[Clinical aspects and therapy of Bureau-Barrière syndrome. Observations of 17 cases with review of the literature]. / Klinik und Therapie des Bureau-Barrière-Syndroms. Beobachtungen an 17 Fällen mit Literaturübersicht.

Bureau-Barrière syndrome can affect various organ systems. It is defined by the triad: painless ulcers at mechanically irritated and hyperkeratotic plantar areas of the feet, sensitive polyneuropathy of the lower legs and osteolysis in the forefoot area. The condition mainly affects middle-aged men suffering from alcoholism, liver disease, obesity or metabolic disorders such as diabetes mellitus. Successful treatment of the Bureau-Barrière syndrome requires an interdisciplinary approach.

Red lunulae in severe alopecia areata.

The development of red nail lunulae has been extremely rarely described in alopecia areata. We observed 2 patients, a 30-year-old woman and a 61-year-old man, both suffering from severe alopecia areata, and having red lunulae. The colour changes, which developed a few weeks after the acute onset of hair loss, disappeared slowly, leaving horizontal fissures (Beau's lines).

[Pseudo-Kaposi sarcoma in Prader-Labhart-Willi syndrome]. / Pseudo-Kaposi-Sarkom bei Prader-Labhart-Willi-Syndrom.

A 25-year-old male patient presenting with Prader-Labhart-Willi syndrome is reported. He had sought medical help because of lichenoid skin eruptions on his lower legs. Histologically the lesions turned out to be pseudo-Kaposi's sarcoma. The possible aetiopathologic relationship between the two primarily unassociated conditions is discussed.

[Benign symmetric Launois-Bensaude type III lipomatosis and Bureau-Barrière syndrome]. / Benigne symmetrische Lipomatose Launois-Bensaude Typ III und Bureau-Barrière-Syndrom.

We report on a patient with an unusual distribution of benign symmetric Launois-Bensaude lipomatosis. In addition, the patient presented with Bureau-Barrière syndrome. With regard to our own observations and those reported in the literature, we propose a new classification of the disease referred to the distribution of the fatty tissue.

[Cutaneous amyloidoses]. / Kutane Amyloidosen.

Significant advances in basic research in biochemistry and immunology has tremendously increased our understanding of amyloidoses. The chemical structure of several amyloid proteins has been partly characterized. Specific antibodies may provide new methods for immunohistological diagnosis of amyloidoses. Cutaneous manifestations can affect the skin only or develop during the course of systemic amyloidoses. Although these conditions are so rare, dermatologists should be aware of the changing clinical manifestations of cutaneous amyloidoses if they wish to avoid diagnostic failures.

[Cyclosporin A treatment of Behçet's disease]. / Cyclosporin-A-Therapie des Morbus Behçet.

We report on an Italian patient with severe mucocutaneous and arthritic manifestations of Behçet's disease. Monotherapy with cyclosporin A led to a complete remission. The therapeutic efficiency could be verified by relapse after discontinuation of treatment and a second remission after its reintroduction.

[Animal experiment studies of free transplantation of striated muscles]. / Tierexperimentelle Untersuchungen zur freien Transplantation quergestreifter Muskulatur.

Heterotopic autologous muscle transplants of the left soleus muscle were performed in 39 Wistar rats. In analogy to the method described by Hakelius, the muscle was implanted into the bed of the contralateral superficial flexor muscle of the fingers with a varying prestretch of 0, 25 and 50% of its original length at rest. The animals were sacrificed postoperatively after 5, 10, 20, 40 and 60 days and their musculature examined. It was found that revascularisation and reneurotisation depended on the degree of prestretching applied. If prestretching amounted to 25 and 50%, complete degeneration of the connective tissue of the transplanted muscle occurred. However, even if prestretching was only low, i.e. between 0 and 5%, only one-third of the muscle mass regenerated and was interspersed with connective tissue of low cellular and vascular content with cicatrisation. These findings were also seen--to a lesser degree--if the soleus muscle was only denervated. We can conclude from this that restretching plays a decisive role even in the transplantation of striated muscle, as far as revascularisation and reneurotisation are concerned, and that even under the most favourable conditions only part of the transplanted muscle retains its full functional capacity. Furthermore, direct nerve anastomoses between the pudendal nerve and a branch of the femoral nerve supplying the transplanted semitendinous muscle, were performed in four Syrian goats. It was found that neurotisation of the muscle via microanastomosis is preferable to diffuse reneurotisation via the transplant bed. However, problems arose in respect of the blood supply of the transplanted semitendinous muscle, when using the technique after Holle and Freilinger.

Free and reverse smooth muscle plasty in rats and goats.

Free autologous smooth muscle transplants were performed in 59 Wistar rats from 70 to 90 days old. The free smooth-muscle transplants were prestretched by 0 percent, 100 percent, 120 percent, 150 percent, and 200 percent under resting conditions, and the animals were sacrificed on postoperative days five, 10, 20, 40, and 60, respectively. Five animals died postoperatively. Three of them died with ileus and stenosis after 150 percent prestretching of the transplant. Complications occurred in 11 other animals, and involved ileus in nine due to stenosis or increasing defecation difficulty resulting from post-150-percent-transplant-prestretching-obstruction phenomena. No relaxation reflex was observed manometrically in any of the transplants. Histology confirmed that smooth-muscle necrosis and connective-tissue multiplication increase with increasing prestretching of the transplant. With prestretching of 150 percent or more, the transplant underwent complete fibrosis. Subsequently anorectal smooth-muscle pedunculated flaps (tunnel grafts) were performed on 20 goats of Syrian and German pedigree. Manometric, histologic, and histochemical results were much better in smooth-muscle transplantation of this nature. Even in these animals, however, increased fibrosis and disintegration of the transplant was shown histologically in all animals with more than 150 percent graft prestretch. After 200 percent prestretch, additional myenteric plexus disintegration was observed and no relaxation could be achieved. Based on these clinical, manometric, and histologic results, smooth-muscle transposition (tunnel graft) can be recommended to improve continence in infants as a non-time-consuming modification of the well-established Rehbein-Romualdi-Kiesewetter pull-through procedure in the high imperforate anus. However, prestretching of the transplanted pedicle flap should not exceed 120 to 140 percent.

[Animal experimental studies on the free transplantation of smooth colon muscles as an artificial sphincter in the rat]. / Tierexperimentelle Untersuchungen zur freien Transplantation glatter Dickdarmmuskulatur als Sphinkterersatz bei der Ratte.

Free autologous muscle transplants were performed on 59 Wistar rats of 70 to 90 days of age according to the method described by Schmidt (1978). The free smooth muscular transplant was prestretched by 0%, 100%, 120%, 150% and 200% and the animals were sacrificed on the 5th, 10th, 20th, 40th and 60th postoperative day, respectively. Five animals died postoperatively, of these three during ileus and stenosis after more than 150% prestretching of the transplant. Complications occurred in eleven further animals, which included again ileus phenomena nine times due to stenosing or disturbance of defecation, also as a result of obstruction phenomena. Complications always occurred in animals with more than 150% transplant prestretching. No relaxation reflex was observed manometrically in any of the transplants. Budding of nerve fibres also did not occur in any of the cases, neither from the plexus myentericus of the inner intestinal wall nor from outside. Histology revealed already after five days' survival leucocytous infiltration and connective tissue multiplication which increased with increasing prestretching of the transplant and longer survival time of the animals until complete metaplasia of the connective tissue. In preparations with 100 up to 120% prestretch not more than 20% of the original muscular mass was preserved. With prestretching of 150% and more the transplant underwent complete fibrosis. On the basis of these studies we must discourage performance of free transplants of smooth-muscle colon segments in newborn and young infants.

[Animal experimental studies of the smooth muscle pedicle flap (tunnel graft) in the goat]. / Tierexperimentelle Untersuchungen zur gestielten glatten Muskel-transplantation (Muff-Plastik) bei der Ziege.

Anorectal smooth muscles pedicle flaps (funnel grafts) according to the dragging method described by Holschneider in 1980, 1981 and Hofmann-von Kap-herr and Koltai 1981 were performed on 20 Syrian goats and German pedigree goats. Postoperatively, three animals died because of pulmonary infection with worms; 17 animals could be examined. Six of these 17 animals died during the postoperative follow-up period from the sequels of the operation. In five animals the cause of death was prestretching of the transplant by 150 and 200%; three of these developed severe enterocolitis, whereas two had stenosis and ileus, resulting in death in each case. In addition, five further animals showed signs of enterocolitis; eight goats had constipation which in every case could be ascribed to prestretching of the transplant by 150% and more. These animals had to be treated by infusion and bouginage, leading to recession of the signs after four to six days. Electromanometrically an increased anorectal resting pressure profile was found postoperatively in all cases, i.e. even if the transplant was prestretched to tension values of only over 100%. Relaxations of the internal anal sphincter could be achieved in all animals; however, in goats with a transplant stretch of 150% and more they were observed only after the resting pressure profile had receded, a few months after the operation. Histologically, there was increasing fibrosis and disintegration of the transplant in all animals with more than 150% prestretch. With 200% prestretch, the plexus myentericus disintegrated also, so that no relaxation could be achieved. Basing on these clinical, manometrical and histological results, smooth muscle transposition (funnel graft) can be recommended to improve continence in infants by employing the dragging method according to Rehbein-Romualdi. However, prestretching of the transplanted pedicle flap should not exceed 120-140%.