[Severe dysphagia and erythrodermia in a 59-year-old man]. / Schwere Dysphagie und flammende Hautrötung bei einem 59-jährigen Patienten.

A 59-year-old man presented with a history of dysphagia and generalized myalgia and muscle weakness and a rash on the face, neck, and upper arms. Serum muscle enzymes, myoglobin, C-reactive protein, and erythrocyte sedimentation rate were elevated and antinuclear antibodies positive. Electromyographic conduction studies showed pathological changes on arm and leg muscles and magnetic resonance imaging of the oral and neck muscles. A diagnosis of dermatomyositis with severe esophageal involvement was established. Treatment with prednisolone was started and methotrexate added. Enteral feeding with a percutaneous endoscopic gastrostomy was started and a therapy with intravenous immunoglobulin (IVIG) initiated, which caused a rapid improvement of the patient's ability to swallow. This case demonstrates a patient with polymyositis/dermatomyositis who showed steroid-resistant life-threatening esophageal impairment. IVIG resulted in a dramatic improvement of symptoms.

[Hepatitis-B reactivation during treatment with tumor necrosis factor-α blocker adalimumab in a patient with psoriasis arthritis]. / Hepatitis-B-Reaktivierung unter TNF-α-Blocker-Therapie mit Adalimumab bei Psoriasis-Arthritis.

HISTORY AND ADMISSION FINDINGS: A 66-year-old man with acute fever, myalgia, arthralgia, restricted movement and neck stiffness was admitted to our emergency unit. Three weeks earlier treatment with adalimumab, a tumor necrosis factor(TNF)-alpha antagonist was started by the patient's dermatologist because of a psoriasis vulgaris with associated psoriasis arthritis. All previous laboratory tests where without pathological findings. INVESTIGATIONS: The imaging procedures showed no pathological findings. Transaminase and CRP levels were markedly elevated. Hepatitis serology revealed reactivation of a chronic hepatitis B infection, while a combined viral and toxic hepatitis was detected by liver biopsy. TREATMENT AND COURSE: On the day of admittance acute neurological symptoms developed including muscular cramps and dyskinesia. Hypotonia and tachyarrhythmia were treated with parenteral infusions and digitoxin. Reactivation of a chronic hepatitis B infection with subfulminant liver failure was diagnosed, caused by immunosuppressive therapy with adalimumab. The patient was transferred to be treated with the antiviral drug entecavir. CONCLUSION: The exclusion of acute and/or chronic hepatitis before using TNF-alpha antagonists is recommended but in daily routine often ignored. The presented case demonstrates the risk of reactivating a chronic hepatitis B during therapy with a TNF-alpha antagonist.

[Butterfly rash: no lupus]. / Schmetterlingsförmiger Hautausschlag: kein systemischer Lupus erythematodes.

A 61-year-old male patient presented with petechiae on the arms and legs due to a thrombocytopenia of 3 Gpt/l (3000/microl). A butterfly rash on the patient's face suggested a diagnosis of systemic lupus erythematosus (SLE). The thrombocytopenia was due to autoimmune thrombocytopenia. The diagnosis of SLE could be excluded and the butterfly rash attributed to a laminar hemorrhage, an ecchymosis due to the autoimmune thrombocytopenia.

Clinical correlation of antimitochondrial antibodies.

BACKGROUND: Antimitochondrial antibodies (AMA) are a hallmark of primary biliary cirrhosis (PBC). They are believed to be absolutely disease specific. It does occur that patients with positive AMA are diagnosed with PBC in the absence of liver specific signs and symptoms. The aim of the present study was to examine the disease spectrum of unselected AMA positive patients of an university hospital. METHODS: All of the AMA tests performed in the immunological laboratory of the hospital between 1992 and 1998 were examined for positivity. 100 patients with a positive result were analyzed retrospectively for diagnosis, clinical and laboratory features. RESULTS: 61 patients suffered from liver diseases and 39 from non-liver diseases. The patients with liver diseases were 36 patients with PBC, 2 patients with PBC/PSC-overlap syndrome, 4 patients with autoimmune hepatitis and 19 patients with different liver diseases of other than autoimmune origin. The 39 patients with non-liver diseases included 9 patients with systemic autoimmune diseases, 3 patients with organ-specific autoimmune diseases, 8 patients with carcinoma and 19 patients with different diseases. 97 patients had an ELISA test for antibodies to the mitochondrial antigen M2 performed in addition to the immunofluorescence test for AMA. 73 patients had positive values for anti-M2 antibodies and 24 patients had negative results. Anti-M2 antibody values were divided in negative, low (5-100 U/ml), medium (101-1000 U/ml), high (1001-10000 U/ml) and very high (>10000 U/ml). Very high and high anti-M2 values were present mainly in patients with PBC and some patients with other liver diseases, medium high and low values in patients with different disease groups. In this unselected patient population only one third of AMA positive patients had an established diagnosis of PBC, about 10% a diagnosis of a systemic autoimmune disease and 3 % had other organ-specific autoimmune diseases. It can be concluded that, although high titers of antibodies against M2 are present mainly in patients with PBC, medium high and low titers may be present in patients lacking symptoms of PBC. CONCLUSIONS: Although there is the possibility that the patients will develop the disease in the future, the presence of AMA alone does not seem to be sufficient to establish a diagnosis of PBC in a patient population in which at a given time point only 38 % of AMA positive patients have the disease.

Characterization of chromosomal aberrations in a case of glioblastoma multiforme combining cytogenetic and molecular cytogenetic techniques.

A case of glioblastoma multiforme (GBM) that was investigated with a broad spectrum of cytogenetic and molecular cytogenetic techniques is reported. The results of cytogenetic studies, interphase fluorescence in situ hybridization, comparative genomic hybridization, and spectral karyotyping (SKY) are reported. Various structural chromosomal aberrations were identified, among which aberrations involving chromosome arm 2p were especially frequent. Using SKY, six translocations not previously described in GBM are reported.

[Abdominal tuberculosis: a rare differential diagnosis of pancreatic carcinoma]. / Abdominale Tuberkulose. Eine seltene Differenzialdiagnose zum Pankreaskarzinom.

HISTORY AND ADMISSION FINDINGS: A 79-year-old local resident, presenting with abdominal pain, sweating and weight loss and suspected of having cancer of the pancreas was referred for diagnosis and treatment. Physical examination was negative except for pain on pressure over the right upper abdomen and the epigastrium. INVESTIGATIONS: Erythrocyte sedimentation rate was increased; as were the transaminases and cholestasis parameters. Ultrasonography and computed tomography of the abdomen revealed an echo-poor mass with cystic areas in the region of the head of the pancreas, as well as extra- and intrahepatic dilatation of the biliary tract. Endoscopic retrograde cholangiopancreatography failed to demonstrate a ductal pancreatic carcinoma. Biopsies of a macroscopically peculiar-looking duodenal ulcer demonstrated a noncaseous epithelioid granuloma. A fine-needle biopsy was performed for further diagnosis. DIAGNOSIS, TREATMENT AND COURSE: Histological examination of the needle biopsy revealed a caseous granuloma and acid-fast bacteria. The tuberculin test (GTI) was strongly positive (14-15 mm), indicating tuberculosis of the pancreas and duodenum. Multiple tuberculostatics rapidly improved the patient's symptoms, and the further course was without complications. CONCLUSION: Tuberculosis should be included in the differential diagnosis of consumptive disease with an atypical presentation, especially because treatment could well be curative.

[Underrepresentation of women among peer reviewers and textbook authors in medicine in Germany]. / Unterrepräsentation von Frauen bei Gutachtern und Lehrbuchautoren in der Humanmedizin in Deutschland.

BACKGROUND AND OBJECTIVE: A characteristic feature of German science is the "disappearance" of women during the different ranks from high-school to professorship. The goal of the present study was to determine the proportion of women among peer reviewers of the German research council and among editors and authors of textbooks in internal medicine. METHODS: The candidate list for the election of the peer reviewers of the German research council in November 1999 was checked for the participation of women. Numbers and proportion of women among the reviewer boards for practical medicine, theoretical medicine, biological chemistry/biophysics and biology were determined. Textbooks of internal medicine in German language were checked for female editors and authors. RESULTS: 1.8% of the present reviewers and 4.3% of the candidate reviewers for the election of the reviewer board for practical medicine were female. The corresponding numbers for the reviewer board for theoretical medicine were 3.2% and 8.2%, respectively. The reviewer board for biological chemistry and biophysics had no woman among the present reviewers and 2.1% among the candidate reviewers. Corresponding proportions for the reviewer board for biology are 18.5% and 13.5%, respectively. The average percentage of women among all 4 reviewer boards which are involved in biomedical research is 5.4% among the present reviewers and 6.5% among the proposed reviewers. A similar situation of underrepresentation of women is present among authors of medical textbooks. Among 65 editors and co-editors of 19 textbooks of internal medicine was no woman at all. Among a total of 1092 authors 47 women corresponding to 4.3% were found. CONCLUSION: These numbers demonstrate that the subject of gender discrimination in higher ranks in medicine and science in Germany is far from being solved.

[Analysis of the proportion of women at different qualification levels in departments of human medicine in Germany]. / Analyse des Frauenanteils bei verschiedenen Qualifikationsstufen des Fachgebietes Humanmedizin in Deutschland.

BACKGROUND AND OBJECTIVE: In Germany leadership positions in medicine are characterized by an almost complete lack of women. The goal of this study was to determine the representation of women among different career ranks during medical school, residency and academic advancement during the past 20 years. METHODS: The annual reports of the German Federal Statistic Office in Wiesbaden were checked for the numbers of medical students, dissertations, physicians, internists, fellows and trainees at universities, habilitations and professorships. RESULTS: Among medical students, final examination, dissertation, physicians, internists and trainees at universities a proportion of women can be found nowadays which is at a 15% higher percentage level than 20 years ago. Among assistant professors and habilitations at universities there is a very low proportion of women, which did increase only slightly from 5 to 10% during the past 20 years. Within the same period of time the total number of professors and the proportion of female professors were nearly unchanged. Reunification of East and West Germany led to an increase of the representation of women among all levels of qualification which is due to the much higher proportion of female physicians in the former GDR. CONCLUSIONS: The lack of women in leadership positions in medicine in Germany is not due to a general lack of women in medicine. Among all other career ranks the proportion of women increased by 15%. The completely unchanged number and composition of the professors during the past 20 years suggests that the lack of women among this professional group has to do with conservatism which is an inherent trait of university professors in Germany.