervous system symptoms (vasomotor and/or sudomotor) and the changes in capillaroscopy. All patients with slow motor conduction and sensor conduction velocities (MCV and SCV) had lower SSR amplitude and the patients with prolonged skin sensory chronaxy had more often delayed latency. The results revealed presence of disturbances of the autonomic nervous system in all varieties of scleroderma, both systemic and localized forms, even without any other signs of autonomic dysfunction.

Adolescent↗
PubMed1991

Immunological markers of the subsets of systemic scleroderma and its overlap.

The studies performed repeatedly in 298 cases of systemic scleroderma (SSc) disclosed high prevalence of antinuclear antibody (in about 95% of the cases) with the use of HEp-2 cells as the substrate for indirect immunofluorescence method. Anticentromere antibody (ACA) was found in 25% of acrosclerosis cases, but in 50% of patients with cutaneous involvement limited to the digits, i.e. almost without cutaneous indurations. The visceral involvement was comparable in cases positive and negative for ACA, and a follow-up of these patients showed that the course, although protracted, was not always mild as previously believed. Scl 70 antibody proved to be a most characteristic marker of SSc, appearing in about 87% of diffuse variety and in above 50% of acrosclerosis cases. The most important finding was the detection of the coexistence of ACA and Scl 70 antibody in 10 of 180 cases studied by three methods: indirect immunofluorescence, double immunodiffusion, (for Scl 70) and immunoblotting with recombinant kinetochore antigen for ACA and recombinant Topoisomerase I antigen for Scl 70 antibody. These antibodies, believed to be mutually exclusive, are most often missed due to masking effect on HEp-2 cells by the stronger antibody, usually Scl 70. Its detection proved to be possible by evaluation of different serum dilutions and several cell mitoses, and confirmed by immunoblotting with recombinant antigens. Of practical significance are the clinical correlations between Pm-Scl antibodies, showing homogeneous nucleolar staining on HEp-2 cells, and overlap or atypical SSc cases with concomitant muscle involvement. This antibody was found to have a surprisingly high association with atypical SSc or scleromyositis of children.

Antibodies, Antinuclear↗
PubMed1990

Antikinetochore and antitopoisomerase I antibodies in systemic scleroderma: comparative study using immunoblotted recombinant antigens, immunofluorescence, and double immunodiffusion.

In 135 patients with systemic scleroderma, we compared three different methods to determine antinuclear autoantibody (ANA) specificity: indirect immunofluorescence, double immunodiffusion, and, employing recombinant antigens, immunoblotting using both marker autoantigens of this disease. A characteristic Scl-70 antibody pattern was found on HEp-2 cells in 83.8% of the patients, double immunodiffusion was positive for the Scl-70 antibodies in 81.9%, and immunoblot with the recombinant topoisomerase I (Topo I) was positive in 71% of the patients. For the centromere autoantibodies we found a high concordance between the anticentromere antibody (ACA) pattern on HEp-2 cells (27 patients positive) and the detection of recombinant kinetochore in immunoblotting (26 patients positive). The three testing techniques gave comparable results, except that the Topo I recombinant antigen used in immunoblotting reacted strongly with fewer than expected of the known Scl-70-positive sera. However, a method using recombinant antigens expressing all epitopes (rather than one of the epitopes of Topo I) will undoubtedly become the method of choice for detecting antibodies in systemic scleroderma. Using the immunoblotting technique with the recombinant antigens we detected in four patients antibodies against both Topo I and kinetochore. More severe symptoms of systemic scleroderma were found in patients who had both antibodies. The combined presence of both marker autoantibodies is therefore not as rare as previously reported and may predict severe disease.

Adolescent↗
PubMed1990

Stratum corneum antibodies detected by hemagglutination are not directed against keratin intermediate filaments.

Autoantibodies to stratum corneum (SC) occur in virtually all normal adult human sera. These antibodies may be directed against various antigens of the SC. They have been detected by indirect immunofluorescence, passive hemagglutination (HA), immune adherence, and most recently by enzyme immunoassay and immunoblot methods. The purpose of our study was to examine whether antibodies to SC antigens as detected by passive HA are similar to the keratin intermediate filament (KIF) reactive antibodies. SC antigen preparation was prepared from psoriatic scales by the trypsin-phenol-water (TPW) extraction method. KIFs were prepared by 8 M urea extraction of normal callus or psoriatic scales. The anti-SC antibody titers of normal human sera were determined by passive HA before and after absorption with TPW-SC antigen preparation and upon absorption with KIFs. Similarly, titers of anti-KIF antibodies were determined on absorbed and unabsorbed sera by immunoblot assay. The results of this study indicate that the absorption of the sera with KIFs did not affect the titer of antibodies to TPW-extractable SC antigens whereas the titer of KIF antibodies dropped. KIF-reactive antibodies, on the other hand, were not affected by absorption with TPW-SC antigen, whereas the latter absorbed out the corresponding reactive antibodies. These results indicate that antibodies directed against SC antigen are different from the KIF-reactive antibodies.

Amino Acids↗