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Biomedical subjects

F Wojnarowska

Publications and source records attributed to F Wojnarowska.

At least 145 records · Page 8Linked to original sources
PubMed1995

Treatment of cicatricial pemphigoid with tetracycline and nicotinamide.

Cicatricial pemphigoid is a rare autoimmune blistering disease involving predominantly mucosal areas in elderly patients. It runs a chronic relapsing course. Immunosuppressive treatments are frequently complicated by adverse effects and require careful monitoring. A 69-year-old woman is reported who responded well to a simple regimen of combined tetracycline and nicotinamide after 15 years of inadequate disease control and numerous adverse effects from immunosuppressive agents.

Aged↗
PubMed1995

Bullous pemphigoid and multiple sclerosis: a report of three cases and review of the literature.

Three patients with longstanding multiple sclerosis (MS) who developed bullous pemphigoid (BP) are reported. All patients had immunological features of typical BP as determined by immunofluorescence and Western immunoblotting studies. The clinical features, however, differed from those observed in typical BP. In two the BP started near an indwelling catheter and two had striking involvement of the soles. None of our patients, or a further nine cases reported in the literature, had mucous membrane involvement. In MS patients BP appears to develop at a younger age. Multiple drugs were taken by the MS patients; these, however, appear not to play a role in triggering their BP. The course of BP in patients with MS is moderate, although the majority require systemic treatment.

Aged↗
PubMed1994

Linear IgA disease and pregnancy.

BACKGROUND: Although many patients with linear IgA dermatosis (LAD) are young and have persistent disease, little is known about the interactions between LAD and pregnancy. OBJECTIVE: Our purpose was to study the effects of LAD on pregnancy, and vice versa. METHODS: Our study included 12 patients with LAD who underwent a total of 19 pregnancies. RESULTS: In all patients the disease improved during pregnancy, enabling therapy to be reduced or stopped. Dapsone was taken by patients during 11 pregnancies, and no adverse effects were seen. No patients had problems in labor. Most patients had a relapse approximately 3 months post partum, even if they had previously been in remission. In two patients, disease started within 3 months of delivery. Fetal outcome was unaffected in all but one fetus, who had a single transient blister. CONCLUSION: We found no contraindication to pregnancy in patients with LAD. We recommend that therapy be reduced or stopped whenever possible during pregnancy and that patients be counseled about the possibility of a relapse post partum.

Adult↗
PubMed1994

Severe case of pemphigoid gestationis with unusual target antigen.

We report a severe case of pemphigoid gestationis (PG) with an unusual target antigen. The patient developed a severe itchy macular rash on the abdomen during her fifth pregnancy, at 38 weeks' gestation. The disease persisted for 8 months post-partum, and could only be controlled with high doses of systemic corticosteroids and dapsone. The diagnosis of PG was confirmed by direct and indirect immunofluorescence. A linear band of IgG and C3 was detected at the epidermal aspect of salt-split skin. Immunoblotting studies revealed circulating IgG antibodies binding to a single 200-kDa protein in epidermal extracts. Immunofluorescence studies using trypsinized human keratinocytes demonstrated uniform staining around the cell peripheries, suggesting that the target antigen was not associated with hemidesmosomes. These findings differ from the usual immunological features seen in PG, and suggest further heterogeneity of the antigens involved in PG.

Adult↗
PubMed1994

Mucosal lichen sclerosus/lichen planus overlap syndromes.

Lichen sclerosus and lichen planus affecting cutaneous sites are easily distinguishable clinical. Clinical signs on mucosal sites, however, may not allow differentiation between these diseases, and reliance is frequently placed on histopathological findings. We report a series of seven patients with clinical evidence of coexisting vulval lichen sclerosus and lichenoid oral lesions. All patients had vulval biopsies, and four had oral biopsies. Histology of all the vulval biopsy specimens showed features consistent with lichen sclerosus. Two of these patients developed vulval intraepithelial neoplasia during the course of their disease, and one progressed to a well-differentiated squamous carcinoma. Histology of the oral lesions showed features that were predominantly those of lichen planus. There are, however, few reports of histologically proven oral lichen sclerosus. Variations in histopathological descriptions of lichen sclerosus, depending on duration of disease, have been reported, particularly with regard to the position of the inflammatory infiltrate in relation to the dermo-epidermal junction. We believe that these patients may have oral lichen sclerosus, or at the very least make up a distinctive group who need to be identified and followed up, as their risk of oral dysplasia is unknown.

Aged↗
PubMed1994

Chronic bullous disease of childhood and linear IgA disease of adults are IgA1-mediated diseases.

Linear IgA disease is characterized by the presence of linear IgA deposits at the basement membrane zone of the skin, and in some cases by circulating basement membrane zone antibodies. The disease occurs in both adults and children, and is designated adult linear IgA disease in the former and chronic bullous disease of childhood in the latter. The subclass distribution of the circulating and bound basement membrane zone antibodies was studied in 32 children and eight adults. The results were compared with five dermatitis herpetiformis patients and five normal controls. The circulating antibodies (39 patients) and the cutaneous deposits (39 patients) were IgA1 in all 40 patients with linear IgA disease. The cutaneous deposits in dermatitis herpetiformis were also all IgA1, and no circulating antibodies were detected. The controls were all negative. This large series of children and adults with linear IgA disease demonstrates that the circulating and cutaneous basement membrane zone deposits are all IgA1, and suggests that linear IgA disease is an IgA1-mediated disease.

Adult↗
PubMed1994

The basement membrane zone of the nail.

The anatomy of the epidermis, dermis and subcutaneous tissues of the nail apparatus is distinct from that of non-appendageal skin. Apart from the demonstration of the longitudinal configuration of the dermal-epidermal junction of the nail bed, there have been no studies of the composition of the basement membrane zone of the nail apparatus. We obtained three human accessory digits, including one thumb, all of which had been amputated for cosmetic reasons, and were without known pathology. Specimens were stained with a battery of monoclonal and polyclonal antibodies which target normal basement membrane zone antigens, and studied by indirect immunofluorescence. This study demonstrated that the four distinct regions of the nail, namely the proximal nail fold, the nail matrix, the nail bed and the hyponychium expressed all the target antigens found in the normal non-appendageal basement membrane. In particular, there was normal expression of the epidermal-associated antigens, the 220- and 180-kDa bullous pemphigoid antigens, and the alpha 6 beta 4 integrin. There was also normal expression of the lamina lucida antigens LH39, GB3 and laminin. It is of interest that the dermal-associated components, namely the 285-kDa linear IgA antigen, the extracellular matrix glycoproteins chondroitin sulphate, type VII collagen and its closely associated proteins, and the poorly characterized antigen for LH24 and LH39 were all normally expressed. Fibronectin, which is not a normal basement membrane zone component, was diffusely expressed in the extracellular matrix, but did not accentuate the basement membrane zone.(ABSTRACT TRUNCATED AT 250 WORDS)

Antibodies, Monoclonal↗
PubMed1994

Acquired bullous diseases of childhood: re-evaluation of diagnosis by indirect immunofluorescence examination on 1 M NaCl split skin and immunoblotting.

Acquired autoimmune bullous diseases of childhood are rare, and can be difficult to distinguish clinically. We have studied 12 children, with an initial diagnosis of bullous pemphigoid (BP) in eight patients, cicatricial pemphigoid (CP) in one, chronic bullous disease of childhood (CBDC) in one, and epidermolysis bullosa acquisita (EBA) in two. All patients had positive indirect immunofluorescence (IIF) of the BMZ with IgG. Using 1 M NaCl split skin, six patients showed epidermal binding of IgG, with additional IgA in three cases, and in five patients IgG antibodies bound a dermal protein. Immunoblotting studies revealed an antibody to type VII collagen (EBA antigen) in three patients who had a dermal pattern on IIF. Six sera reacted with an epidermal protein of 180 and/or 220 kDa, characteristic of BP and CP. One of the three IgA-positive sera detected 220- and 180-kDa epidermal proteins using anti-IgA antibody. Following these studies the diagnosis was changed in three of the children. The diagnosis of CBDC was changed to either BP or EBA because of the presence of circulating IgG autoantibodies. In two children with an initial diagnosis of BP the diagnosis was changed to EBA. We conclude that the clinical picture in bullous disorders of childhood shows considerable overlap, and is often misleading. Additional circulating IgA autoantibodies seem to be more common in BP than has been recognized previously. Indirect immunofluorescence investigation on 1 M NaCl split skin may be helpful in differentiating between BP and EBA, but does not replace immunoblotting studies. EBA is apparently more common in children than in adults. No difference was found between the children with BP and EBA with regard to the duration of disease. The long-term outlook is good, although the course may be protracted.

Adolescent↗
PubMed1994

Cicatricial pemphigoid presenting with unusual palmar involvement, successfully treated with a combination of nicotinamide and tetracycline.

Cicatricial pemphigoid is a rare subepidermal blistering disease, mainly of elderly women, that primarily involves mucous membranes. Oral, ocular or genital mucous membranes are most frequently involved and skin involvement is less frequent. We report a case of cicatricial pemphigoid with extensive cutaneous involvement, including unusual bilateral palmar involvement. Clinical improvement has been achieved with a combination of topical steroids, oral nicotinamide and tetracycline.

Aged↗
PubMed1994

Linear IgA disease: a heterogeneous disease.

Data from our series of 70 patients support the hypothesis that linear IgA disease is a heterogeneous disease as regards clinical features, target antigens and immunogenetics. The clinical presentation varies as regards age of onset and severity of skin and mucosal involvement and scarring. The use of split skin and of the novel substrate cylindroma has identified 2 target antigens. The majority of patients have a target antigen associated with epidermal cells and possibly cylindroma hemidesmosomes. A minority have a dermal antigen which resembles collagen VII on cylindroma. There is an association with HLA-B8, -DR3, Cw7 and with the linked rare tumour necrosis factor alpha allele, but this is not universal. These differences do not correlate with each other.

Adolescent↗
PubMed1994

Linear IgA disease: cylindroma demonstrates heterogeneity of the target antigens.

Serum from 58 patients with linear IgA disease was tested by indirect immunofluorescence on the novel substrate cylindroma, which produces an abundance of basement membrane components. The use of split skin and of cylindroma has identified 2 target antigens. The majority of patients have a target antigen associated with epidermal cells and similar in distribution on cylindroma to hemidesmosome proteins. A minority have a dermal antigen with the distribution of collagen VII on cylindroma. These data support the hypothesis that linear IgA disease is a heterogeneous disease with regard to the target antigens.

Adenoma↗
PubMed1994

Molecular overlap of the IgA target antigens in the subepidermal blistering diseases.

Linear IgA disease (LAD) has circulating IgA auto-antibodies which have been found to be associated with epidermal- and dermal-derived antigens. The localization and molecular weight(s) of the IgA target antigen(s) implicated in LAD and their relationship to the target antigens for the IgA antibodies in bullous pemphigoid (BP) and cicatricial pemphigoid (CP) are unknown. Sera from patients with all 3 diseases who had circulating IgA antibodies were studied by immunoblotting with both epidermal and dermal extracts. Our findings indicate that there is heterogeneity in the localization and molecular weights of the target antigen(s), with multiple bands demonstrated with some sera. Three principal target antigens were identified with molecular weights of 110-120, 160-180 and one approximately 285 kD. These antigens were common to all 3 diseases and were found in 25 patients. These results suggest that the target antigens for IgA basement membrane zone antibodies in LAD, BP and CP are multiple, complex in composition and may differ from the conventional BP antigens.

Autoantibodies↗