[Muir-Torre syndrome]. / Muir-Torre-Syndrom.

The Muir-Torre syndrome (MTS) is a rare, autosomal-dominant inherited disease characterized by sebaceous gland tumors and at least one internal malignancy. In many cases a genetic defect known as microsatellite instability can be identified. Similar pathogenetic mechanisms are found in patients with the hereditary non-polyposis colon cancer syndrome, so that at least some patients with MTS are considered as having a phenotypic variant of that syndrome. A 66-year-old woman with MTS, developed multiple malignant cutaneous and visceral tumors over 32 years; in addition, she had multiple sebaceous gland adenomas. Microsatellite instability could be proved with 2 out of 5 studied markers. The family history was positive as numerous relatives of the patient's mother were reported to have developed internal malignancies.

[Disseminated small-node cutaneous sarcoidosis]. / Disseminierte kleinknotige kutane Sarkoidose.

HISTORY AND ADMISSION FINDINGS: For 6 months before admission an 83-year-old woman had experienced itching from papules on her back. Despite local and systemic corticosteroid treatment the lesions had spread further over the back and also began to involve the proximal limbs. On examination the patient was noted to be fit for her age. At the described sites there were scattered red-brown maculopapular lesions, pin-head sized, some circumscribed, others confluent. Diascopy demonstrated them to be greyish yellow. INVESTIGATIONS: Erythrocyte sedimentation rate was 15/42 mm, serum concentrations of angiotensin converting enzyme (ACE) and calcium were within normal limits. The Tine test was negative. Skin biopsy revealed a granulomatous epithelioid cell inflammation in the corium without central necrosis. She had diabetic retinopathy, a complication of type II diabetes mellitus for many years, and hepatic steatosis. TREATMENT AND COURSE: The results of the clinical and histological findings and exclusion of systemic involvement established the diagnosis of disseminated small node cutaneous sarcoidosis. Under clofazimine (Lampren), at an initial dosage of 3 x 100 mg daily by mouth, gradually reduced over 4 months, the cutaneous lesions healed completely and lastingly. CONCLUSION: This form of sarcoidosis has a good prognosis. Clofazimine, being efficacious and well tolerated, provides a good therapeutic alternative.

[Pseudopelade Brocq--possible sequela of stage III borrelia infection?]. / Pseudopelade Brocq--mögliche Folge einer Borreliose Stadium III?

The differential diagnosis of cicatricial alopecia includes a wide variety of diseases. A 60-years-old woman was admitted to the hospital because of an unsuccessfully treated, progressive pseudopelede of Brocq. Further examination revealed an borreliosis (Stage III). After 3 weeks of treatment with intravenous Cefotaxime (Claforan), follow-up examination one year later show an complete arrest of the alopecia. This first description of cicatricial alopecia to be causally related to borreliosis Stage III extends the differential diagnosis of pseudopelede of Brocq and offers a curative therapy.

Epidemiological features of Adamantiades-Behçet's disease in Germany and in Europe.

The German Registry of Adamantiades-Behçet's disease was founded in 1990 in Berlin and it provides current data on the epidemiology, the clinical manifestations and the course of the disease in Germany on a continuous basis. A total of 218 patients, including 89 German and 100 Turkish patients, had been reported to the German Registry until October 1997. One hundred and ninety-six patients fulfilled the criteria of the Behçet's disease classification tree. The prevalence of the disease evaluated in Berlin-West was 1.68/100,000 in 1989 and had risen to 2.26/100,000 by 1994. The median age of onset was 25 years (range 5 to 66 years; German-Turks, ns). Juvenile disease was recorded in 6.9% of patients. The complete clinical picture according to the criteria of the International Study Group of Behçet's Disease developed in 15.5 months. The interval between onset of the disease and diagnosis was 35 months, which was significantly longer than the duration of the development of the complete clinical picture (p < 0.0001). The disease was diagnosed later in German (48.5 months) than in Turkish patients (25.5 months, p = 0.003). While German patients presented an equal male-to-female ratio, a male predominance was shown in Turkish patients (M:F 2.1:1, p = 0.022). Familial occurrence was detected in 2.0% of German and 15.9% of Turkish patients (p = 0.013). The frequencies of major clinical manifestations were: oral ulcers 99%, skin lesions 76%, genital ulcers 75%, ocular manifestations 59%, arthritis 59%, and positive pathergy test 52%. Clinical differences between German and Turkish patients were only found in the frequency of ocular lesions (48% vs. 66%, p = 0.025). Oral ulcers were with 72% the most common onset symptom of the disease followed by erythema nodosum (9%), uveitis (7%), arthritis (7%), genital ulcers (3%), superficial thrombophlebitis (2%) and papules/sterile pustules (2%). Uveitis and erythema nodosum as onset symptoms shortened the median interval to diagnosis to 1.5 and 15 months, respectively, while arthritis delayed diagnosis (43.5 months; p = 0.029). A severe course developed in 25% of the patients; irreversible retinal vasculitis to blindness in 15%, sterile meningoencephalitis in 8%, severe arthritis in 5%, hemoptysis in 2%, lethal outcome in 2% and bowel perforation in 1%. The relative risk of HLA-B5 positive German natives developing the disease. HLA-B5 was confirmed as a marker of severe prognosis. Cardiolipin autoantibodies were associated with cutaneous vasculitis and superficial thrombophlebitis was correlated with systemic vessel involvement.

[The HLA pattern in Adamantiades-Behçet's disease in Germany. Association of occurrence, clinical symptoms and follow-up in 39 patients]. / HLA-Muster bei Morbus Adamantiades-Behçet in Deutschland. Assoziation zum Auftreten, der klinischen Symptomatik und dem Krankheitsverlauf bei 39 Patienten.

The frequencies of HLA-class I antigens were studied in 39 patients with Adamantiades-Behçet's disease and were compared with those in 1415 [corrected] healthy controls; both patients and controls were of German origin. Moreover, the correlations of HLA antigens with onset, various clinical features and the course of the disease were investigated and the familial clustering of the disease was examined. We found an increased frequency of the HLA-B5 antigen in these series (P < 0.05, relative risk 2.6, confidence interval 1.2-5.5). The presence of HLA-B5 was more frequently detected in male (41%, relative risk 4.9, confidence interval 1.8-13.1) than in female patients (9%; P < 0.05). A significantly higher frequency of HLA-B5 was found in male patients with severe vascular involvement, including blindness and thrombosis, than in HLA-B5-positive male patients without these features. Incidences of genital ulcers, ocular involvement, cutaneous features, positive pathergy test and of secondary symptoms as well as the age of onset and the duration of development of the complete clinical picture were all found not to be significantly correlated with any of the HLA-A, -B, -C alleles. Familial clustering was also not found in German patients. Because of the low relative risk in the presence of HLA-B5 and the lacking familial clustering, it seems likely that there is a weak immunogenetically determined predisposition for Adamantiades-Behçet's disease in Germany. HLA-B5-positive male persons of German origin show a higher relative risk to develop or acquire Adamantiades-Behçet's disease than female ones.(ABSTRACT TRUNCATED AT 250 WORDS)

[Cutaneous manifestation of Crohn disease]. / Kutane Manifestation des Morbus Crohn.

In a 20-year-old women having suffered from Crohn's disease for many years, we found granulomatous fistular ulcerations on her right distal lower leg. After histological verification of the clinical diagnosis, she was orally treated with metronidazole for 6 weeks with excellent results: The cutaneous lesions healed completely.

[Churg-Strauss syndrome]. / Das Churg-Strauss Syndrom.

Churg-Strauss syndrome is a systemic, granulomatous, necrotizing, and eosinophilic angiitis, associated with febrile bronchitis. The full-blown picture of the disease does not develop, however, but in the course of several years. We discuss the problems of diagnosis and treatment of this syndrome in the light of a case report concerning a 43-year-old woman suffering from Churg-Strauss syndrome.

[Malignant blue nevus]. / Maligner blauer Naevus.

In a 6-year-old boy, we found a malignant blue nevus with metastases of the lymph nodes. After surgical therapy under melanoma conditions, he was treated with interferon. Up to now (2 years after therapy), the follow-up examinations have been negative.

[Granulocyte function in the aged]. / Granulozytenfunktion im Alter.

22 patients (10 female, 12 male) aged between 60 and 76 were tested in vitro with regard to granulocyte function (i.e., leucotaxis, chemotaxis, capability of phagocytosis, intracellular killing of bacteria, and oxidase reaction). Most of the patients showed reduced chemotactic activity of the granulocytes, although the motility of these cells was normal. With regular phagocytosis of candida albicans cells--either active or heat-inactivated the intracellular killing rate of bacteria was clearly decreased in all cases. The intracellular oxidase activity of granulocytes always showed normal function.

[Oral acyclovir therapy in recurrent herpes simplex]. / Oral Aciclovir-Therapie des Herpes simplex rezidivans.

25 patients suffering from recurrent herpes simplex infection were orally treated with acyclovir 5 X 200 mg daily for 5 days. Control examinations were carried out on the 1st, 3rd, 5th, and sometimes on the 7th day of treatment. All patients treated showed a significantly shortened course of the disease and quick decrease of the symptoms. If acyclovir was applied during the prodromal phase, we additionally found inhibited vesicular eruption. There were not observed any dangerous side effects.

[Evaluation of micro- and macrophage function in splenectomized patients--possible dermatologic consequences]. / Mikro- und Makrophagenfunktionsprüfungen bei splenektomierten Patienten--mögliche dermatologische Konsequenzen.

Various micro- and macrophage functions were studied in vitro in 15 patients having previously undergone posttraumatic splenectomy as well as in 100 healthy persons. We found reduced capacity of intracellular inactivation of microorganisms by microphages (7/15) and slight impairment of chemotactic activity in both systems (8/15; 9/13). Possible dermatological consequences are discussed.

[5-Fluorouracil treatment of condylomata acuminata]. / 5-Fluoro-Uracil-Behandlung der Condylomata acuminata.

25 patients (2 females, 23 males, 20 to 40-year-old) suffering from condylomata acuminata (19 genital, 2 anal, and 4 genitoanal lesions) were locally treated with salicylic acid containing 5-fluorouracil (Verrumal) once a day. This therapy resulted in complete healing within 12 days on an average. 5 patients showed a relaps after 2 weeks to 5 months and were treated again. The treatment failed in a 40-year-old patient suffering from condylomata acuminata genitalis.

[The "unspecific" immune state in skin diseases with special reference to microphage functions]. / Der "unspezifische" Immunstatus bei Hautkrankheiten unter besonderer Berücksichtigung der Mikrophagenfunktionen.

Polymorphonuclear leucocytes (PMNL) or microphages resp. are essentially involved, on account of their ubiquity within the entire organism as well as their phagocytic and intracellular killing properties, in the complex organization of the antimicrobial and antitumoral protective capacity of man. Positive chemotaxis triggers the migration of PMNL to the specific danger area and thus initiates an immune reaction. An either inborn or acquired defect or functional weakness of PMNL may entail a state of persisting viral, fungal and/or bacterial infections. Impairment of various PMNL functions may result from different metabolic disorders, internal malignancies, and/or immunosuppressive therapy. Disturbances of various PMNL functions can be especially analysed in vitro. After delineation of different techniques available to check the immunological defense system of the human organism, we report on our results of PMNL function analysis in patients suffering from chronically recurrent pyoderma (n = 22), oral precancer and cancer (n = 13), chronic mucocutaneous candidosis (n = 10), recurrent aphthous ulcers (n = 33), as well as Behcet's disease (n = 20).