[Successful long-term therapy of Stewart-Bluefarb syndrome]. / Erfolgreiche Langzeittherapie bei Stewart-Bluefarb-Syndrom.

The Stewart-Bluefarb syndrome is defined as an unilateral angiodermatitis due to multiple arterio-venous fistules accompanied by acroangiodermatitis resembling Kaposi sarcoma (pseudo-kaposi sarcoma). The acroangiodermatitis is most common on the lower limb. It leads to ulcerated nodules with a high risk of bleeding and infection, as well as edema, pain and seldom limb hypertrophy. Curative therapy requires elimination of the arteriovenous shunts. Surgical destruction of the multiple small fistulae is a limitating factor. A better alternative is embolisation, but this approach carries the risk of ischemia and necrosis. A 32 year old female patient with Stewart-Bluefarb syndrome is presented; she has been successfully treated with embolisation on eight occasions.

[Biker's nodule"--perineal nodular induration of the cyclist]. / "Biker's nodule"--die perineale knotige Induration des Radsportlers.

The "biker's nodule" is a rarely appearing perineal nodular induration of the cyclist. Repeated microtrauma to the subcutaneous fatty tissue or collageneous tissue, caused by pressure or vibration which the bicycle's saddle exerts on the perineal region, leads to collagenous degeneration, myxoid alteration and pseudocyst formation. Because of the painful subcutaneous nodules the patient is often forced to reduce or even give up his training. Therapy of the "biker's nodule" consists in avoiding the pathogenetic factors, i.e. giving up training for a temporary period of time and reducing pressure on the perineal region. Intralesional injection of either hyaluronidase or corticosteroids may also be helpful.

Non-human immunodeficiency virus Kaposi's sarcoma can be effectively treated with low-dose interferon-alpha despite the persistence of herpesvirus-8.

Three patients, negative for human immunodeficiency virus (HIV), with histologically and polymerase chain reaction-proven non-HIV Kaposi's sarcoma who received low-dose interferon (IFN) as first-line treatment because of disseminated symptomatic disease are reported. Applying 3-18 million IU of IFN-alpha 2a per day, 3 days a week, subcutaneously for 8-20 months, major responses were achieved in all three cases. Tumour regression was observed within 4 months and has continued for 57 and 18 months to date (cases 1 and 2, respectively). Influenza-like symptoms, including fever, headaches and fatigue, were mild side-effects. However, in the third patient interferon injections had to be stopped because of hepatic enzyme elevation. Including this case report, 27 non-HIV Kaposi's sarcoma patients subcutaneously treated with IFN-alpha have been reported in literature. Most therapy regimens included 3-18 million IU IFN-alpha per day for 3 days a week. Twenty of 27 patients, or 74%, responded to therapy, whereas seven patients or 26% had stable or progressive disease. Relapse after IFN withdrawal can occur but is frequently delayed and limited, as in case 1. Following the response to IFN treatment, human herpesvirus-8 DNA was detected in the blood mononuclear cells of all three patients, possibly contributing to future relapses.

Specific catalytic activity of cathepsin S in comparison to cathepsins B and L along the rat nephron.

Assay conditions were elaborated to determine the catalytic activity of cathepsin S fluorometrically for direct comparison with the activities of cathepsins B + L(+S) and B along the nephron of the normal rat. These conditions include the use of 0.5 mM Z-Phe-Arg-AMC as substrate, which is saturating for the three enzymes. The stability of cathepsin S at pH 7.5 and the resistance of cathepsin B against inactivation by 0.5 microM Z-Phe-Phe-CHN2 permitted differentiation of these enzyme activities. The catalytic activity of cathepsin S in rat kidney homogenate (1.11 mumol/min x g protein) amounted to 2.1% of that of cathepsins B + L(+S) and to 3.2% of that of cathepsin B. It was ten-fold higher in the cortex (1.54 mumol/min x g protein) than in the medulla resembling the activity ratio of cathepsins B + L(+S) and B. In suspensions of isolated glomeruli and isolated proximal tubules the activities of cathepsin S were 0.76 and 3.21 mumol/min x g protein, respectively. The corresponding activities of cathepsins B + L(+S) amounted to 80.0 and 211.7 mumol/min x g protein consisting of 71% cathepsin B activity. In nephron segments microdissected from lyophilized renal sections, highest cathepsin S activity was found in the proximal convoluted tubules (4.21 mumol/min x g dry weight) followed by 0.83 mumol/min x g dry weight in proximal straight tubules of the superficial cortex. In the remaining segments cathepsin S activity was hardly detectable. Unlike cathepsin S activity, the activity of cathepsin B was distributed in parallel to that of cathepsins B + L(+S). The presence of relatively high cathepsin S activity in proximal convoluted tubules in co-localization with the activities of cathepsins B + L(+S) and B suggests a primary role of these enzymes in heterophagocytosis of proteins from the ultrafiltrate.

[Photo-brine therapy in patients with psoriasis and neurodermatitis atopica]. / Photosoletherapie bei Patienten mit Psoriasis und Neurodermitis atopica.

The effectiveness of salt water baths and subsequent selective ultraviolet phototherapy (SUP) was investigated in a prospective study on 40 patients with psoriasis vulgaris and atopic dermatitis. There were two groups with 20 patients each. The first group was treated with salt water (15%) that contained synthetic Dead Seas salt called "Psorisal"; the patients in the second group had a bath in a 3% NaCl solution. After 4 weeks of daily treatment, we found that 80% of the patients in the group treated with "Psorisal" had significantly better results than the second group. The subjective feeling of being ill had decreased significantly in both groups by the end of the study. The only side effect we found was the occurrence of sunburn in few cases, but this occurred significantly less in the "Psorisal" group. Both groups generally accepted the balneophototherapy, so it can easily be employed on an outpatient basis.