Lipomatosen.

Als Lipomatosen bezeichnet man gutartige, meist ohne eindeutige Auslöser auftretende Vermehrungen des Fettgewebes, oft mit typischem Verteilungsmuster. Im Gegensatz zu umschriebenen Lipomen entwickeln sie sich meist diffus-symmetrisch und sind nicht von einer fibrösen Pseudokapsel umgeben. Am häufigsten ist die benigne symmetrische Lipomatose (BSL; Launois-Bensaude-Syndrom), von der aufgrund des Verteilungsmusters des hyperplastischen Fettgewebes vier Typen unterschieden werden. Ätiologie und Pathogenese der Erkrankung sind noch weitgehend unbekannt, wobei manche Formen eine hereditäre Basis zu haben oder mit vermehrtem Alkoholkonsum assoziiert zu sein scheinen. In einigen Fällen wurden mitochondriale Fehlfunktionen nachgewiesen. Lipomatosen können isoliert, aber auch mit Begleitsymptomen oder -erkrankungen (Komorbidität) vergesellschaftet auftreten. Wirksame medikamentöse Therapien sind nicht bekannt; chirurgische Verfahren können hingegen zur Besserung der Symptome führen. Die von der BSL abzugrenzende Lipomatosis dolorosa tritt bevorzugt bei Frauen mittleren Alters auf und ist durch Vermehrung des Fettgewebes sowie starke Schmerzen in den betroffenen Bereichen gekennzeichnet. Verschiedene Differenzialdiagnosen und Begleiterkrankungen sind abzuklären, insbesondere Depressionen. Evidenzbasierte Therapieempfehlungen existieren nicht. Repetitive Lidocain-Infusionen können temporäre Besserungen bewirken; nach chirurgischen Interventionen treten häufig Rezidive auf, allerdings werden auch langfristige Erfolge erzielt.

Lipomatoses.

Lipomatoses are benign proliferations of adipose tissue, often with typical distribution patterns, which usually occur without clear causes. In contrast to circumscribed lipomas, they develop diffusely and symmetrically and are not surrounded by a fibrous pseudocapsule. The most common form is benign symmetric lipomatosis (BSL; Launois-Bensaude syndrome), of which four types are distinguished based on the distribution pattern of hyperplastic adipose tissue. The etiology and pathogenesis of the disease are still largely unknown, although some forms appear to have a hereditary basis or to be associated with increased alcohol consumption. In some cases, mitochondrial dysfunctions have been detected. Lipomatoses may be solitary, but may also be associated with other symptoms or illnesses (comorbidity). Effective drug therapies are not known, but surgical procedures can alleviate the symptoms. Lipomatosis dolorosa, which can be distinguished from BSL, occurs predominantly in middle-aged women and is characterized by proliferation of adipose tissue and severe pain in the affected areas. Various differential diagnoses and concomitant illnesses need to be considered, in particular depression. There are no evidence-based recommendations for therapy. Repetitive lidocaine infusions can achieve temporary improvement. Recurrence after surgical intervention is not uncommon, but long-term success is also possible.

Patients with pyoderma gangrenosum - analyses of the German DRG data from 2012.

Pyoderma gangrenosum (PG) is a rarely diagnosed non-infectious neutrophil ulcerative dermatosis with only limited knowledge on the underlying auto-inflammatory process. To unravel common cofactors and comorbidities in patients with PG we analysed Diagnosis Related Groups (DRG) cases of all inpatients diagnosed with PG in German hospitals in 2012. We received data of 1227 inpatient cases having PG as primary diagnosis and 985 inpatient cases with PG as secondary diagnosis. The ratio of women to men was 2:1, and the most often registered age was 75-79 years. Common comorbidities were arterial hypertension (50·3%), non-insulin-dependent diabetes mellitus (25·1%) and dysfunction of lipid metabolism (10·8%). In sum, 94·8% of the patients suffered from aspects of metabolic syndrome. Other comorbidities were Crohn's disease (4·5%), ulcerative colitis (4·2%), chronic polyarthritis (5·2%), monoclonal gammopathy or myelodysplastic syndrome (2·5%), leukaemia (1·1%) and lymphoma (0·4%). DRG data do not reflect individual patients, but rather patient cases. We described the worldwide largest PG population and confirmed a wide range of potentially relevant and partly not yet described cofactors and comorbidities such as metabolic syndrome.

Therapy of pyoderma gangrenosum in Germany: results of a survey among wound experts.

BACKGROUND: There are currently no therapeutic guidelines with respect to pyoderma gangrenosum (PG). MATERIAL AND METHODS: In order to systematically record currently practiced therapeutic options for PG, a questionnaire was developed and sent to experts at various German dermatology departments. RESULTS: Overall, 50 fully completed questionnaires were returned to us. On average, the diagnosis of PG is made 15 times per year. Of these patients, 11.1 % receive only topical therapy; 22.7 %, exclusively systemic therapy; and 66.2 %, both topical and systemic immunomodulatory therapy. Topically, the experts most commonly use class III (78 %) and class IV corticosteroids (88 %). All experts (100 %) reported the frequent use of systemic corticosteroids in the therapy of PG patients. Cyclosporine is used in 74 %, TNF-α inhibitors in 24 %, and azathioprine or mycophenolate mofetil each in 20 % of patients. Systemic agents such as dapsone (16 %), intravenous immunoglobulins (14 %), methotrexate (6 %), cyclophosphamide (4 %), tacrolimus (4 %), and rituximab (2 %), however, are given less frequently. CONCLUSIONS: Worldwide, no uniformly accepted standard is available for the treatment of patients with PG. In our survey among experts, treatment regimens currently used in Germany, and based on expert clinical experience, have now been recorded, analyzed, and benchmarked for the first time.

Evaluation of the Essen Rotary as a new technique for bacterial swabs: results of a prospective controlled clinical investigation in 50 patients with chronic leg ulcers.

Most chronic wounds are colonised with different microorganisms, especially problematic bacteria like methicillin-resistant Staphylococcus aureus (MRSA), which represent an increasing therapeutic challenge in the modern wound therapy regimen. Therefore, it is essential to specify the bacteria in wounds for an individual-specific treatment. In most patients, an exemplary bacterial swab is taken from the centre of the wound surface. This so-called Levine technique is propagated currently as the gold standard. The aim of our clinical investigation was to compare the results of different swab techniques to the new established Essen Rotary. In this monocentric prospective investigation, 50 patients with chronic leg ulcers were examined consecutively. The results of our clinical study show that bacteria are heterogeneously spread on wound surfaces. The analysis of the semiquantitative measured results showed that the Essen Rotary could detect significant more bacteria with a total amount of 111 bacteria (P = 0·049) compared to usual swab techniques. Considerably, only the Essen Rotary identified five compared to three MRSA-patients detected by other techniques. The Essen Rotary is an efficient, economic and uncomplicated modification of bacteriological swab techniques which detects significant more bacteria compared to other conventional swab techniques. Therefore, the Essen Rotary may become the new gold standard in routinely taken bacteriological swabs especially for MRSA screenings in patients with chronic leg ulcers.

Investigation of new co-factors in 49 patients with pyoderma gangrenosum.

BACKGROUND: Pyoderma gangrenosum is a rare, destructive, ulcerative neutrophilic dermatosis of unknown origin that has been investigated insufficiently in clinical studies. According to current textbooks, it is often associated with chronic inflammatory bowel diseases or other autoimmune disorders. PATIENTS AND METHODS: We retrospectively analyzed data from 49 patients with pyoderma gangrenosum. RESULTS: Our results showed that although only 6.1 % of patients had chronic inflammatory bowel disease, 22.4 % patients had a malignancy, 18.4 % had chronic renal insufficiency, and 42.8 % had anemia. A potentially relevant aspect that has received little attention is an association with endocrine diseases in 38.8 % of patients. 28.6 % of patients had diabetes. Given that 32.6 % of patients were obese, a potential association with metabolic syndrome may be considered as a possible new risk factor for pyoderma gangrenosum. CONCLUSIONS: The clinical data from the present study are insufficient for drawing any firm conclusions. We did, however, observe an association between pyoderma gangrenosum and certain as yet unreported co-factors, in particular metabolic syndrome. This should be considered in further studies.

Updated results of 100 patients on clinical features and therapeutic options in necrobiosis lipoidica in a retrospective multicentre study.

BACKGROUND: Necrobiosis lipoidica (NL) is a rare granulomatous disorder of unknown aetiology which can manifest in ulcerative skin lesions and is widely recognised in association with diabetes. We evaluated in total 100 patients to improve knowledge about the epidemiology and clinical course of NL. Additionally we focused on treatment options according to evidence level and expert opinion. OBJECTIVE: To examine the clinical features of patients with necrobiosis lipoidica and to give recommendations for topical and systemic treatment. MATERIALS & METHODS: This multicentre retrospective study covers data from 100 patients with the diagnosis of NL of the lower leg, treated during the period between 01/2006 and 06/2014. Data from 5 German dermatological centres with 23 men and 77 women with a median age of 52 years were evaluated. We also asked 15 dermatological experts for their opinions about ulceration, associated diseases and their preferences for treatment modalities of NL. RESULTS: Patients' ages ranged from 15 to 95 years (median age 52 years, mean age 49.8 years). NL was significantly more common in women (77%). Ulceration of the NL was observed in 33% of cases, in particular in male patients and in patients with concomitant diabetes mellitus. Thyroidal disorders were found in 15% of all cases. CONCLUSION: Our data confirm that female sex, middle age and endocrine disorders like diabetes mellitus or thyroid dysfunction are characteristics of patients suffering from NL. Existing therapeutic options were very variable and - according to the opinion of our experts - only a few highly effective.

Lower prevalence of lymphatic metastasis and poorer survival of the sentinel node-negative patients limit the prognostic value of sentinel node biopsy for head or neck melanomas.

Head or neck location of primary cutaneous melanomas has been described as an adverse prognostic factor, but this has to be reassessed after the introduction of sentinel lymph node (SLN) excision (SLNE). Descriptive statistics, Kaplan-Meier estimates and Cox proportional hazard models were used to study retrospectively a population of 2302 consecutive melanoma patients from three German melanoma centres undergoing SLNE. Approximately 10% of the patients (N=237) had a primary melanoma located at the head or neck (HNM). In both the SLN-positive and SLN-negative subpopulation, patients with HNM were significantly older, more frequently men and had thicker primaries compared with patients with tumours in other locations. The proportion of positive SLNs was lower in HNM compared with other locations of the primary (20 vs. 26%, P=0.048). The false-negative rate was higher in HNM (17.5 vs. 8.4%, P=0.05). In patients with HNM, the SLN status was a significant factor for recurrence-free survival but not for overall survival. SLN-negative HNM patients had a significantly worse overall survival than the SLN negatives with primaries at other sites, whereas the prognosis of the SLN-positive patients was similar in both groups. The prevalence of lymph node metastases after SLNE is lower in patients with HNM compared with other melanoma locations. As a result, the prognostic information provided by the SLN for HNM seems less important. Decision making for SLNE in HNM should be carefully balanced considering the potential morbidity of the procedure.

Associated factors and comorbidities in patients with pyoderma gangrenosum in Germany: a retrospective multicentric analysis in 259 patients.

BACKGROUND: Pyoderma gangrenosum (PG) is a rarely diagnosed ulcerative neutrophilic dermatosis with unknown origin that has been poorly characterized in clinical studies so far. Consequently there have been significant discussions about its associated factors and comorbidities. The aim of our multicenter study was to analyze current data from patients in dermatologic wound care centers in Germany in order to describe associated factors and comorbidities in patients with PG. METHODS: Retrospective clinical investigation of patients with PG from dermatologic wound care centers in Germany. RESULTS: We received data from 259 patients with PG from 20 different dermatologic wound care centers in Germany. Of these 142 (54.8%) patients were female, 117 (45.2%) were male; with an age range of 21 to 95 years, and a mean of 58 years. In our patient population we found 45.6% with anemia, 44.8% with endocrine diseases, 12.4% with internal malignancies, 9.3% with chronic inflammatory bowel diseases and 4.3% with elevated creatinine levels. Moreover 25.5% of all patients had a diabetes mellitus with some aspects of potential association with the metabolic syndrome. CONCLUSIONS: Our study describes one of the world's largest populations with PG. Beside the well-known association with chronic bowel diseases and neoplasms, a potentially relevant new aspect is an association with endocrine diseases, in particular the metabolic syndrome, thyroid dysfunctions and renal disorders. Our findings represent clinically relevant new aspects. This may help to describe the patients' characteristics and help to understand the underlying pathophysiology in these often misdiagnosed patients.