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Many systemic diseases go along with dermal involvement. Only a few of systemic diseases show characteristic skin derangements directly leading to the diagnosis of the underlying disease. Ten of these entities are described within this article. Some of these are autoimmune diseases, such as systemic lupus erythematodes, dermatomyositis or the anti-synthetase-syndrome. Others are genetic diseases with benign or malignant neoplasias (Peutz-Jeghers-syndrome, neurofibromatosis, tuberous sclerosis, Birt-Hogg-Dubé-syndrome, Cowden-syndrome). Other genetically based diseases with typical skin manifestations are characterised by vascular malformations (Pseudoxanthoma elasticum and Osler-Weber-Rendu syndrome) or metabolic and structural organ defects (Morbus Fabry, cystic fibrosis). Being familiar with the typical skin-appearance of these diseases will allow physicians and general practitioners to make a timely diagnosis., Competing Interests: Disclosure The authors report no conflicts of interest in this work., (© Georg Thieme Verlag KG Stuttgart · New York.)

Pruritus (itch) is the most frequent skin symptom. It can be attributed to a skin disease in many cases. Furthermore pruritus may also be a precursing symptom or principal complaint of an internal, neurological or psychiatric disease. It has been observed that elderly patients are increasingly affected. In many cases pruritus has multifactorial causes. This requires careful diagnostics, including a detailed medical history and medical examination of the patient. Previous diseases and comorbidities need to be considered as possible causes, as well as current and previous drug intake. By this important information can be obtained at an early time, which may lead to further investigation. Aim of rational diagnostics is to evaluate especially internal diseases which need to be assessed by laboratory testing and more specific diagnostics. Due to the multitude of possible causing diseases, diagnosis and therapy of chronic itch need an interdisciplinary approach., (© Georg Thieme Verlag KG Stuttgart · New York.)

History and Clinical Findings: A 20 year old, previously healthy woman presented with a four week history of progressive oedema of the legs and the eyelids and a weight gain of 10 kg., Investigations: Biochemical tests revealed a nephrotic syndrome with a protein-loss in urine of 13.6 g/24 hours and a serum-albumin of 1.2 g/dl. Serological tests showed positive response for antinuclear antibodies, anti-double-stranded-DNA antibodies and cardiolipin antibodies. Renal histology revealed a lupus-associated diffuse membranous nephropathy (WHO-type Vd)., Diagnosis, Treatment and Course: We first started a treatment regimen with oral steroids and intravenous loop-diuretics without improvement of the nephrotic syndrome. Two weeks after initial presentation the patient developed dialysis-dependent, acute renal failure. Having excluded a renal vein-thrombosis and the transition to diffuse proliferative form of the glomerulonephritis we suggested a nephrotic etiology of the acute renal failure. We initiated cyclophosphamide pulse-therapy which led to dramatic improvement of the nephrotic syndrome after the first cycle with reduction of protein-loss to 6 g/24 hours. Concurrently renal function recovered and treatment with hemodialysis could be stopped after 3 weeks., Conclusion: Acute renal failure in nephrotic syndrome has also to be considered in an acute form of a membranous lupus nephritis. Renal recovery is much better when acute renal failure is caused by nephrotic syndrome.

History and Clinical Findings: A 35-year-old previously healthy man developed a painful swelling in the area of the left sternoclavicular joint (SCJ), restricting movement. There had been no history of trauma., Investigations: A moderately increased erythrocyte sedimentation rate was the only abnormal finding among the usual laboratory tests. Radiologically there were marked hyperostotic changes in the area of the left upper thoracic cage with synostosis of the SCJ. Biopsy of the left clavicle showed non-specific chronic sclerosing osteomyelitis., Treatment and Course: Over the next 6 years the SCJ became completely ankylosed and there were now extensive fibroses, some presternal and some in the upper mediastinum with thrombosis of the left subclavian, axillary and jugular veins causing inflow occlusion (Paget-von-Schroetter-syndrome). Lymph nodes in the region of the mandible, jugular veins bilaterally and mediastinum were noted for the first time, remaining unchanged in size over six months., Conclusion: Sternoclavicular hyperostosis is an important condition in the differential diagnosis of inflammatory or malignant processes of this joint.

A 71-year-old man with an aortobifemoral bypass graft had experienced several episodes of severe pain in the thoracic and lumbar spine, followed by severe abdominal pain, during the preceding two weeks. Physical examination, chest X-ray, ultrasonography and computed tomography of the abdomen provided no specific pointers to a diagnosis. He was anaemic (haemoglobin 95 g/l), but had a normal WBC count with neutrophilia (up to 91%) and thrombocytopenia (up to 24,000/microliters). The transaminase and lactate dehydrogenase activities were raised, as were the bilirubin level and the retention values. There was a metabolic acidosis (pH 7.20). Because of suspected septicaemia ciprofloxacin (twice daily 200 mg) was administered intravenously. But the patient gradually lost consciousness, required mechanical ventilation and died 24 hours after admission in circulatory failure. Autopsy revealed a meningoencephalitis, carcinoma of unknown primary with bone marrow carcinomatosis as well as widespread carcinomatosis of blood and lymph vessels. The findings of blood, urine and sputum cultures, available only postmortally, revealed growth of tetracycline-resistant Salmonellae enteritidis.

A 37-year-old man suddenly fell ill with high fever (up to 39.6 degrees C), headache and lumbar pain. There was marked thrombocytopenia (minimal level of 48,000/microliters), moderate anaemia and a slow rise in serum lipase concentration to maximally 1352 U/l. Marked sinus bradycardia (to as low as 34 beats/min) occurred in the further course of the disease, as well as upper abdominal pain (endoscopically diagnosed as antral gastritis), subileus and splenomegaly. Two haemodialysis treatments were needed because of acute renal failure. An IgG antibody titre of 1:512 and an IgM titre of 1:80 against hantavirus antigen confirmed that this virus was responsible for the haemorrhagic fever with renal syndrome. This infection, transmitted by wild rodents and frequently observed in Asian countries, has a rising incidence in Central Europe. It should be included in the differential diagnosis of acute renal failure of uncertain cause.

From 1979 to 1989, continuous ambulatory peritoneal dialysis (CAPD) was undertaken for terminal renal failure in 104 patients (56 women and 48 men; average age 54 +/- 15.3 years at the onset of dialysis), for a total observation period of 175 patients years. Survival rate for patients and methods and dialysis effectiveness were analysed retrospectively, the incidence of peritonitis prospectively, 40 patients were aged 60 years and over. Diabetic nephropathy was the most common cause of terminal renal failure (44%). Cumulative patient survival rate was 80% in the first year of treatment; 57% of patients were still alive after two years. The cause of death in 45 of the 54 patients who had died was unrelated to CAPD, cardiac disease and cerebrovascular accident being the most frequent causes (n = 26). During the first treatment year 47% of patients contracted bacterial peritonitis, 59% during the first two years. In 9% of patients CAPD had to be discontinued within the first two treatment years because of CAPD-related complications. There was no case of sclerosing peritonitis or of ultrafiltration loss forcing CAPD termination. These data indicate that there is no plausible explanation from a medical viewpoint for the highly restrictive use of CAPD in the Federal Republic of Germany.