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Biomedical subjects

F Wojnarowska

Publications and source records attributed to F Wojnarowska.

At least 163 records · Page 9Linked to original sources
PubMed1994

Pemphigus in Oxford, UK, and New Delhi, India: a comparative study of disease characteristics and HLA antigens.

A study of pemphigus in New Delhi, India, and Oxford, UK, was undertaken including 20 patients in Oxford and 50 in New Delhi. Data included clinical and histological subtypes and socio-economic data; patients were HLA typed. In New Delhi pemphigus vulgaris predominated, but in Oxford pemphigus vulgaris and pemphigus foliaceus have equal prevalence. Disease distribution with sex was the same, but age at onset was significantly lower in New Delhi (p = 0.0019). HLA typing in pemphigus vulgaris patients revealed a significant reduction in HLA-DR2 in New Delhi (p = 0.0008) and Oxford (p = 0.09). A small increase in HLA-DR1 and -DR4 was found in both groups and, in males only, a subtle increase in HLA-DR6 and reduction in HLA-DR3. No differences were found in the class I antigens. Thus there are striking differences in the types of pemphigus between the two populations, yet the genetic predisposition is the same.

Adolescent↗
PubMed1994

Flame figures in bullous pemphigoid.

We report a patient with bullous pemphigoid showing prominent flame figures on skin biopsy. Although flame figures are the histological hallmark of eosinophilic cellulitis, they are occasionally seen in a variety of other dermatoses.

Eosinophils↗
PubMed1993

Bullous pemphigoid and autoimmunity.

BACKGROUND: There are many case reports of bullous pemphigoid (BP) in association with a variety of autoimmune disorders, but no large case controlled studies have been performed. OBJECTIVE: Our purpose was to determine whether there is an increased incidence of autoimmune disorders in patients with BP and whether there is a particular haplotype associated with this. METHODS: A total of 108 patients with BP were studied and compared with a group of 108 age- and sex-matched controls. HLA typing at the A, B, C, and DR loci was performed on 55 of the 108 patients with BP. RESULTS: The difference in incidence of autoimmune disorders between patients and controls was not significant. The haplotypes of patients with BP were similar to those of a locally drawn population. CONCLUSION: There is no increase in the incidence of autoimmune disorders in BP, and no particular haplotype appears to be associated with a predisposition to this condition.

Aged↗
PubMed1993

Bullous pemphigoid and associated autoimmune thrombocytopenia: two case reports.

We describe two patients with bullous pemphigoid in whom autoimmune thrombocytopenia developed. Only one case of bullous pemphigoid associated with autoimmune thrombocytopenia has previously been reported, and in that case, autoimmune hemolytic anemia was also present (Evans' syndrome). However, a wide range of other autoimmune diseases have been described in association with bullous pemphigoid, and this literature is reviewed.

Autoimmune Diseases↗
PubMed1993

An immunohistological study of desmosomal components in pemphigus.

Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are autoimmune diseases in which there is loss of cohesion between keratinocytes (acantholysis) and blistering within the epidermis. PV is characterized by acantholysis predominantly between the epidermal basal cells and suprabasal layers, whereas in PF intraepidermal cleavage is higher in the epidermis. Adhesion between keratinocytes is dependent on the function of transmembrane glycoproteins of the cadherin family present in specialized adhesion junctions, the desmosomes. The pathogenesis of acantholysis in pemphigus is uncertain, but the pemphigus autoantibodies bind to epithelial cadherins. We have used monoclonal antibodies to desmosomal components to investigate their distribution in different forms of pemphigus. Our results show that the localization of desmosomal components is abnormal in intact perilesional epidermis, intact epidermis above the blisters in PV and intact epidermis below the blisters in PF. We suggest that autoantibody binding may have a direct effect on the function of specific epithelial cadherins, but will only cause cell separation where the antigen is the principal adhesion molecule.

Acantholysis↗
PubMed1993

Pemphigus associated with nail dystrophy.

Two patients suffering from pemphigus vulgaris were found to have nail dystrophies which antedated the onset of mucocutaneous lesions by many years. The nail changes improved substantially on treatment of the bullous disease. One patient had nail matrix histology consistent with pemphigus, and both had positive direct immunofluorescence with intercellular IgG in the matrix epithelium, as well as at other body sites. We propose that dystrophic nails, as a non-specific indicator of autoimmune disease, are a genuine and relevant finding in pemphigus.

Aged↗
PubMed1993

Isotretinoin-associated pseudoporphyria.

We present two patients who developed pseudoporphyria whilst taking isotretinoin (Roaccutane). At that time, both patients were receiving other agents which could be incriminated as causing their pseudoporphyria. However, it is significant that these other agents had been longstanding and that the porphyria-like cutaneous changes did not develop until after the introduction of the isotretinoin (Roaccutane). In both cases, withdrawal of the isotretinoin resulted in clinical improvement of their cutaneous signs. The interaction of isotretinoin and another agent, rather than isotretinoin independently, may be involved in the development of pseudoporphyria.

Adult↗
PubMed1993

An immunohistochemical study of the distribution of plasminogen and plasminogen activators in bullous pemphigoid.

Abnormalities of the cutaneous plasminogen/plasminogen activator system have been associated with acantholytic disorders, psoriasis, keratinocytes in culture, and epidermis in healing wounds. The present study was undertaken to investigate the possible role of the plasmin/plasminogen protease system in lesion development in bullous pemphigoid (BP). Using polyclonal antibodies and a fluorescent technique, the immunohistochemical distribution of plasmin/plasminogen, fibrinogen and the plasminogen activators, urokinase (uPA) and tissue plasminogen activator (tPA), were studied in lesional and non-lesional skin from nine BP patients, one with linear IgA disease (LAD) and one with pemphigoid gestationis (PG). The distribution of the proteases was compared with that in normal skin (n = 4) and in suction blisters (n = 2). In normal skin, fibrinogen, tPA and uPA were absent from the epidermis and plasminogen was confined to the basal layer. Uninvolved BP skin was identical to controls. Focal areas of suprabasal plasminogen expression in the region of a blister was seen in 3/9 BP lesions and in 1/2 suction blisters. In 6/9 BP lesions and both uninvolved and lesional LAD and PG skin were identical to controls, and no suprabasal expression of plasminogen was present. These findings suggest that suprabasal plasminogen expression is unlikely to play a fundamental role in the pathogenesis of blister formation in BP as enhanced expression was not present in every case and the finding was not specific to BP, also occurring in a suction blister. Enhanced plasminogen expression rather may be a reflection of the processes of tissue repair.

Humans↗
PubMed1993

IgG subclasses in pemphigus in Indian and UK populations.

The autoimmune blistering disease pemphigus is more common in the Indian subcontinent than in the UK. This study of 19 patients from Oxford, UK and 39 patients from New Delhi, India demonstrates that the incidence of the disease subtypes is different in the two countries. In the UK the commonest subtypes are pemphigus vulgaris and foliaceus with equal prevalence (both eight of 19), but in India pemphigus vulgaris is the most frequent (31 of 39), while pemphigus foliaceus is uncommon (three of 39) and with equal prevalence to the other subtypes. These populations also differ with a younger age at onset in the Indian patients (36.9 India; 52.7 UK) though the sex distribution is the same. Study of the immunopathology shows that the antibodies produced by patients in the two countries do not differ significantly, and are predominantly of the IgG4 subclass. The antibody produced does not vary with the subtype of pemphigus or the age or sex of the patient. Although there are considerable differences between the two groups of patients this difference is not reflected by the subclass of auto-antibody response.

Adult↗
PubMed1993

Suction-induced basal cell cytolysis in the Weber-Cockayne variant of epidermolysis bullosa simplex.

In the Weber-Cockayne form of epidermolysis bullosa simplex (EBS-WC), trauma induces blisters which are confined to the palms and soles. Histologically, basal cell cytolysis is seen. We studied 6 patients with EBS-WC to determine the ultrastructural level at which artificially-induced suction blisters form. Blisters were raised by application of a suction blister cup to uninvolved forearm skin, the cup being connected to a negative pressure of 200 mm of mercury. The blisters were biopsied and examined by light and electron microscopy. On light microscopy, all biopsies showed marked vacuolization of keratinocytes in the lower two-thirds of the epidermis, and in all but one there was a cleavage plane through the basal keratinocytes. These findings were confirmed by electron microscopy in 4 patients. The separation through the basal cells is in contrast to the situation in normal individuals in whom cleavage occurs below the level of the basal cells, within the lamina lucida. Thus, even apparently normal skin from non-acral sites has the same structural abnormality as the affected acral sites in EBS-WC.

Adolescent↗
PubMed1993

Bullous systemic lupus erythematosus--a variable disease.

We describe a patient with the rare diagnosis of 'Bullous Systemic Lupus Erythematosus'. She is unusual in presenting with classical dermatitis herpetiformis, before the emergence of systemic features. In addition, on indirect immunofluorescence on sodium chloride split skin, there was epidermal binding. Immunoblotting was negative for type VII collagen.

Adult↗
PubMed1993

Long-term control of vulval lichen sclerosus after treatment with a potent topical steroid cream.

Super potent topical steroids are an effective treatment for many patients with vulval lichen sclerosus, but concerns exist about their long-term safety. Review of 12 women up to 39 months following a course of super potent topical steroids shows that subsequent control of disease activity and symptoms is much easier than before therapy, with most patients requiring only small amounts of moderate potency topical steroid. Apart from a background erythema no long term cutaneous side effects were observed.

Administration, Topical↗
PubMed1993

Vulvar involvement in autoimmune bullous diseases.

The autoimmune blistering diseases are characterized by involvement of the skin and mucous membranes. On mucosal surfaces, although the initial lesion is often a blister, friction results in erosions. We report on 140 female patients with a variety of blistering diseases examined in the bullous disease clinic. Vulval involvement was found in 5 of 55 adult patients and in 2 of 3 girls with bullous pemphigoid. Sexual abuse was initially suspected in one child. Twenty-six women had cicatricial pemphigoid; of these, 14 had vulval involvement, often with severe scarring. Chronic bullous disease of childhood was identified in 20 girls; perineal involvement was present in 16 causing initial misdiagnosis of herpes simplex in one case and sexual abuse in another. Vulval involvement was less frequent in linear IgA disease (the adult counterpart of chronic bullous disease of childhood), occurring in 9 of 22 patients. One of two adults and two of three girls with epidermolysis bullosa acquisita had vulval involvement. Finally, of nine female patients with pemphigus, four had vulval involvement, all of whom had pemphigus vulgaris. Blistering diseases are rare, but vulval involvement is common across the spectrum of these diseases. When mucosal involvement predominates, diagnostic difficulties can arise.

Adult↗
PubMed1992

Lack of predictive factors for the clinical course of bullous pemphigoid.

BACKGROUND: Bullous pemphigoid is a clinically heterogeneous disease although little is known of the factors affecting its course and outcome. OBJECTIVE: Our purpose was to document the clinical course, outcomes, and causes of death in treated bullous pemphigoid and to determine the predictive factors affecting outcome. METHODS: The clinical course was documented in 82 patients with immunologically proven bullous pemphigoid (mean follow-up 3 years 2 months). To identify factors predictive of outcome, 16 patients with "good prognosis bullous pemphigoid" (no systemic treatment or in remission within 2 years) were compared with 12 patients with recurrent disease requiring maintenance therapy who still needed treatment after 3 years or longer. Remission was defined as 3 months free of lesions, without systemic treatment. RESULTS: The disease duration varied from 9 weeks to 17 years (estimated median treatment time 2 years 1 month). Of patients followed up for at least 2 years, 30% achieved remission and by 3 years the remission rate was 50%. Two patients had a subsequent relapse (9%). The mortality rate at 1 year was 19%, and treatment was believed to be contributory in seven deaths. No clinical, immunologic, or immunogenetic factors were predictive of disease duration. CONCLUSION: Despite the heterogeneity of the clinical course and duration of bullous pemphigoid, no predictive factors are recognized.

Aged↗
PubMed1992

The distribution of alpha 6 beta 4 integrins in lesional and non-lesional skin in bullous pemphigoid.

The alpha 6 beta 4 integrin is associated ultrastructurally with the hemidesmosomes of the basal keratinocytes and with the bullous pemphigoid antigen (BPA), suggesting an important role in adhesion of epidermal cells to the basement membrane. Using an immunofluorescence technique with chain-specific monoclonal antibodies to the alpha and beta subunits we have investigated the distribution of the alpha 6 beta 4 integrin in normal skin (n = 3) and in BP skin (uninvolved, perilesional and lesional) [n = 11]). The findings have been compared with other types of subepidermal blisters and with normal skin split by chemical means (n = 2) and by suction (n = 2). The distribution of alpha 6 beta 4 integrin was compared with that of bullous pemphigoid antigen (BPA) and with other basement membrane zone (BMZ) macromolecules, laminin, collagen type IV, collagen type VII and the BM600 antigen. In uninvolved, perilesional and early pre-blistered lesional BP skin the distribution of both the alpha 6 and beta 4 integrin subunits, BPA laminin, collagen types IV and VII and the BM600 antigen was identical to normal skin, i.e. a linear band in the BMZ. Within BP blisters, both alpha 6 and beta 4 integrin subunits and BPA were absent, except in two blisters in which the integrin expression was retained in the blister roof, despite loss of BPA. The other BMZ components were expressed on the blister floor.(ABSTRACT TRUNCATED AT 250 WORDS)

Antibodies, Monoclonal↗
PubMed1992

Allergic contact dermatitis and vulvar dermatoses.

Vulval itch and discomfort are often intractable symptoms and the response to standard therapy can be unsatisfactory. There is some evidence that vulvar sensitivity to irritants is higher than that of forearm skin, but the incidence of relevant allergic contact sensitivity amongst this patient population is unknown. The patch-test data over a 5-year period of 135 patients with persistent vulval symptoms were reviewed and 63 patients had positive results. Of these, 39 had positive results considered to be relevant to their clinical condition. Medicaments and their constituents were responsible for most of these reactions and more than half the patients had multiple allergies.

Adolescent↗