Granulomatous rosacea: Like leukemid in a patient with acute myeloid leukemia

Introduction. Skin findings in leukemias may be divided into specific lesions (leukemia cutis) and non-specific lesions (leukemids) which may be found in up to 80% of all patients with leukemias. The leukemids vary clinically and they are usually a manifestation of bone marrow or immunologic impairment, but also Sweet syndrome, pyoderma gangrenosum, erythroderma, maculopapular exanthema, prurigo-like papules, generalized pigmentation, follicular mucinosis, generalized pruritus may be found during the course of leukemia. Case report. We report a 70-year-old male with a 3-month history of erythema, papules and pustules on the face, ears and neck and over a month history of refractory anemia, anorexia, weight loss, malaise, and fever. Physical examination revealed symmetric erythematous, violaceous papules, papulo-nodules and plaques with slate scale and sparse, small pustules on the face, earlobes and neck. Histopathologic findings of involved skin showed diffuse mixed inflammatory cell infiltrate with perifollicular accentuation and focal granulomatous inflammation in the papillary and upper reticular dermis. Extensive checkup revealed the presence of acute myeloid leukemia French- American-British (FAB) classification subtype M2, with signs of three-lineage dysplasia. The patient was treated by L6 protocol which led to complete remission, both in bone marrow and skin, but after seven months he had relapse of leukemia with the fatal outcome. Conclusion. This case indicates the importance of skin eruptions in the context of hematological malignancies

Atypical pyoderma gangrenosum in a patient with osteomyelofibrosis

Background. Atypical forms of pyoderma gangrenosum generally appear on the upper extremities; most frequently they are associated with myeloproliferative disorders, including osteomyelofibrosis. A response to systemic steroids is more pronounced than in classical form. Sometimes it may be the first sign of an underlying malignancy. Case report. We reported a patient with atypical pyoderma gangrenosum developed during the course of a myeloid malignancy - osteomyelofibrosis. The lesions occurred after a minor trauma. Painful blistering plaques, with an elevated, bluish-gray border were located on the dorsal aspect of hands. No skin malignancy was found. The lesions resolved rapidly to systemic steroids. Conclusion. Considering the unusual clinical presentation which makes the diagnosis difficult, as well as the fact that atypical forms of pyoderma gangrenosum can be the first sign of malignancies, especially myeloproliferative ones, recognizing this entity enables timely guiding future investigations toward their prompt detection

Correlation of antibodies against desmogleins 1 and 3 with indirect immunofluorescence and disease activity in 72 patients with pemphigus vulgaris

The enzyme-linked immunosorbent assay (ELISA) and indirect immunofluorescence (IIF) have both been used for testing of antibodies to desmogleins 1 and 3 (anti-Dsg1 and anti-Dsg3) and for the serologic diagnosis of pemphigus. IIF values and antibody concentrations and profile do not always correlate with a specific clinical phenotype and with the disease activity. The purpose of the present study was to correlate the clinical phenotype of patients with pemphigus vulgaris (PV) and the disease activity with anti-Dsg1 and anti-Dsg3 antibodies and IIF titers. A total of 72 patients with PV underwent ELISA serum testing for the presence and titers of anti-Dsg1 and anti-Dsg3 and IIF which were correlated with the severity of the disease (evaluated using the Pemphigus Disease Area Index, PDAI), clinical phenotype, and clinical course. In 79.2% patients there was a perfect correlation between the clinical phenotype and antibody profiles; in 20.8% patients, clinical features and antigenic findings were discordant. A statistically significant correlation was found between disease activity and a) anti-Dsg3 and anti-Dsg1 concentrations (Rho=0.679, P&lt;0.001 and Rho=0.363, P=0.02, respectively) and b) IIF titers (Rho=0.426, P&lt;0.01), as well between IIF titers and anti-Dsg3 and anti-Dsg1 antibodies (Rho=0.742, P&lt;0.01 and Rho=0.372, P=0.02, respectively).  This study supports the previous observations that the disease severity in most patients with pemphigus correlates with IIF titers, which in turn is determined by the quantities of Dsg1 and Dsg3 antibodies, as well as the previous observation that the clinical phenotype and antibody profile are not always in correlation. </p

Kvalitet života bolesnika s venskim ulkusima

Introduction. Chronic venous disease has been shown to have a significant impact on patients' quality of life (QoL). Objective. The aim of this study was to estimate the impact of chronic venous insufficiency (CVI) on QoL in patients with terminal stages of HVI, classified according to the CEAP clinical classification into classes C5 (healed ulcers) and C6 (active ulcers), on admission and after applied therapy. Methods. A cross-sectional study performed between October 2007 and June 2008 in a Belgrade outpatient clinic involved a total of 82 patients with venous ulcers (38 C5 and 44 C6) examined at the beginning of therapy. Of these, 14 C5 and 15 C6 patients in remission were re-examined after therapy from November 2007 to January 2010. QoL was assessed using a standard short-form (SF-36) questionnaire, and additionally by a brief CVD questionnaire specific for chronic venous disease. Results. At the beginning of therapy the SF-36 scores showed significant (p lt 0.05) reductions in all QoL domains of C5 and C6 patients regarding physical, general health, and vitality in C5 and C6 patients. After therapy QoL was significantly improved in both classes of patients, but remained unchanged in the domain of emotional functioning suggesting the patients' fear and worry of HVI progression. Conclusion. In patients with terminal stages of CVI QoL was decreased at the beginning of therapy, but with the remission of the disease and ulcer healing it was significantly improved. This indicates the significance of prevention and timely treatment, and the need for patients' education about the chronic nature of the disease.Uvod. Istraživanja vršena poslednjih decenija pokazuju da hronično oboljenje vena i hronična venska insuficijencija (HVI) nepovoljno utiču na kvalitet života bolesnika. Cilj rada. Cilj studije bio je da se utvrdi uticaj HVI na kvalitet života bolesnika u terminalnoj fazi HVI, svrstanih prema klasifikaciji CEAP u klasu 5 (zarasli ulkusi) i klasu 6 (aktivni ulkusi), na prijemu i posle primenjene terapije. Metode rada. Istraživanje je izvedeno u dve faze. Prva faza je dizajnirana kao studija preseka u kojoj su od oktobra 2007. do juna 2008. godine ispitana 82 bolesnika s venskim ulkusima (38 je pripadalo klasi 5, a 44 ispitanika su pripadala klasi 6). U drugoj fazi, izvedenoj od novembra 2009. do januara 2010. godine, 14 ispitanika klase 5 i 15 bolesnika klase 6 iz prve faze ispitano je nakon primenjene terapije, u periodu remisije HVI. Kvalitet života je ocenjen primenom standardnog upitnika SF-36 i originalnog upitnika specifičnog za hronično oboljenje vena. Rezultati. Svi ispitanici su na početku lečenja imali značajno slabiji kvalitet života u domenima onesposobljenosti zbog fizičkog zdravlja, opšteg zdravlja i vitalnosti. Nakon primenjene terapije, njihov kvalitet života se značajno poboljšao u svim domenima zdravlja, ali je ostao nepromenjen u domenu emotivnog funkcionisanja. Nepromenljivost ovoga domena u obe klase bolesnika ukazuje na njihovu zabrinutost i strah od napredovanja oboljenja. Zaključak. Kvalitet života bolesnika u terminalnim fazama HVI je smanjen na početku lečenja, ali se s remisijom bolesti i zarastanjem ulkusa značajno poboljšava. Ovo ukazuje na značaj edukacije bolesnika o hroničnoj prirodi oboljenja i potrebi za prevencijom i blagovremenim lečenjem

Imunski sistem uretre muškaraca

Sluznica muške uretre je inicijalno mesto infekcije brojnim patogenima koji su prenosivi seksualnim putem. U njenim donjim segmentima ce odvijaju složene aktivnosti vezane za kontrolu infekcije i sprečavanje njenog širenja prema gornjim segmentima muškog genitalnog trakta. Sluznica urogenitalnog trakta i kod muškaraca i kod žena sagledava ce kao integralni deo svekolikog mukoznog imunskog sistema, ali i kao imunološki samosvojni induktivni prostor sa specifičnim mehanizmima ugrađivanja i procesiranja antigena i pokretanja sekretornih imunskih odgovora. U velikoj meri neispitan, imunoglobulinski sistem muške uretre je predmet naših istraživanja i povod za ovaj osvrt na novija saznanja o mukoznom imunitetu, jednom od uzbudljivih polja savremenih istraživanja u imunobiologiji