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Background: Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease of the skin and mucous membranes. This disease typically affects the elderly and presents with itch and localized or, most frequently, generalized bullous lesions. A subset of patients only develops excoriations, prurigo‐like lesions, and eczematous and/or urticarial erythematous lesions. The disease, which is significantly associated with neurological disorders, has high morbidity and severely impacts the quality of life. Objectives and methodology: The Autoimmune blistering diseases Task Force of the European Academy of Dermatology and Venereology sought to update the guidelines for the management of BP based on new clinical information, and new evidence on diagnostic tools and interventions. The recommendations are either evidence‐based or rely on expert opinion. The degree of consent among all task force members was included. Results: Treatment depends on the severity of BP and patients' comorbidities. High‐potency topical corticosteroids are recommended as the mainstay of treatment whenever possible. Oral prednisone at a dose of 0.5 mg/kg/day is a recommended alternative. In case of contraindications or resistance to corticosteroids, immunosuppressive therapies, such as methotrexate, azathioprine, mycophenolate mofetil or mycophenolate acid, may be recommended. The use of doxycycline and dapsone is controversial. They may be recommended, in particular, in patients with contraindications to oral corticosteroids. B‐cell‐depleting therapy and intravenous immunoglobulins may be considered in treatment‐resistant cases. Omalizumab and dupilumab have recently shown promising results. The final version of the guideline was consented to by several patient organizations. Conclusions: The guidelines for the management of BP were updated. They summarize evidence‐ and expert‐based recommendations useful in clinical practice. [ABSTRACT FROM AUTHOR]

Summary: Background: Antilaminin 332 mucous membrane pemphigoid (MMP) is an autoimmune subepidermal blistering disease with predominant mucosal involvement and autoantibodies against laminin 332. Malignancies have been associated with this disease; however, no standardized detection system for antilaminin 332 serum antibodies is widely available. Objectives: Development of a sensitive and specific assay for the detection of antilaminin 332 antibodies. Methods: An indirect immunofluorescence (IF) assay using recombinant laminin 332 was developed and probed with a large number of antilaminin 332 MMP patient sera (n = 93), as well as sera from patients with antilaminin 332‐negative MMP (n = 153), bullous pemphigoid (n = 20), pemphigus vulgaris (n = 20) and noninflammatory dermatoses (n = 22), and healthy blood donors (n = 100). Results: In the novel IF assay, sensitivities with the laminin 332 heterotrimer and the individual α3, β3 and γ2 chains were 77%, 43%, 41% and 13%, respectively, with specificities of 100% for each substrate. The sensitivity for the heterotrimer increased when an anti‐IgG4 enriched antitotal IgG conjugate was applied. Antilaminin 332 reactivity paralleled disease activity and was associated with malignancies in 25% of patients with antilaminin 332 MMP. Conclusions: The novel IF‐based assay will facilitate the serological diagnosis of antilaminin 332 MMP and may help to identify patients at risk of a malignancy. What's already known about this topic? Antilaminin 332 mucous membrane pemphigoid (MMP) is an autoimmune subepidermal blistering disease with predominant mucosal involvement characterized by antilaminin 332 autoantibodies.Malignancies have been associated with this disease, but currently no standardized detection system for antilaminin 332 serum antibodies is widely available. What does this study add? A sensitive and highly specific immunofluorescence assay for the detection of antilaminin 332 autoantibodies using recombinant laminin 332‐expressing human cells was developed, which will soon be widely available. What is the translational message? This assay may not only facilitate the diagnosis of antilaminin 332 MMP but may also be valuable to monitor disease activity and identify patients at risk of a malignancy. Respond to this article [ABSTRACT FROM AUTHOR]

To improve our understanding on the neuromechanics of finger movements, a comprehensive musculoskeletal model is needed. The aim of this study was to build a musculoskeletal model of the hand and wrist, based on one consistent data set of the relevant anatomical parameters. We built and tested a model including the hand and wrist segments, as well as the muscles of the forearm and hand in OpenSim. In total, the model comprises 19 segments (with the carpal bones modeled as one segment) with 23 degrees of freedom and 43 muscles. All required anatomical input data, including bone masses and inertias, joint axis positions and orientations as well as muscle morphological parameters (i.e. PCSA, mass, optimal fiber length and tendon length) were obtained from one cadaver of which the data set was recently published. Model validity was investigated by first comparing computed muscle moment arms at the index finger metacarpophalangeal (MCP) joint and wrist joint to published reference values. Secondly, the muscle forces during pinching were computed using static optimization and compared to previously measured intraoperative reference values. Computed and measured moment arms of muscles at both index MCP and wrist showed high correlation coefficients (r = 0.88 averaged across all muscles) and modest root mean square deviation (RMSD = 23% averaged across all muscles). Computed extrinsic flexor forces of the index finger during index pinch task were within one standard deviation of previously measured in-vivo tendon forces. These results provide an indication of model validity for use in estimating muscle forces during static tasks. [ABSTRACT FROM AUTHOR]

Anxiety sensitivity (AS), which refers to the tendency to interpret anxiety-related bodily sensations as having potentially harmful somatic, psychological or social consequences, has been proposed as a vulnerability factor for the development of panic disorder (PD). The current study examined the anxiety sensitivity levels in children of parents with panic disorder. Children of panic disorder patients ( n=68) and children of healthy parents ( n =68) filled out the Childhood Anxiety Sensitivity Index, while parents completed the Anxiety Sensitivity Index. Children of parents with panic disorder did not display higher levels of anxiety sensitivity than children of healthy parents. Furthermore, no association between anxiety sensitivity levels of parents with panic disorder and their children was found. Anxiety sensitivity is not clearly manifest in children of parents with panic disorder and might be a developing vulnerability factor that may increase towards late adolescence or early adulthood. [ABSTRACT FROM AUTHOR]

Anxiety sensitivity (AS) has been proposed as a risk factor for the development of panic disorder. Strong familial-genetic influences in panic disorder (PD) have been reported. AS may be familial too. The current study therefore examined whether first-degree relatives of PD patients are more anxiety-sensitive than normals. Twenty-three first-degree relatives of PD patients, 38 PD patients and 30 normals were given the Anxiety Sensitive Index and the Body Sensations Questionnaire. It was found that the first-degree relatives were, indeed, more anxiety-sensitive than the normals, but less so than the PD patients, suggesting that AS runs in families. [Copyright &y& Elsevier]

Chemotherapy-induced hair follicle (HF) damage and alopecia are common side-effects of cancer therapy and constitute a major burden of disease in clinical oncology. Although several potential treatment strategies have shown promising effects in animal models, e.g. on cyclophosphamide-induced alopecia (CIA) in C57BL/6 mice, these strategies remain to be transferred to human therapy. 17-beta estradiol (E2) and glucocorticoids are potent modulators of murine CIA in vivo: topical treatment accelerates the regrowth of normally pigmented hair shafts by shifting HFs into the ‘dystrophic catagen’ pathway, which is associated with greater initial alopecia, but much faster HF recovery. In the current study, we asked whether E2 and/or prednisolon display similar activities in the human system, using our recently established human HF dystrophy in vitro-model (Bodó et al., Am J Pathol 2007: 171(14):1153–67). E2 and/or prednisolon were pre- and co-administered with a key cyclophosphamide metabolite (4-HC). Indeed, E2 shifted 4-HC-treated human HFs into the dystrophic catagen pathway in vitro, and potentiated the 4-HC-induced pigmentary disturbances. 4-HC-induced hair growth inhibition was further enhanced by E2, associated with an additional reduction of proliferation and increased apoptosis of matrix keratinocytes. These effects tended to be further enhanced by co-administration of prednisolon to the HF organ culture medium. These observations suggest that, just as in murine CIA in vivo, E2 and glucocorticoids can force chemotherapy-damaged human HFs into the dystrophic catagen pathway. The preclinical data provide first indications that a combination of E2 with prednisolone may indeed serve as an effective clinical tool for accelerating hair re-growth in human CIA. [ABSTRACT FROM AUTHOR]