BACKGROUND: Several European countries recently developed international diagnostic and management guidelines for pemphigus, which have been instrumental in the standardization of pemphigus management. OBJECTIVE: We now present results from a subsequent Delphi consensus to broaden the generalizability of the recommendations. METHODS: A preliminary survey, based on the European Dermatology Forum and the European Academy of Dermatology and Venereology guidelines, was sent to a panel of international experts to determine the level of consensus. The results were discussed at the International Bullous Diseases Consensus Group in March 2016 during the annual American Academy of Dermatology conference. Following the meeting, a second survey was sent to more experts to achieve greater international consensus. RESULTS: The 39 experts participated in the first round of the Delphi survey, and 54 experts from 21 countries completed the second round. The number of statements in the survey was reduced from 175 topics in Delphi I to 24 topics in Delphi II on the basis of Delphi results and meeting discussion. LIMITATIONS: Each recommendation represents the majority opinion and therefore may not reflect all possible treatment options available. CONCLUSIONS: We present here the recommendations resulting from this Delphi process. This international consensus includes intravenous CD20 inhibitors as a first-line therapy option for moderate-to-severe pemphigus.
- MeSH
- akademie a ústavy normy MeSH
- antigeny CD20 imunologie MeSH
- delfská metoda MeSH
- dermatologie metody normy MeSH
- glukokortikoidy aplikace a dávkování MeSH
- imunologické faktory aplikace a dávkování MeSH
- intravenózní podání MeSH
- kombinovaná farmakoterapie metody normy MeSH
- kombinovaná terapie metody normy MeSH
- konsensus MeSH
- lidé MeSH
- pemfigus diagnóza imunologie terapie MeSH
- plazmaferéza * MeSH
- rituximab aplikace a dávkování MeSH
- směrnice pro lékařskou praxi jako téma * MeSH
- stupeň závažnosti nemoci MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- Geografické názvy
- Evropa MeSH
Anti-basement membrane zone (anti-BMZ) antibodies are detectable in a low percentage of elderly subjects without clinical signs of bullous pemphigoid (BP). BP may initially mimic other pruritic dermatoses and may be more common in patients with diabetes mellitus (DM), since DM is frequently associated with pruritic disorders. The aim of the present study was to analyse a possible association of BP and DM and to detect subclinical BP among elderly patients with pruritic dermatoses. Ninety elderly patients (78.6 +/- 4.7 years) treated for dermatologic conditions were divided into four groups: I. DM+/pruritus+, II. DM-/pruritus+, III. DM+/pruritus-, and IV. DM-/pruritus-. Patients' sera were tested by indirect immunofluorescence (IIF) on monkey oesophagus; positive or dubious results were further evaluated by ELISA with human recombinant BP180 and BP230 proteins and purified laminin 5. Positive results were found in 1 of 21 (4.8%) patients in group I, 6/31 (19.3%) patients in group II, 1/18 (5.5%) patients in group III, 3/20 (15%) patients in group IV. In the whole cohort positive anti-BMZ antibodies of linear or basal cell cytoplasmic and membrane pattern were found in 11 cases (12.2%). ELISA was positive in 11/29 (37.9%) tested sera for at least one antigen (BP180, BP230 and laminin 5 ELISA was positive in 7, 5, and 2 sera, respectively). Positive IIF corresponded with positive ELISA in 6/11 (54.5%) cases (ELISA with BP180, BP230, laminin 5 was positive in 5, 3, and 1 serum, respectively). Thus, by IIF, a significant portion of elderly patients had anti-BMZ antibodies and these findings were confirmed by ELISA. There was no statistically significant difference in the presence of anti-BMZ antibodies among the groups I-IV. Thus, the association of anti-BMZ antibodies with age overrules the potential association with DM and/or pruritus.
Epidermolysis bullosa acquisita (EBA) is an autoimmune bullous disorder with mucocutaneous involvement, skin fragility, and tendency to scarring. The mechanobullous form of EBA has a chronic relapsing course and is difficult to treat. We describe herein the therapeutic response of 2 patients with recalcitrant mechanobullous EBA to combined treatment with immunoadsorption and rituximab, an anti-CD20 monoclonal antibody that induces depletion of B cells in vivo. OBSERVATIONS: Two patients with mechanobullous EBA received combined treatment with immunoadsorption and rituximab, resulting in an almost complete clinical remission in one patient and stable disease in the other patient. In the patient with complete remission, prolonged B-cell depletion and clinical improvement with disappearance of mucocutaneous erosions paralleled the decline in titers of circulating anti-basement membrane zone autoantibodies. In the other patient, combined treatment with immunoadsorption and rituximab reduced the de novo appearance of blisters but did not lead to significant improvement of gingivitis, despite depleted B cells for 6 months that remained at 5% 12 months after the last administration of rituximab, as well as a reduction in autoantibody titers. CONCLUSION: The patients' response suggests that combined treatment with immunoadsorption and rituximab may be a valuable adjuvant treatment regimen for severe mechanobullous EBA, which is in line with recently observed beneficial effects in inflammatory EBA.
2nd, revised and enlarged ed. xviii, 469 s. : barev. obr.
1st ed. 17, 373 s.
2., überarbeitete Auflage xx, 661 stran : ilustrace, 28 cm
Ein Universitätslehrbuch mit Schwerpunkt auf der Differentialdiagnose in der Pädiatrie.
- Konspekt
- Pediatrie
- Učební osnovy. Vyučovací předměty. Učebnice
- NLK Obory
- pediatrie
- NLK Publikační typ
- učebnice vysokých škol
6.ueberarb.,erweit.Aufl. 529 s.
161 s. : il.
Theoretische und klinische Medizin in Einzeldarstellungen ; Bd. 30
135 s. : il.
