Epidermolysis bullosa acquisita mit facettenreichem klinischen Bild und erfolgreicher Mycophenolatmofetiltherapie

A 60-year-old woman with epidermolysis bullosa acquisita (EBA) presented with a highly variable clinical picture. First she developed oral erosions and ulcers covered with shaggy epithelial rests. Later she developed pustular and target lesions as well as tense cutaneous blisters. Clinically the alterations were strongly suggestive of aphthae or a pustular dermatosis. Histological and immunological examinations revealed subepidermal blister formation, neutrophilic infiltration, linear deposition of IgG and C3 at the basement membrane in the direct immunofluorescence and the evidence of circulating IgG-antibodies against basement membrane (monkey esophagus) in the indirect immunofluorescence. Thus our working diagnosis was a pustular or nodular form of bullous pemphigoid, as a linear IgA dermatosis was excluded. Indirect immunofluorescence using NaCl-separated primate skin localized the basement membrane antibodies in the base of the artificial blister, while immunoblotting of dermal extracts disclosed binding of serum antibodies to a 290-kDa protein. Bullous systemic lupus erythematosus was excluded, leading to the definitive diagnosis of EBA. The clinical variability of this disease is well known; the polymorphic picture in our patient with erosions, pustules, target lesions and blisters is unique in the literature. The similarity in histology and immunofluorescence of the clinically quite different lesions is another unexpected event. Also the speedy response to therapy with the immunosuppressive agent mycophenolate mofetil has only been described once before. The patient remains in remission after 6 months. Because of the poor response to therapy in most cases, the significant skin lesions are a source of physical as well as emotional distress. Additional complications include stenosis of trachea or bronchi injure.     

Rechallenge of targeted therapy in metastatic melanoma

Rechallenge of targeted therapy in patients with BRAFV⁶⁰⁰‐mutated melanoma plays an important role, because of the prolonged overall survival of melanoma patients. Patients may be rechallenged after a drug‐free interval following adverse drug reactions, after radiation therapy or surgery, following disease progression on subsequent immunotherapy or chemotherapy, or after disease progression without interim therapeutic intervention. To date, only few data has been published on treatment outcomes associated with rechallenge. The articles published on this topic included a total of 238 patients. In general, it was shown that patients did respond to rechallenge, even if they had previously experienced disease progression on targeted therapy. Patient response varied from stable disease to partial response and even complete remission in some cases. Our analysis showed overall response rates to rechallenge of 47 %, with disease control rates of 67 %. While mean progression‐free survival was 6.4 months, this was shorter than after the first round of targeted therapy (mean progression‐free survival of 9.2 months). Rechallenge of targeted therapy offers another option in the management of melanoma patients who have received extensive prior treatment. In particular, it will be important to clarify whether the type of interim treatment has an impact on the response to rechallenge.     

The physics of FeldenkraisPart 3: stability

In the last article in this series, we briefly examined the Principle of Least Effort and the five forms of strain. Strain, you may recall, in physics, describes a change in the volume of a material when a force is applied. Our treatments are a blend of rotation and translation movements of our hands with a changing mix of strains being applied onto the client's tissues. Harnessing and interweaving the various forms of strain with dexterity may aid us in improving our treatment efficacy.In this tissue, we will consider the importance of anchorage and stability in treatment, We will consider a set of ‘House Rules' for improving treatment. In addition, we will explore Bernstein's concept of degrees of freedom.     

Predictors of successful labor induction with oral or vaginal misoprostol

Objective: To identify independent predictors of successful labor induction with oral or vaginal misoprostol.

Methods: Women enrolled in four previous randomized trials involving oral or vaginal misoprostol for cervical ripening and labor induction were included in the present cohort study, with dosing of 25–50?μg every 4 to 6?h vaginally (n?=?574) or 50?μg every 4?h orally (n?=?207). Multiple logistic regression was performed to identify factors independently associated with successful labor induction – defined as vaginal delivery within 12?h, vaginal delivery within 24?h and spontaneous vaginal delivery. Predictors of Cesarean birth and the need for only one dose of misoprostol were also identified. Variables included in the models were maternal age, weight, height, parity, gravidity, membrane status, route of misoprostol, gestational age, birth weight, and Bishop score and its individual components.

Results: Maternal age, height, weight, parity, birth weight, dilatation, effacement and cervical station were associated with vaginal delivery within 24?h of induction. Maternal age, height, weight, nulliparity, birth weight and route of misoprostol were associated with Cesarean birth, with oral misoprostol being associated with a lower rate of Cesarean birth. The need for only one dose of misoprostol was predicted by maternal height, weight, parity, gestational age, Bishop score and route of misoprostol.

Conclusion: Characteristics of the woman (height, weight, parity), the fetus (birth weight) and some of the individual components of the Bishop score, were associated with successful labor induction, with oral misoprostol being associated with a lower rate of Cesarean birth.     

Establishment and characterization of an Epstein-Barr virus transformed cell line with strong phagocytic activity

An immunoglobulin M (IgM)-positive cell line, Ms 28, apparently spontaneously transformed by Epstein-Barr virus (EBV) was established from peripheral blood cells of a patient with immature myeloblastic leukemia. It has been characterized according to phenotype, cytochemistry, and membrane antigen pattern. The cell line expresses lymphoid markers like CD 19, CD 22, and CD 30 and synthesizes and secretes IgM. Monocyte markers CD 11c, CD 14, and CD 15 are absent. Neither interleukin-1 (IL-1), nor tumor necrosis factor (TNF-alpha) are produced. But Ms 28 cells show strong phagocytic activity and engulf Latex particles and sheep RBCs (SRBCs) that need not to be opsonized. The phagocytic activity can be inhibited by chloroquine. Both phagocytosis and EBV nuclear-antigen (EBNA) expression can be observed in one and the same cell. Ms 28 cells might be useful to study immunologic activities like antigen processing and presentation.