Serum autoantibody reactivity in bullous pemphigoid is associated with neuropsychiatric disorders and the use of antidiabetics and antipsychotics: a large, prospective cohort study.

BACKGROUND: Bullous pemphigoid (BP), the by far most frequent autoimmune blistering skin disease (AIBD), is immunopathologically characterized by autoantibodies against the two hemidesmosomal proteins BP180 (collagen type XVII) and BP230 (BPAG1 or dystonin). Several comorbidities and potentially disease-inducing medication have been described in BP, yet a systematic analysis of these clinically relevant findings and autoantibody reactivities has not been performed. OBJECTIVE: To determine associations of autoantibody reactivities with comorbidities and concomitant medication. METHODS: In this prospective multicenter study, 499 patients diagnosed with BP in 16 European referral centers were included. The relation between anti-BP180 NC16A and anti-BP230 IgG ELISA values at the time of diagnosis as well as comorbidities and concomitant medication collected by a standardized form were analysed. RESULTS: An association between higher serum anti-BP180 reactivity and neuropsychiatric but not atopic and metabolic disorders was observed as well as with the use of insulin or antipsychotics but not with dipeptidyl peptidase-4 (DPP4) inhibitors, inhibitors of platelet aggregation and L-thyroxine. The use of DPP4 inhibitors was associated with less anti-BP180 and anti-BP230 reactivity compared with BP patients without these drugs. This finding was even more pronounced when compared with diabetic BP patients without DPP4 inhibitors. Associations between anti-BP180 and anti-BP230 reactivities were also found in patients using insulin and antipsychotics, respectively, compared with patients without this medication, but not for the use of inhibitors of platelet aggregation, and L-thyroxine. CONCLUSION: Taken together, these data imply a relation between autoantibody reactivities at the time of diagnosis and both neuropsychiatric comorbidities as well as distinct concomitant medication suggesting a link between the pathological immune mechanisms and clinical conditions that precede the clinically overt AIBD.

[Scabies therapy in Germany : Results of a nationwide survey with a special focus on the efficacy of first-line therapy with permethrin]. / Skabiestherapie in Deutschland : Ergebnisse einer bundesweiten Umfrage mit besonderem Fokus auf die Wirksamkeit der Erstlinientherapie mit Permethrin.

BACKGROUND: According to the current scabies guideline, topical treatment with permethrin 5% cream is the first-line therapy of common scabies in Germany. However, in the course of growing incidence of scabies in recent years there have been increasing numbers of reports of reduced efficacy in standard therapy. OBJECTIVE: To establish a better understanding for the efficacy of scabies therapy under practice conditions, a survey focusing on the first-line therapy with permethrin was conducted in dermatological hospitals, outpatient clinics and dermatology practices. METHODS: The postal survey addressed all dermatologically active physicians in Germany and queried diagnostic methods, the individual initial treatment, the estimated efficacy of permethrin therapy, the frequency and reasons for therapy failures under permethrin and the therapeutic approach in case of failure of the initial therapy. RESULTS: The efficacy of permethrin was rated with 73% by 187 physicians. About 74% reported to treat initially with 5% permethrin cream. The most common reasons identified for treatment failure were application errors, lack of compliance and inadequate hygiene measures. Suspected diminished efficacy or development of resistance against permethrin and re-infestations were also mentioned. In the case of ineffectiveness of initial therapy, most clinicians opt for oral ivermectin, a repetition of permethrin therapy or a combination of both. CONCLUSION: Although there are localized reports of decreased effectiveness of permethrin therapy, results of this survey point towards a persisting high efficacy of first-line standard treatment of scabies with permethrin 5% cream in Germany.

[Reality of inpatient vasoactive treatment with prostacyclin derivatives in patients with acral circulation disorders due to systemic sclerosis in Germany]. / Versorgungsrealität der stationären vasoaktiven Therapie mit Prostazyklinderivaten bei Patienten mit akralen Durchblutungsstörungen bei systemischer Sklerose in Deutschland.

BACKGROUND: Raynaud's phenomenon and the frequently ensuing digital ulcerations represent an early and very distressing symptom in patients with systemic sclerosis (scleroderma, SSc) causing significant limitations in the ability to work and quality of life. The use of vasoactive drugs (especially intravenous prostacyclin derivatives) is recommended to reduce the risk of hypoxic tissue damage up to the loss of fingers. METHODS: In order to obtain information about the current state of treatment of patients with prostacyclin derivatives in routine clinical life in Germany, a survey was conducted among the centers affiliated to the German Network for Systemic Scleroderma (DNSS). In addition, a separate patient survey was conducted by the schleroderma self-help group (Sklerodermie Selbsthilfe e. V.), which only covered the symptoms Raynaud's syndrome, digital ulcers and the use of intravenous prostacyclin derivatives. RESULTS: Of the 433 patients surveyed 56% stated that they had already been treated with prostacyclin derivatives (iloprost/alprostadil) because of their illness and symptoms. A total of 61% received the treatment for severe Raynaud's phenomenon and 39% for digital ulcerations. Most respondents not only experienced an improvement in Raynaud's phenomenon and digital ulcers but also a significant improvement of limitations in everyday life. They also needed significantly less outside help and absenteeism from work was much lower. CONCLUSION: Patients consistently reported a positive effect of treatment with prostacyclin derivatives on Raynaud's phenomenon, acral ulcerations, pain and daily restrictions and felt well and safely cared for during inpatient treatment. These positive effects in the patients' perceptions provide crucial information supporting and confirming the current European and international treatment recommendations.

[Bullous pemphigoid on a healed herpes zoster : A rare example of an isotopic response]. / Bullöses Pemphigoid auf einem ausgeheilten Zoster thoracalis : Ein seltenes Fallbeispiel für eine isotope Reaktion.

Wolf's isotopic response describes the eruption of a secondary, pathogenically independent skin disease within the borders of a primary, already healed skin disease. The exact mechanism is unknown, but the restriction to diseases with a known T­cell component in the pathogenesis suggests an involvement of persistent local immune activation. We report the case of an 87-year-old woman who developed bullous pemphigoid lesions within the boundaries of a previous, already healed herpes zoster C7-C8, which had been diagnosed clinically and treated 2 months previously. Histopathological examination revealed subepidermal blisters, and indirect immunofluorescence showed IgG antibodies against the basal membrane zone. Both BP 180 and BP 230 enzyme-linked immunoassays (ELISAs) were positive. Based on these findings, we diagnosed a bullous pemphigoid arising on the site of the already healed herpes zoster, linked by an isotopic response.

Increased sensitivity and high specificity of indirect immunofluorescence in detecting IgG subclasses for diagnosis of bullous pemphigoid.

BACKGROUND: Indirect immunofluorescence (IIF) microscopy on monkey oesophagus is an important assay for the diagnosis of bullous pemphigoid (BP). Its relatively low sensitivity (60-80%) may be partly due to insufficient detection of minor IgG subclasses. AIM: To determine the operating characteristics of an IgG subclass in IIF. METHODS: We designed a retrospective, dual-centre, controlled cohort study on sera from 64 BP sera that had been rated as false negatives by traditional IIF microscopy, and assessed circulating IgG1 , IgG3 and IgG4 autoantibodies. RESULTS: The sensitivities of IIF in detecting IgG1 , IgG3 , IgG4 and all three in combination were 45.3%, 18.8%, 32.8% and 48.4%, respectively. Specificities were > 97%. CONCLUSION: Detection of IgG subclass (especially IgG1 and IgG4 ) autoantibodies by IIF on monkey oesophagus can significantly improve diagnostic performance of IIF microscopy for diagnosis of BP.

[Inflammatory diseases of oral mucous membranes]. / Entzündliche orale Schleimhauterkrankungen.

BACKGROUND: Mucous membranes are involved in many different inflammatory disorders. METHODS: We describe the clinical features, diagnostic workup, and therapy of inflammatory diseases of mucous membranes. RESULTS: The diagnostic workup is based on medical history, clinical manifestations, histopathology, and serology. Inadequate dental hygiene is one of the main pathogenetic factors. Treatment options depend on the diagnosis, underlying cause, and disease severity. Both local and systemic immunosuppressive or immunomodulatory strategies may be effective. CONCLUSIONS: The diagnostic and therapeutic management of inflammatory mucous membrane disorders is usually more difficult than that of skin diseases and requires interdisciplinary cooperation.

[Pemphigoid diseases. Autoimmune diseases in the elderly]. / Pemphigoiderkrankungen. Autoimmunerkrankungen des Alters.

BACKGROUND: The incidence and thereby also the relevance of pemphigoid diseases have significantly increased in elderly patients. METHODS: The clinical features, diagnostic workup, and therapy of pemphigoid diseases based on the recently published German guidelines and expert opinions are described. RESULTS: The diagnosis is based on medical history, clinical manifestations, histopathology, and autoimmune serology. Treatment options depend on the diagnosis and disease severity. Both local and systemic immunosuppressive or immunomodulatory strategies have been proven to be effective. CONCLUSIONS: Chronic, itchy, inflammatory skin disorders in elderly patients are generally suspicious for pemphigoid diseases. The prognosis is usually good. For decisions about (local or systemic) immunosuppressive therapy, gerontological aspects should be taken into consideration.