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F Wojnarowska

Publications and source records attributed to F Wojnarowska.

At least 181 records · Page 10Linked to original sources
PubMed1992

Variation in the deposition of the antibodies at different anatomical sites in linear IgA disease of adults and chronic bullous disease of childhood.

The diagnosis of linear IgA disease of adults (LAD) and chronic bullous disease of childhood (CBDC) relies upon finding a linear band of IgA at the basement membrane zone on direct immunofluorescence. This study examines the regional variation in antigen expression in the skin of affected individuals. Direct immunofluorescence was performed on biopsies from four different sites in 17 patients with these diseases. In two patients a biopsy from the volar surface of the forearm was negative, but other sites were positive; in the remaining patients there was no variation in antibody expression with site. It is therefore recommended that, if a single diagnostic biopsy is to be taken, the volar surface of the forearm is avoided.

Adolescent↗
PubMed1992

Bullous and haemorrhagic lichen sclerosus with scalp involvement.

We describe a patient who developed a generalized blistering eruption due to lichen sclerosus and who was observed to have scalp involvement. Both are unusual manifestations of this disease which merit consideration. Lichen sclerosus is an uncommon disease that most frequently affects the external genitalia of perimenopausal women. The aetiology is unknown. Approximately 20% of affected patients have extragenital lesions that present as small, ivory, shiny round macules or papules that later become atrophic; extragenital lesions are generally asymptomatic. Bullous and haemorrhagic forms may occur but these are generally localized and reports of extensive or generalized involvement are rare. We describe an elderly woman with generalized bullous lichen sclerosus. As an incidental finding, she was observed to have lichen sclerosus affecting her scalp. This has rarely been described and it would appear that she is the third reported case of scalp involvement.

Aged↗
PubMed1992

Vitamin E and discoid lupus erythematosus.

We treated seven patients with discoid lupus erythematosus (DLE) with Vitamin E in an oral dose of 400 mg three times per day for 12 weeks. All other systemic and topical treatments were discontinued 1 month before initiation of the trial. The drug was then stopped and follow-up continued for at least another 4 weeks. No patient showed clearing of lesions. The trial was conducted during summer, when DLE is likely to be most active. There was no deterioration in any patient. No side effects were noted.

Administration, Oral↗
PubMed1992

Absence of expression of class II major histocompatibility complex determinants on keratinocytes in bullous pemphigoid.

Aberrant expression of class II products of the major histocompatibility complex (HLA-D locus antigens) occurs on keratinocytes in several inflammatory dermatoses and on thyroid epithelial cells in autoimmune thyroiditis. The functional significance of aberrant HLA-D expression is unclear but it has been hypothesized that epithelial cells bearing these determinants may act as antigen-presenting cells for autoantigens. The aim of the present study was to investigate the pathogenesis of bullous pemphigoid using immunohistochemical methods to determine whether the HLA-D locus antigens are aberrantly expressed on keratinocytes in lesional and uninvolved skin. A panel of monoclonal antibodies to each of the HLA-D subregions (DR, DP and DQ) and to Langerhans cells was used. Epidermal expression of the HLA-D locus antigens was similar in patients and controls, and there was no significant increase in expression in lesional skin compared with uninvolved skin in six out of nine patients. In three out of nine patients slight enhancement of epidermal HLA-D expression in lesional epidermis corresponded to increased Langerhans cells rather than expression on keratinocytes. HLA-D locus antigens are absent from keratinocytes in bullous pemphigoid skin and aberrant expression of these determinants cannot therefore be implicated in antigen presentation.

HLA-D Antigens↗
PubMed1991

Association of autoimmunity and cicatricial pemphigoid: is there an immunogenetic basis?

A group of 34 patients with cicatricial pemphigoid was investigated for the presence of autoimmune disorders. Thirty-two percent of patients had autoimmune disease compared with 7% in the control population, a highly significant difference (p less than 0.002). Circulating autoantibodies were also significantly more common in patients (p less than 0.05). Twenty-four patients with cicatricial pemphigoid were typed for HLA. A statistically significant increase in the frequency of DR4 and DQw3 antigens was observed in these patients. These findings suggest a possible genetic basis for the autoimmune association and predisposition for development of cicatricial pemphigoid.

Adolescent↗
PubMed1991

Identification of the target antigen in chronic bullous disease of childhood and linear IgA disease of adults.

Disease-associated autoantibodies to basement membrane proteins have been used to characterize structural components of the epidermal basement membrane such as bullous pemphigoid (BP) antigen and epidermolysis bullosa acquisita (EBA) antigen (type VII collagen). The autoimmune bullous diseases characterized by IgA autoantibodies to the basement membrane zone (BMZ), i.e. linear IgA disease of adults (LAD) and chronic bullous disease of childhood (CBDC) may have circulating antibodies. Previous studies of tissue distribution and ultrastructural binding have suggested that the LAD and CBDC antigens are similar, if not identical, and differ from the target antigens of the other bullous diseases. We present the molecular characterization of the LAD/CBDC antigens by Western blotting of a large series of antisera. Seven of 33 sera (21%) were positive on immunoblotting and bound to the same antigen which has a molecular weight (MW) of 285 kDa. Using both defined polyclonal antisera to BP and LH 7.2 monoclonal antibody to type VII collagen (carboxy terminal) we have shown that the LAD and CBDC antisera both bind to an identical molecular weight protein which clearly differs from both the BP and EBA (type VII collagen) antigens. Although detectable in dermal tissue extracts like EBA, the MW of 285 kDa is heavier than type VII collagen (250 kDa, in our system, using non-collagenous standards). This study confirms the identity of LAD and CBDC antigens to be the same and to differ from previously described basement membrane proteins.

Adolescent↗
PubMed1991

Hailey-Hailey disease: a widespread abnormality of cell adhesion.

Suction has been used to investigate cell adhesion in clinically normal skin in Hailey-Hailey disease. We have demonstrated that there is a widespread subclinical abnormality in keratinocyte adhesion in this disease. There may be a synthesis of functionally deficient adhesion junctions, increased breakdown of adhesion junctions or abnormalities in other adhesion proteins in the epidermis in Hailey-Hailey disease. The findings contrast with those in Darier's disease in which abnormal cell adhesion was only demonstrable in clinically involved skin.

Adult↗
PubMed1991

The treatment of vulval lichen sclerosus with a very potent topical steroid (clobetasol propionate 0.05%) cream.

The clinical and histological response to 12 weeks of treatment with a very potent topical fluorinated steroid was studied in 15 patients with vulval lichen sclerosus (LS) who were treated with twice daily applications of clobetasol propionate 0.05% cream (Dermovate, Glaxo U.K.). Thirteen patients completed the study and all showed a marked clinical improvement. Histological measurements of skin biopsies taken before and after treatment showed a significant reduction in the characteristic features of LS. One patient developed contact sensitivity to clobetasol propionate. There was no evidence of infection or skin atrophy during the study. Patients completing the study have been followed up for up to 22 months and have been maintained in remission with moderately potent topical steroids which had previously been ineffective.

Administration, Topical↗
PubMed1991

Lichen planus pemphigoides: its relationship to bullous pemphigoid.

Clinical and immunopathological studies of three patients with lichen planus pemphigoides (LPP) were carried out to investigate the relationship between LPP and bullous pemphigoid (BP) and to determine whether the antigen in LPP is the classical BP antigen. LPP is usually considered to be the coexistence of lichen planus with BP. The bullae in LPP were subepidermal and indistinguishable from BP. Indirect immunofluorescence demonstrated antibody binding to the epidermal surface of 1 M NaCl-split skin and mucosae, as in BP. The tissue distribution of the LPP antigen mirrored the distribution of BP in stratified squamous epithelia but was absent from transitional epithelia (pig bladder). Immunoelectron microscopy, both direct (two cases) and indirect (one case), showed binding to the lamina lucida as with BP antigen. Western blotting of epidermal extracts using the patients' sera showed that instead of reacting with the classical bullous pemphigoid antigen (220 kDa in our series), the antisera reacted with a unique band of 200 kDa in addition to the band of 180 kDa found as a minor antigen in bullous pemphigoid, but more commonly in pemphigoid gestationis. The relationship between these antigens awaits molecular characterization. These findings suggest that the target antigen in LPP may be unique.

Adult↗
PubMed1991

The clinical expression of bullous pemphigoid is not determined by the specificity of the target antigen.

The major bullous pemphigoid (BP) antigen is a 220-240-kDa polypeptide, although some BP sera recognize bands of 180-200 kDa or lower molecular weight. We have investigated to what extent this heterogeneity of the target antigen accounts for the clinical diversity of BP. Immunoblotting studies against extracts of salt-separated epidermis were performed on sera from 39 patients with BP. The blotting patters obtained were correlated with the clinical findings, with particular reference to prodromal itching, lesion morphology and severity, mucosal involvement, presence of milia, dapsone responsiveness and disease duration. The results confirm that the major BP antigen is a 220-kDa polypeptide, and that the 180-kDa polypeptide is a second and sometimes the sole BP antigen identified in immunoblots. Rarely, multiple bands of lower molecular weight were found. There was no correlation between the pattern of BP antigens detected in immunoblots and the clinical presentation and course of BP. There was considerable clinical diversity even among the nine patients showing specificity for a single 220-kDa target antigen. Although two patients with a single 180-kDa antigen specificity had a disease of unusually long duration, factors other than antigen specificity must determine the clinical expression of BP.

Aged↗
PubMed1991

Chronic bullous disease of childhood with oral mucosal scarring.

Chronic bullous disease of childhood (CBDC) is an acquired subepidermal bullous disease; oral involvement occurs with mouth ulcers and blisters in 57% of patients. The condition usually remits before puberty but persistence of the disease in adulthood is recognized. We report a case with severe oral scarring similar to that described in patients with adult linear IgA disease. To our knowledge this has not been previously described.

Child↗
PubMed1991

Bullous eruption of SLE--a case report and investigation of the relationship of anti-basement-membrane-zone antibodies to blistering.

We describe the clinical and immunopathological findings in a patient with a bullous eruption and systemic lupus erythematosus (SLE). The bullous eruption preceded a dramatic flare of the SLE with a rise in anticardiolipin antibodies and life-threatening cardiac vasculitis. The clinical and histological findings were similar to those described in the classic bullous eruption of SLE but, unlike previous cases, IgG anti-basement-membrane-zone (anti-BMZ) antibodies were detected on the epidermal as well as the dermal side of the split in chemically separated human skin. We screened the sera of another eight patients with SLE and 10 patients with chronic cutaneous lupus erythematosus (CCLE) without evidence of systemic involvement for the presence of anti-BMZ antibodies and demonstrated that these were present in a low titre in a further two SLE patients neither of whom had a history of blistering. Once more there was binding to both sides of the split. We conclude that although there may be low titres of antibodies to several BMZ antigens in patients with SLE, these are not always associated with blistering and their role in the initiation or perpetuation of cutaneous disease is uncertain.

Autoantibodies↗
PubMed1991

Oesophageal webs preceding carcinoma and rupture of the oesophagus in cicatricial pemphigoid.

A case in which oesophageal webs preceded the development of carcinoma and rupture of the oesophagus in a 77-year-old woman with cicatricial pemphigoid is reported. Oesophageal webs in cicatricial pemphigoid have been reported but are rare. Clinical, histological, radiological and post-mortem features are described. Western immunoblotting of serum demonstrated a 180-kDa antigen which comprises one of the antigens reported in cicatricial pemphigoid.

Aged↗
PubMed1991

Cyproterone acetate for severe hirsutism: results of a double-blind dose-ranging study.

OBJECTIVE: The objective was to determine if cyproterone acetate (CPA) therapy for hirsute women demonstrated a dose response. DESIGN: A double-blind dose-ranging study of the effect of 3 doses of cyproterone acetate for a period of 12 months. PATIENTS: Twenty-one hirsute women received the Dianette contraceptive pill (35 micrograms ethinyl oestradiol + 2 mg CPA, Schering Healthcare, UK), 20 received Dianette plus 20 mg CPA and 19 received Dianette plus 100 mg CPA: supplementary CPA was administered on days 1-10 of the birth control pill cycle as described by Hammerstein. MEASUREMENTS: Hair growth was measured using the clinical scale of Ferriman and Gallwey and by direct measurement of hair shaft diameter and linear growth of hair on the face, forearm, abdomen and thigh. RESULTS: Thirty-eight women completed 12 months therapy, eight withdrew due to side-effects and 14 were lost to follow-up. All three dose schedules produced significant reductions in clinical hair growth scores. This reduction was seen after 6 months with Dianette alone (P less than 0.005) and after 3 months with both the higher doses (P less than 0.01). There were no significant differences between the effect of different doses at any of the three-monthly time points. Hair diameter measurements were reduced by all doses after 12 months: face by 27*, 37, 37%*, forearm 5, 10, 8%*, abdomen 22*, 39*, 33%*, thigh 12, 24*, 30%* (median reductions (*P less than 0.01) for Dianette, D + 20 mg CPA and D + 100 mg CPA respectively). There were no significant reductions in daily linear growth rates. A comparison of the percentage reduction in hair shaft diameter at each site demonstrated no significant difference between doses, although the reductions by the three doses on the forearm, abdomen and thigh suggested a trend towards a dose response. CONCLUSIONS: We conclude that cyproterone acetate 2 mg daily appears to be as effective as higher doses in the therapy of hirsute women.

Adolescent↗
PubMed1991

Lichen planus pemphigoides: a clinicopathological study of nine cases.

Lichen planus pemphigoides is a rare condition characterized by blisters arising on normal or erythematous skin in a patient with concurrent lichen planus. It must be distinguished from bullous lichen planus, in which, as a consequence of severe basal cell hydropic degeneration, blisters arise within lichenoid papules or plaques. We present a clinicopathological study of nine cases of lichen planus pemphigoides, and report histological, immunofluorescent, ultrastructural and immuno-electronmicroscopical observations. We distinguish lichen planus pemphigoides from bullous lichen planus and consider the differential diagnosis. We propose that lichen planus pemphigoides does not represent a homogeneous condition: it may represent a number of bullous dermatoses that develop as a consequence of exposure of different basement membrane antigens following severe damage to the epidermal basement membrane as part of the lichenoid inflammatory process.

Adult↗