Malignant syphilis (MS) is a rare form of secondary syphilis with grotesque skin lesions, systemic manifestation and life-threatening complications. This article presents a case of MS in an ...immunocompetent 41-year-old female, who initially manifested with a generalized nonpruritic erythematous rash and systemic symptoms. She was mistreated for generalized impetigo and hepatitis attributed to chronic alcoholism. After partial recovery and a 3-month latent period, she developed infiltrated plaques with crusts on the trunk, head and neck; pharyngitis and laryngeal lesions; generalized lymphadenopathy and nonspecific systemic symptoms. Serologic tests confirmed syphilis, and cerebrospinal fluid analyses indicated the presence of anti-treponemal antibodies. Urine drug screening was positive for cannabinoids. The polymerase chain reaction from skin biopsy samples identified
, confirmed with Warthin-Starry staining. Immunohistochemical analysis was uncharacteristic. Tertiary syphilis, neurosyphilis, ocular syphilis and otosyphilis were excluded. However, the patient was treated for neurosyphilis with benzylpenicillin (18 million IU intravenously daily, 14 days) and corticosteroids. No Jarisch-Herxheimer reaction occurred. Ten months after treatment, residual scars were visible, and 1 year later, she attempted suicide. Since MS can resemble other diseases, it should be suspected in a mentally ill patient with chronic drug abuse, systemic nonspecific manifestations and dermatological abnormalities, including the head and neck region.
Schnitzler syndrome is a rare acquired autoinflammatory syndrome. It presents with an urticarial rash and a monoclonal gammopathy, usually of the IgM kappa type. In addition, patients can present ...with bone and/or joint pain, recurrent fever, asthenia, weight loss, myalgia, headache, lymphadenopathy, hepatomegaly, or splenomegaly. An elevation of blood inflammation markers is commonly found. Skin biopsy of the urticarial rash reveals neutrophilic infiltrate, known as neutrophilic urticarial dermatosis. To confirm the diagnosis, two sets of diagnostic criteria have been established. The syndrome shares many features with other autoinflammatory disorders, such as adult-onset Still's disease and NLRP3-auto-inflammatory disorders (NLRP3-AID, formerly known as cryopyrin-associated periodic syndromes, or CAPS). The pathogenesis of the disease is not yet fully understood; however, it is believed that interleukin (IL)-1β plays a crucial role and explains the excellent effectiveness of IL-1 blocking agents. It is a chronic disease, and some patients develop lymphoproliferative disease, and seldom AA amyloidosis.
Malignant syphilis (MS) is a rare form of secondary syphilis with grotesque skin lesions, systemic manifestation and life-threatening complications. This article presents a case of MS in an ...immunocompetent 41-year-old female, who initially manifested with a generalized nonpruritic erythematous rash and systemic symptoms. She was mistreated for generalized impetigo and hepatitis attributed to chronic alcoholism. After partial recovery and a 3-month latent period, she developed infiltrated plaques with crusts on the trunk, head and neck; pharyngitis and laryngeal lesions; generalized lymphadenopathy and nonspecific systemic symptoms. Serologic tests confirmed syphilis, and cerebrospinal fluid analyses indicated the presence of anti-treponemal antibodies. Urine drug screening was positive for cannabinoids. The polymerase chain reaction from skin biopsy samples identified T. pallidum, confirmed with Warthin-Starry staining. Immunohistochemical analysis was uncharacteristic. Tertiary syphilis, neurosyphilis, ocular syphilis and otosyphilis were excluded. However, the patient was treated for neurosyphilis with benzylpenicillin (18 million IU intravenously daily, 14 days) and corticosteroids. No Jarisch-Herxheimer reaction occurred. Ten months after treatment, residual scars were visible, and 1 year later, she attempted suicide. Since MS can resemble other diseases, it should be suspected in a mentally ill patient with chronic drug abuse, systemic nonspecific manifestations and dermatological abnormalities, including the head and neck region.
Background. In years 2000–1 European Society of Cardiology performed an »EuroHeart Failure« Survey in 24 European countries, including Slovenia. The aim of the survey was to determine the ...characteristics, diagnostic and therapeutic management of patients, hospitalised for heart failure.Methods. In the prospective survey medical records of all patients admitted during six weeks of April and May 2000 to the University Medical Centre, Internal Medicine Clinic, and Internal medicine departments of General Hospitals Murska Sobota and Slovenj Gradec were screened. Only patients with diagnosis of heart failure on discharge or death were included.Results. Out of 1351 screened we included 358 patients (52% males), aged 73 ± 12 years. Hospitalization index was first hospitalization for heart failure in 41% of patients. On admission, 84% of patients presented with signs of heart failure, 68% were in New York Heart Association class III, and 28% in class IV. Myocardial infarction was recorded in 22%, angina pectoris in 34%, arterial hypertension in 46%, Diabetes mellitus in 34%, respiratory disease in 34%, and renal failure in 28% of patients. During hospitalization (11.6 ± 8.1 days), 11% of patients died and during 12 weeks after discharge additional 7% of them. 26% of patients were rehospitalized in twelve weeks after discharge. Almost all patients had electrocardiogram, chest X-ray and results of basic laboratory tests (full blood count, electrolytes, creatinine). Echocardiography report was found in 57% of patients. Systolic dysfunction was present in 70% of investigations.Conclusions. Heart failure is very common in internal medicine patients. Short term prognosis is gloomy with frequent rehospitalizations. Coronary disease and arterial hypertension are major aetiologies of heart failure, accompanied by many comorbidities. Echocardiography is still not accepted as standard method in diagnostic assessment of heart failure.
Povzetek Izhodisca. Evropsko kardiolosko zdruzenje je v letih 2000-2001 v 24 evropskih drzavah izvedlo raziskavo »EuroHeart Failure« Survey. Njen cilj je bil ugotoviti, kaksne so znacilnosti ...bolnikov, ki so bolnisnicno zdravljeni zaradi srcnega popuscanja, ter kaksna sta njihova diagnosticna obravnava in zdravljenje. V raziskavi je sodelovala tudi Slovenija. Metode. Raziskava je bila prospektivna. V obdobju 6 tednov v aprilu in maju 2000 smo pregledali bolnisnicno dokumentacijo vseh sprejetih bolnikov na Interni kliniki Klinicnega centra Ljubljana ter na internih oddelkih Splosne bolnisnice Murska Sobota in Splosne bolnisnice Slovenj Gradec. V raziskavo smo vkljucili bolnike z diagnozo srcnega popuscanja ob odpustu ali smrti. Rezultati. Od 1351 sprejetih se je zaradi srcnega popuscanja zdravilo 358 bolnikov, starih 73 ± 12 let, med njimi je bilo 52% moskih. Prvic je bilo hospitaliziranih 41% bolnikov. Ob sprejemu so bili znaki srcnega popuscanja izrazeni pri 84% bolnikov. Po klasifikaciji Newyorskega zdruzenja za srce (NYHA) je bilo v funkcijskem razredu III 68%, v IV. pa 28% bolnikov. Srcni infarkt je prebolelo 22% bolnikov, angino pektoris je imelo 34% in arterijsko hipertenzijo 46% bolnikov. Najpogostejse soobolevnosti so bile sladkorna bolezen pri 34%, bolezni dihal pri 34% in ledvicna odpoved pri 28% bolnikov. Bolnisnicno zdravljenje je povprecno trajalo 11,6 ± 8,1 dneva. Med bolnisnicnim zdravljenjem je umrlo 11%, v 12 tednih po odpustu pa se 7% bolnikov. V istem obdobju je bilo v bolnisnico ponovno sprejetih 26% bolnikov. Med diagnosticnimi preiskavami so bili elektrokardiogram, rentgenogram srca in pljuc in osnovne laboratorijske preiskave (krvna slika, elektroliti, dusicni retenti) opravljeni pri domala vseh bolnikih. Izvid ultrazvocne preiskave srca je imelo 57% bolnikov. Med temi je bila sistolicna disfunkcija ugotovljena pri 70%. Zakljucki. Srcno popuscanje je zelo pogosta diagnoza med bolniki, bolnisnicno zdravljenimi na internih oddelkih. Kratkorocna napoved je zanje slaba, pogosta so ponovna bolnisnicna zdravljenja. Glavna vzroka srcnega popuscanja med nasimi bolniki sta koronarna bolezen in arterijska hipertenzija. Pogosta so soobolenja. Ultrazvocna preiskava srca se vedno ni standardna metoda pri diagnosticni obravnavi srcnega popuscanja.
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