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Biomedical subjects

F Wojnarowska

Publications and source records attributed to F Wojnarowska.

At least 217 records · Page 12Linked to original sources

The localization of the bullous pemphigoid and cicatricial pemphigoid antigens: direct and indirect immunofluorescence of suction blisters.

The location of in vivo bound immunoreactants was studied in 37 patients with subepidermal blistering diseases by direct immunofluorescence (IMF) on suction blisters taken from uninvolved forearm skin. The patients studied included 18 with bullous pemphigoid (BP), nine with cicatricial pemphigoid (CP), three with acquired epidermolysis bullosa (EBA) and 7 hybrid cases. The patterns of IMF in the suction blisters were: BP, epidermal 1, dermal 1, combined 4, negative 12; CP, epidermal 1, dermal 2, negative 6; EBA, dermal 2, negative 1; and 'hybrid' patients, epidermal 3, negative 4. The different patterns of suction blister staining could not be correlated with the clinical features of the patients in respect of mucous membrane involvement, scars or milia or a history of skin fragility. Both BP and CP are probably heterogeneous in respect of their antigen specificity, and the two diseases cannot reliably be distinguished by the patterns of direct IMF on suction blisters. In addition, some individual patients with BP have more than one target antigen as indicated by a combined pattern of suction blister fluorescence. The lack of correlation between the pattern of suction blister fluorescence and the clinical features suggests that factors other than antigen specificity determine the clinical expression of subepidermal blistering diseases.

Adult↗

Dapsone as first line therapy for bullous pemphigoid.

In an open study, a total of 18 patients with proven bullous pemphigoid (16 new patients and two in relapse) were treated with a trial of dapsone (17 patients) or sulphonimide (one patient). Overall, eight patients were controlled on one of these agents as the sole treatment (44%). Six patients had a partial, but inadequate response, while four did not respond. The responses to the sulpha drugs were generally rapid, i.e. within 2 weeks, and the maximum doses used in any patient were 100 mg/day dapsone and 1.5 g/day of sulphapyridine or sulphamethoxypyridazine. Significant side-effects to dapsone occurred in six of the 17 patients. A trial of a sulphone or sulphonamide drug is warranted in bullous pemphigoid both as an initial treatment or in the treatment of relapse, particularly when there is a contraindication to the use of corticosteroids. Side-effects are common and therapy needs to be closely monitored.

Aged↗

Mucosal involvement in systemic and chronic cutaneous lupus erythematosus.

The prevalence and pattern of mucosal involvement in 121 patients with lupus erythematosus (LE) was investigated. Fifty-three patients had systemic LE (SLE) and 68 patients had chronic cutaneous LE (CCLE). Twenty-one per cent (11/53) of patients with SLE and 24% (16/68) of patients with CCLE had signs of mucosal involvement, but the pattern of involvement differed in the two groups. Nasal mucosal lesions were a feature in 2% (I/53) of patients with SLE and 9% (6/68) of patients with CCLE. Hyperkeratotic lichen planus-like plaques on buccal mucosa and the palate occurred in 9% (6/68) of patients with CCLE and 4% (2/53) of patients with SLE. Episcleritis occurred in 9% (5/53) of patients with SLE and not seen in CCLE. Erythematous plaques on the lower eyelids were present in 6% (4/68) of patients with CCLE and these were associated with conjunctival scarring in two patients. Vulval lesions were present in 5% (2/42) of female patients with CCLE. Oral plaques may occur when the disease is relatively quiescent elsewhere. The prevalence of mucosal involvement in lupus is underestimated as the lesions may be asymptomatic.

Adult↗

The lupus band test in oral mucosa, conjunctiva and skin.

The prevalence and clinical significance of subepithelial immunoglobulin and complement deposition (the lupus band) were examined in the uninvolved sun-protected skin of the forearm, the uninvolved sun-protected lip mucosa and sun-protected bulbar conjunctival mucosa in systemic lupus erythematosus (SLE) and chronic cutaneous lupus erythematosus (CCLE). In SLE, linear deposition of an immunoreactant at the BMZ was detected in 32% (6/19) of skin biopsies; 21% (4/19) of lip mucosal biopsies and 42% (5/12) of conjunctival biopsies. There was no significant difference in the sensitivity of the test at different sites in SLE and no correlation between a positive test in skin, lip or conjunctiva and clinical mucosal involvement. In CCLE, linear deposition of an immunoreactant at the BMZ was found in 3% (1/32) of skin biopsies; 3% (1/29) of lip mucosal biopsies and 50% (10/20) of conjunctiva and clinical mucosal involvement. In the conjunctiva, IgG was present in all but one of the biopsies and was the only immunoreactant in 90% (9/10) of positive CCLE biopsies and 60% (3/5) of positive SLE biopsies. In lupus erythematosus immunoreactants may be deposited in the basement membrane zone beneath non-keratinizing mucosal surfaces of the lip and the eye as well as the skin. In CCLE, the test may be positive in conjunctiva when skin and lip are negative.

Adolescent↗

HLA type in bullous pemphigoid, cicatricial pemphigoid and linear IgA disease.

In a study of 32 patients with bullous pemphigoid (BP), 16 patients with cicatricial pemphigoid (CP) and 10 patients with linear IgA disease (LAD) no significant association was found between these diseases and HLA type of the A, B, C and DR loci. In order to determine whether HLA type modified the clinical expression of these subepidermal diseases, the results were analysed for any association with mucosal involvement, the presence of scarring or the occurrence of a circulating anti-basement membrane zone antibody. No significant associations were found.

Adolescent↗

Conjunctival involvement in cicatricial and bullous pemphigoid: a clinical and immunopathological study.

Patients with bullous pemphigoid were found to have significant ocular abnormalities. In a group of 18 patients one had conjunctival shrinkage, and 11 of 15 (73%) had positive linear direct immunofluorescence on conjunctival biopsy from a clinically uninvolved site. Our ocular findings in a group of 14 with cicatricial pemphigoid are also reported and compared with those from a control group of 20. Our findings suggest there is overlap between the pemphigoid groups and raise further questions about the pathogenicity of immunoreactants within the basement membrane zone. Bulbar conjunctival biopsy was simple and well tolerated, and the rate of immunofluorescence positivity of conjunctiva was twice that of skin in both pemphigoid groups.

Aged↗

Spironolactone is an effective and well tolerated systemic antiandrogen therapy for Hirsute women.

We treated 22 hirsute women with spironolactone in an open trial to determine whether it caused objective changes in hair growth. Among them, 18 women completed 12 months therapy with 200 mg spironolactone daily. During this period, the mean daily linear growth rates of hair on the face, abdomen, and thigh were reduced to 66% (P less than 0.001), 75% (P less than 0.01), and 72% (P less than 0.001) of their pretreatment values. The mean hair shaft diameters were reduced to 83% (P less than 0.01) on the face, 88% (P less than 0.001) on the arm, 74% (P less than 0.01) on the abdomen, and 80% (P less than 0.001) on the thigh. Daily hair volume production was calculated from the diameter and daily growth rate; it was reduced to 60% (P less than 0.01) on the face, 52% (P less than 0.01) on the arm, 34% (P less than 0.001) on the abdomen, and 48% (P less than 0.001) on the thigh. Six of the 18 women who completed the study developed midcycle vaginal bleeding, and 3 women had previously irregular menstrual cycles regulated. We conclude that spironolactone is effective and well tolerated for hirsute women.

Adult↗

Pemphigoid and ulcerative colitis.

We report eight patients with ulcerative colitis who have subsequently developed pemphigoid. Four patients were investigated to determine the pattern of pemphigoid antibody staining on whole and chemically split skin and oral mucosa to distinguish between pemphigoid and epidermolysis bullosa acquisita. The findings were suggestive of pemphigoid with a predominantly epidermal pattern. Three patients were studied for the presence of antibodies against the colon (which were absent). The relationship between pemphigoid and ulcerative colitis is discussed in relation to a case-control study.

Adult↗

Chronic bullous disease of childhood, childhood cicatricial pemphigoid, and linear IgA disease of adults. A comparative study demonstrating clinical and immunopathologic overlap.

Linear IgA disease of adults, chronic bullous disease of childhood, and the rare childhood cicatricial pemphigoid currently are regarded as separate clinical entities despite their many shared features. All are sulfone-responsive subepidermal bullous diseases associated with linear IgA deposition at the basement membrane zone. In this paper we present a long-term study of 25 cases of adult linear IgA disease, 25 cases of chronic bullous disease of childhood, and four cases of childhood cicatricial pemphigoid, which has revealed further similarities among all three groups. The morphology and distribution of the cutaneous and mucosal lesions were similar; mucosal involvement was present in 80% of patients with adult linear IgA disease, 64% of those with chronic bullous disease of childhood, and 100% of those with childhood cicatricial pemphigoid, and ocular scarring affected patients in all groups. Remission occurred in 64% of those with chronic bullous disease of childhood (the disease was active in 12% after puberty), 48% of those with adult linear IgA disease, and in no cases of childhood cicatricial pemphigoid. HLA B8 and circulating IgA anti-basement membrane zone antibody were more common in chronic bullous disease of childhood than adult linear IgA disease. There were no absolute differences among the three groups, and we suggest that adult linear IgA disease, chronic bullous disease of childhood, and childhood cicatricial pemphigoid are the same disease, with childhood cicatricial pemphigoid being a more severe form of chronic bullous disease of childhood.

Adolescent↗

Mucosal involvement in bullous and cicatricial pemphigoid. A clinical and immunopathological study.

Thirty-six patients with bullous pemphigoid (BP) and 15 with cicatricial pemphigoid (CP) were studied for evidence of mucosal involvement both clinically and by direct immunofluorescence. Twenty-one patients with BP and all those with CP had lesions of the mucous membranes. Eighteen patients with BP and 14 patients with CP had involvement of the oral mucosa. Lesions of the eyes, nose, pharynx, vulva and urethra also occurred in both groups. The lesions tended to be more widespread in CP patients. Twenty-one patients with BP and 14 with CP were examined by an ophthalmologist. Fine scarring of the tarsal conjunctiva was found in both groups, but also in elderly controls. However, symblepharon occurred in one patient with BP as well as in three patients with CP, but was not seen in a group of 20 controls. Direct immunofluorescence (IF) studies showed linear deposition of IgG or C3 or both in the basement membrane zone of the skin, oral mucosa and conjunctival mucosa in both groups. The frequency of positive immunofluorescence was higher in the conjunctiva than in the lip mucosa or skin in both BP and CP patients. The significance of these findings and the relationship between BP and CP is discussed.

Aged↗

The use of chemically split tissue in the detection of circulating anti-basement membrane zone antibodies in bullous pemphigoid and cicatricial pemphigoid.

Human skin and mucous membranes were used to detect circulating auto-antibodies by indirect immunofluorescence in 20 patients with bullous pemphigoid and eight with cicatricial pemphigoid. The tissue substrate was used intact and after chemical separation through the basement membrane zone (BMZ) by incubation with I M NaCl. Chemically split skin and oral mucosa provided a more sensitive assay for demonstrating circulating anti BMZ antibodies. Use of a battery of substrates increased the number of positives in bullous pemphigoid from 30% detected on monkey oesophagus to 100% (tissue battery). In cicatricial pemphigoid there was an increase in the proportion of positive sera from 13% (monkey oesophagus) to 88% (tissue battery). In addition, a different class of antibody was frequently detected on split tissue substrate suggesting that new antigens are exposed by this procedure.

Aged↗

Distribution of the antigen in adult linear IgA disease and chronic bullous dermatosis of childhood suggests that it is a single and unique antigen.

The tissue distribution and species expression of the antigens in adult linear IgA disease (LAD) and chronic bullous dermatosis of childhood (CBDC) was studied and compared with those of bullous pemphigoid (BP) and epidermolysis bullosa acquisita (EBA) antigens. Positive sera from five adult LAD patients, 15 CBDC patients, three BP patients and two EBA patients were studied, using skin and mucosal tissues of humans and animals as substrates. The tissue distribution and phylogenetic distribution of adult LAD antigen and CBDC antigen were the same, suggesting that they are identical diseases. The antigens were both demonstrable only in stratified squamous epithelia and amnion of mammalian species. They were absent from transitional and columnar epithelium. The EBA antigen was not expressed in pig tissues or human placenta, unlike adult LAD and CBDC antigens. Expression of adult LAD, CBDC and BP antigens was observed in all tissues except bladder, which lacked LAD and CBDC antigens. These results suggest that the antigens in adult LAD and CBDC are the same and differ from EP and EBA antigens.

Animals↗

Expression of a pemphigoid gestationis-related antigen by human placenta.

We studied the antigen binding of sera from 25 patients with pemphigoid gestationis (PG) using skin and amnion as substrates in an indirect immunofluorescence technique. In addition, skin was studied after prior incubation with I M NaCl which allows BMZ cleavage through the lamina lucida. Linear BMZ staining was obtained with sera from patients with PG using human amnion as substrate. This was seen with both full-term and second trimester placentae, but not with first trimester placentae. In NaCl incubated skin all sera bound to the epidermal aspect of the separated tissue. The pattern of staining in skin, chemically split skin and amnion was identical to that previously seen with the sera of patients with bullous pemphigoid suggesting that the antigen against which circulating anti-BMZ antigens are directed may be the same in the two conditions.

Amnion↗

Acanthosis nigricans, insulin resistance and cutaneous virilism.

Thirteen patients with the syndrome of acanthosis nigricans and insulin resistance are described. They all had a combination of dermatoses related to hyperandrogenism (cutaneous virilism): hirsuties (II), acne vulgaris (6), hidradenitis suppurativa (5) and androgenic alopecia (4). In addition, 9 out of 13 had keratosis pilaris. The patients had raised fasting plasma insulin levels compared with matched normal controls (P less than 0.01) and increased insulin resistance (P less than 0.02). Insulin resistance correlated with total serum testosterone (rs = 0.65; P less than 0.02).

Acanthosis Nigricans↗

A clinicopathological study of mucosal involvement in linear IgA disease.

Mucosal involvement in linear IgA disease was assessed clinically and immunologically using direct and indirect immunofluorescence (IF) techniques. There was clinical evidence of oral mucosal involvement in all 10 patients examined and conjunctival diseases in six. Direct IF findings correlated well with clinical oral disease, with all patients demonstrating linear IgA deposits in the basement membrane zone of oral mucosa. However, this was not true of conjunctiva where no linear IgA could be demonstrated. Conjunctiva did provide a good substrate for indirect immunofluorescence using patients' sera, and showed that five of the 10 patients had circulating anti-basement membrane zone IgA.

Adolescent↗