Notes: Summary Conventional first-line treatments for linear IgA disease (and the related chronic immunobullous disease of childhood) include topical steroids and dapsone, both of which may be associated with potentially serious side-effects. Alternative anti-inflammatory therapies such as tetracycline and macrolide antibiotics, have been used to treat other immunobullous disorders and we now report an adult case of linear IgA disease and a paediatric case of mixed immunobullous disease of childhood that both responded to the macrolide antibiotic erythromycin. The mode of action is speculative and anti-inflammatory mechanisms are unclear. Nevertheless, from a clinical perspective, erythromycin may have a role in the treatment of linear IgA disease and could be considered ahead of many other, perhaps potentially more toxic, therapies.

Notes: Summary We present a child on long-term treatment with levamisole, and heterozygous for factor V Leiden, who developed cutaneous necrosis associated with formation of p-ANCA and lupus anticoagulant. Symptoms resolved when the levamisole treatment was stopped.

Notes: Background  In bullous pemphigoid (BP), cicatricial pemphigoid (CP) and linear IgA disease (LAD), autoantibodies to the basement membrane zone (BMZ) are found in skin and mucosa, blood and blister fluid. Objectives  To assess whether BMZ antibodies might also be detected in urine. Methods  Urine and serum samples from 62 patients (32 with BP, 17 with CP and 13 with LAD) were analysed for antibody isotypes and subclasses by indirect immunofluorescence, and urine and serum samples from 40 patients (25 with BP, eight with CP and seven with LAD) were screened for target antigens using immunoblotting. Results  Fourteen of 32 patients with BP had detectable levels of IgG BMZ autoantibodies in their urine, and all 32 had positive sera. Of these 14 BP patients, 13 had epidermal-binding serum autoantibodies at a titre 〉 1 : 160, and one had dermal-binding serum antibodies at a titre of 1 : 40. BMZ autoantibodies were not detected in the urine of the CP or LAD patients, but the corresponding sera were of low titre or negative. IgG subclasses (IgG1–4) were less frequently detected in urine than in serum. IgG4 was the predominant subgroup found (10 urine samples and all 14 sera), followed by IgG1 (two urine samples and 12 sera); IgG2 was detected in a single urine sample and three sera, and IgG3 was not detected. Eight of 25 BP and one of eight CP urine samples were positive on immunoblotting, and bound BP230 and/or BP180 with IgA and/or IgG autoantibodies. IgA autoantibodies were not detected in the urine of the seven LAD patients. The corresponding sera were often more positive, with 21 of 25 BP, five of eight CP and six of seven LAD sera immunoblotting the major BP antigens. Conclusions  The detection of IgG autoantibodies from urine samples using indirect immunofluorescence correlated with a high titre of IgG autoantibodies in the serum. IgG and IgA autoantibodies in the urine were detected by immunoblotting, although less frequently than in serum. The finding of BMZ antibodies in the urine of many BP patients may have clinical relevance, and may have a restricted application in the diagnosis of immunobullous disease.

Notes: Background Over the years, a substantial number of scientific papers has been published considering the possible relationship between oral contraceptive use and various skin disorders. Objectives We aimed to investigate the possible effects of oral contraceptives and other factors including age, social class, smoking and body mass index on the occurrence of hospital referral for skin disorders within the Oxford Family Planning Association (Oxford-FPA) contraceptive study. Methods We carried out a cohort study with long-term follow up of 17,032 women aged 25–39 years recruited at 17 family planning centres in England and Scotland between 1968 and 1974. The main outcome measure was the pattern of referral to hospital for skin disorders among the participants. The conditions considered (number of women affected in parentheses) were: malignant melanoma (48); other skin cancers (83); benign skin neoplasms (384); boils and cellulitis (334); eczema and dermatitis (327); erythematous disease (54); psoriasis (92); hypertrophic and atrophic conditions (59); diseases of the nails (80); diseases of the hair and hair follicles (74); diseases of the sebaceous glands (362); pigmented naevi (383); and unspecified eruptions (199). Some of these disease categories are broad, which complicates interpretation of the findings. Results As the data relate to hospital referrals, while most skin disorders are largely managed within primary care, it is difficult to know to what extent our findings can be generalized to disease patterns in the community. None the less, a number of interesting results emerged. These included increased risks (approximately twofold) of referral to hospital for boils and cellulitis in heavy women, for psoriasis in women who smoke, and for pigmented naevi in women of higher social class. Referral for pigmented naevi was reduced by about half in women with a high body mass index. Of the oral contraceptive associations studied, we consider that only an increased risk of referral for eczema or dermatitis in current or recent pill users (relative risk 1·6, 95% confidence interval 1·2–2·1) might reflect a causal relationship. There was no increased risk in past users nor was the risk related to duration of use. These findings are very similar to those reported from the Royal College of General Practitioners oral contraception study. Conclusions Hospital referral for certain skin disorders was related to age, social class, smoking and body mass index within the Oxford-FPA study. Hospital referral for skin disorders generally seems to be unrelated to oral contraceptive use within the study, the possible exception being eczema and dermatitis, which occur more commonly in current and recent pill users than in non-users.

Notes: Lichen sclerosus is among the most frequently seen paediatric vulval disorders. In adults a strong association between lichen sclerosus and autoimmune diseases, and also with HLA class II locus DQ7, has been well demonstrated in women and a weaker association in men. These associations have not previously been studied in children, although in other autoimmune diseases, the HLA associations have been strongest in children. We performed HLA tissue typing and looked for autoimmune associations in a group of 30 children with vulval lichen sclerosus. HLA DQ7 was present in 66% of female children with lichen sclerosus compared with 31% in controls. Previous studies reported DQ7 in 51% of adult female patients and 45% of male patients. Sixteen per cent of the children were homozygous for DQ7 as opposed to 5% of controls. In the childhood group, only 4% had another autoimmune disease, but 56% of their parents or grandparents did. Age differences make comparison difficult, but the family history of autoimmunity appears to be strong in the early-onset group, in addition to the stronger association with DQ7.

Notes: Background Immunobullous diseases are uncommon in childhood. In contrast to adults, the most commonly seen is IgA-mediated chronic bullous disease of childhood (CBDC), while IgG-mediated bullous pemphigoid (BP), cicatricial pemphigoid (CP) and epidermolysis bullosa acquisita (EBA) are rare. We have demonstrated both IgG and IgA autoantibodies to basement membrane zone target antigens in eight children with ‘mixed immunobullous disease of childhood’. Objectives To elucidate whether a dual antibody response makes these patients distinct regarding their presentation, immunopathology, course and prognosis. Methods We compared the eight children showing the double antibody response with 62 children with CBDC, BP, CP and EBA in whom only one antibody isotype was demonstrated. Clinical information at presentation, clinical course and response to treatment were recorded, and immunoblotting and direct and indirect immunofluorescence (IF) were performed. Results Six of the eight patients presented with clinical features of CBDC. In two others, it was uncertain whether they had CBDC or BP. Seven of the eight demonstrated a dual antibody response on indirect IF and three on direct IF. Immunoblotting revealed a variety of epidermal and dermal target antigens (BP230, BP180, 97-kDa protein and laminin 5). Five of the eight responded well to dapsone, two to sulphonamides, and one to systemic erythromycin alone. The clinical course was not protracted. Five are in remission 1–4 years following treatment, and three still have active disease suppressed by treatment after 6 months−2 years. Conclusions Although we do not know why these children have ‘mixed immunobullous disease’ (the dual antibody response), our results indicate that the presence of IgA is associated with a good response to treatment with antimicrobials (dapsone, sulphonamides, erythromycin), and the clinical course is no more protracted than that found in children with a single antibody response.