Risk Perception Plays Minimal Role in Sun Exposure Behaviours.

Despite decades of public health campaigns, tanning and sunburn still occur at unacceptably high rates. Skin cancer prevention campaigns predominately focus on increasing awareness of the risk of excessive sun exposure. This study sought to assess the efficacy of this approach, by interrogating correlations between risk perception and sun exposure behaviour. A 31-item questionnaire assessing skin cancer risk factors, tanning attitudes, sunburn and tanning behaviour was undertaken by individuals who attended a workplace skin check. Validated questions were included to assess cognitive and affective risk perception and to frame risk as absolute, comparative and conditional. One hundred sixty-seven respondents completed the questionnaire. No aspects of risk perception (absolute cognitive, affective or conditional) significantly correlated with protective sun exposure behaviour, with the exception of perceived comparative severity of skin cancer. Instead, positive tanning attitudes were far more significantly correlated with sun exposure behaviour. Actual risk and risk perception have very limited impact on sun exposure behaviour. Instead, sun exposure behaviour was significantly linked with positive tanning attitudes. It is suggested, therefore, that campaigns focussing solely on education regarding risk factors appear to have been ineffective in behaviour mitigation, and innovative approaches, aimed at influencing tanning norms, might complement the existing educational campaigns.

Three difficult cases: the challenge of autoimmunity, immunodeficiency and recurrent infections in patients with Good syndrome.

Good syndrome (GS) is a rare, adult-acquired primary combined immunodeficiency syndrome arising in the context of previous or current thymoma. Patients with GS frequently develop recurrent sinopulmonary infections and are also at high risk of autoimmune manifestations, including skin conditions such as lichen planus. We report three middle-aged patients with GS complicated by multiple autoimmune and infectious manifestations. The combination of immunodeficiency, autoimmunity and recurrent infections seen in patients with GS continues to present a management challenge, particularly in patients with oral mucosal disease and recurrent candidiasis. Clinicians should be prompted to investigate an underlying immunodeficiency in patients with multiple autoimmune conditions and recurrent sinopulmonary infections.

Immunopathological characteristics of patients with bullous pemphigoid and neurological disease.

BACKGROUND: The relationship between bullous pemphigoid (BP) and neurological disease has been the subject of numerous recent studies and BP antigens and their isoforms have been identified in the central nervous system (CNS). Whilst epidemiological data support this association, little is known about the pathomechanism behind this link and the immunological characteristics of patients with BP and neurological disease, other than multiple sclerosis (MS), has not been studied. OBJECTIVE: We aimed to compare the cutaneous immune response in BP patients with and without neurological disease, to investigate whether or not there is a distinctive immunopathological profile in patients with concomitant BP and neurological disease. METHODS: Seventy-two patients with BP were included and divided into two groups; those with neurological disease (BP+N, n = 43) and those without (BP-N, n = 29). Patients in BP+N group had a confirmed neurological disease by a hospital physician, neurologist or psychiatrist with positive neurological imaging where appropriate, or a Karnofsky score of 50 or less due to mental impairment. All sera were analysed with indirect immunofluorescence (IIF) using serial dilutions up to 1:120000, immunoblotting (IB) and Enzyme-linked immunosorbent assay (ELISA) for BP180 and BP230. RESULTS: Median antibody titres by IIF were 1:1600 vs. 1:800 for BP-N and BP+N, respectively, although the difference did not reach statistic significance (P = 0.93, Mann-Whitney U-test). ELISA values for both BP180 and BP230 did not differ significantly between the two groups. Similarly, autoantibodies to specific antigens as identified by ELISA and IB were not related to the presence of neurological disease. CONCLUSION: The results of this study indicate that patients with BP and neurological disease exhibit an immune response to both BP180 and BP230, thus the link between the CNS and the skin is not dependent on a specific antigen, but possibly both antigens or their isoforms may be exposed following a neurological insult, and play a role in generation of an immune response.

Guidelines for the management of bullous pemphigoid.

These guidelines have been prepared for dermatologists on behalf of the British Association of Dermatologists. They present evidence-based guidance for treatment, with identification of the strength of evidence available at the time of preparation of the guidelines and a brief overview of epidemiological aspects, diagnosis and investigation. The guidelines reflect data available from Medline, Embase, the Cochrane library, literature searches and the experience of the authors of managing patients with bullous pemphigoid in special and general clinics for over 10 years. However, caution should be exercised in interpreting the data obtained from the literature because only six randomized controlled trials are available involving small groups of patients.

Bullous pemphigoid: correlation of mucosal involvement and mucosal expression of autoantigens studied by indirect immunofluorescence and immunoblotting.

Twenty patients with bullous pemphigoid were studied prospectively: sequential sera, in different phases of the disease, were collected over a period of approximately 2 years. The sera were tested using standard immunofluorescence techniques with salt-split and intact human tissue from different sites of the body (thigh, breast, oral mucosa, vagina); an early serum of each patient was tested by Western blotting. The concentration of circulating antibodies detected by the intact skin and intact mucous membranes was similar; split tissue was more sensitive than intact tissue. For eight of 19 patients, split vagina and occasionally split oral mucosa (in the same patients) were much less sensitive than all other tissues. Furthermore, there was a correlation between autoantibody reactivity with split mucous membrane tissues and clinical mucosal involvement. These results strongly suggest heterogeneity of antigens or epitopes expressed between tissues. In both split skin and mucosa all sera consistently detected an antigen on the epidermal side of the split regardless of the stage of the disease. Immunoblotting studies showed no correlation between specific antigens and mucosal expression or skin involvement.

The association of HLA-DQ7 with bullous pemphigoid is restricted to men.

This study examines in detail the HLA associations of 74 patients (40 women and 34 men) with bullous pemphigoid (BP) and compares their immunogenetic profile with that of 604 unrelated control subjects (238 women and 366 men). Correlations were sought between HLA antigens and the various BP disease parameters investigated. The presence of milia was the only clinical or laboratory finding which was linked with a specific HLA antigen, HLA-DQ6, in both men and women with BP (P < 0.01). BP has previously been linked with the HLA-DQ7 antigen and this association was confirmed in 39 of our patients (14 women and 25 men). Twelve of these patients (four women and eight men) were homozygous for HLA-DQ7. The association of HLA-DQ7 with BP was gender-restricted and only significant for men (P < 0.01). No equivalent HLA disease susceptibility risk factor could be identified for our female BP patients. This difference in HLA association between men and women with BP has not been reported previously, and its significance for disease pathogenesis is not known. No specific link could be found between HLA-DQ7 and BP for any of the clinical, immunofluorescence, western blotting, treatment or prognostic disease factors studied.

Acquired subepidermal bullous diseases associated with psoriasis: a clinical, immunopathological and immunogenetic study.

Clinical, immunopathological and immunogenetic studies of four patients with a subepidermal bullous disease associated with psoriasis were carried out to determine the true nature of the blistering disease and to investigate further the relationship between psoriasis and acquired subepidermal bullous diseases. Autoantibodies in all four patients bound to the epidermal side of salt-split skin by indirect immunofluorescence and detected the major bullous pemphigoid (BP) antigens by immunoblotting. One had additional IgA autoantibodies binding an epidermal polypeptide of 270/280 kDa and another had circulating IgG autoantibodies which detected both BP and epidermolysis bullosa acquisita (EBA) antigens. All patients had active psoriasis at the onset of the bullous disease. Three patients were being treated with tar when blisters developed; one patient also received UVB radiation and experienced a relapse after exposure to sunlight. HLA phenotypes in three patients were determined. All the patients responded well to methotrexate. These findings demonstrate that BP is the subepidermal bullous disease most associated with psoriasis. Changes at the basement membrane zone in psoriasis may be responsible for the heterogeneous antibody response and may trigger the bullous disease, as may antipsoriatic treatment including tar and UV radiation. However, common immunogenetic mechanisms may play a crucial part in this disease association.

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.

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.

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.