The case report literature on ulcus vulvae acutum Lipschütz (UVAL) is scant, and specific guidelines on its diagnosis and treatment are lacking. Our study's aim was to perform a systematic literature ...review of UVAL in order to formulate a diagnostic and therapeutic algorithm. Using the PRISMA criteria, we searched PubMed and MEDLINE for the terms ‘ulcus vulvae acutum’, ‘Lipschütz ulcer’ and ‘acute genital ulcer AND vulva’. We extracted relevant data on ‘type of article’, ‘patients’ age’, ‘amount and localization of ulcers’, ‘presence of flu‐like symptoms’, ‘prior sexual contacts’, ‘diagnostic workup’ (including histology, blood count and serology such as Epstein–Barr virus testing) and ‘treatment/outcome’. Data were meta‐analysed and comparative analyses were discussed in order to create a diagnostic algorithm and recommendations for management. Twenty‐one publications reporting a total of 60 cases of UVAL were included for analysis. On this basis, we formulated a diagnostic and therapeutic algorithm defined by two major and four minor criteria. The major criteria were (i) acute onset of one or more painful ulcerous lesions in the vulvar region and (ii) exclusion of infectious and non‐infectious causes for the ulcer. The minor criteria were (i) localization of ulcer at vestibule or labia minora, (ii) no sexual intercourse ever (i.e. patient was a virgin) or within the last 3 months, (iii) flu‐like symptoms and/or (iv) systemic infection within 2–4 weeks prior to onset of vulvar ulcer. Use of a symptom‐based treatment algorithm based on our proposed major and minor criteria will improve the diagnosis and management of UVAL.
Chronic graft-versus-host disease (GVHD) remains a serious complication after allogeneic hematopoietic stem cell transplantation (HCT). In 2005 the National Institutes of Health (NIH) established new ...criteria for chronic GVHD based on retrospective data and expert recommendations. We prospectively evaluated the incidence of NIH-defined chronic GVHD and its prognostic impact in 178 consecutive patients. The cumulative incidence of chronic GVHD at 3 years was 64, 48 and 16% for chronic classic GVHD and overlap syndrome. Prior acute GVHD and myeloablative conditioning were significantly associated with increased risk of chronic GVHD. Three-year survival (overall survival (OS)) for late-acute GVHD, chronic classic and overlap chronic GVHD when assigned on day 100 were 69, 83 and 73%. OS was significantly worse for patients with platelet counts below 100 g/l at onset of chronic GVHD (35% versus 86%, P<0.0001) and progressive as compared with de novo and quiescent onset of chronic GVHD (54.5% versus 89.5% versus 84%, P = 0.022 and 0.001). Peak severity of chronic GVHD had no impact on non-relapse mortality (NRM) and OS. Recurrent acute GVHD, platelet counts below 100 g/l at diagnosis of chronic GVHD, progressive onset of chronic GVHD and advanced disease stage prior to HCT were significantly associated with increased NRM. This prospective analysis provides for the first-time data on the incidence rates of NIH-defined chronic GVHD categories and identified risk factors for the occurrence of chronic GVHD. A prognostic value of thrombocytopenia and progressive onset type of chronic GVHD for survival after HCT was observed in NIH-defined chronic GVHD.
Summary
Supportive therapy plays a central role in the management of cutaneous and musculoskeletal manifestations of chronic graft‐versus‐host disease (cGVHD), either alone or in combination with ...systemic approaches. We present results from the German–Austrian–Swiss Consensus Conference on clinical practice in cGVHD, held in Regensburg, Germany, in November 2009. The intention was to achieve a consensus on current evidence‐based treatment options as well as to provide guidelines for daily clinical practice. Skin is the most common organ involved in cGVHD. Its clinical presentation varies considerably. Patients may have pruritus, rash, pain, dyspigmentation and fibrotic or sclerodermatous lesions, often leading to contractures. Treatment options for supportive therapy in cutaneous cGVHD include topical therapies such as topical steroids and topical calcineurin inhibitors, as well as phototherapy and physiotherapy. The most relevant manifestation in musculoskeletal cGVHD is fasciitis which must be distinguished from sclerodermatous skin cGVHD. Physiotherapy is the mainstay of supportive treatment in fasciitis in cGVHD. Successful therapy of cutaneous and musculoskeletal cGVHD depends on interdisciplinary management to improve patients’ quality of life.
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