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The article looks at the use of wounds-at-risk (WAR) score to assess the individual patient's risk of wound infection. Information on the WAR score is presented, which is based on anamnestic and clinical criteria wherein patients are assigned point values. An overview of a multicentre study evaluating data from 10 specialised dermatological wound centres in Germany is provided. A table of the therapeutic steps for antimicrobial wound treatment is also included.

Background Cutaneous angiosarcoma (AS) is a rare aggressive malignant vascular tumour of the skin. Methods We analysed the cases of cutaneous AS in two large dermatological departments of Saxony during the last 10 years. Results A total of eight AS cases were identified, mostly with head and neck involvement. Because of age and late diagnosis, only a minor part was completely resectable. As a palliative therapy pegylated liposomal doxorubicin was used. The drug was well tolerated. Our cases and those published in the literature suggest a high rate of responses and a possible sensitisation to subsequent radiation therapy. Conclusions Although complete surgical resection followed by wide-field radiation therapy has been established as a therapeutic standard for AS, delay in diagnosis and older age with comorbidities warrant for a safe and effective adjuvant treatment. Pegylated liposomal doxorubicin combined with radiotherapy seems to be a useful and safe alternative. [ABSTRACT FROM AUTHOR]

Background Late recurrent melanoma (MM) is rare. Objective In the present study, we analysed the frequency of late recurrent MM in south-eastern Germany. Patients and methods In our centre, 2314 MM patients were documented (1972–2001). A total of 1881 patients in stage I or II (AJCC) with a follow-up of ≥10 years were selected and screened for late recurrence (≥10 years after diagnosis). Results Twenty patients were identified (1.1%), 13 women and 7 men, median age 44 years (age range 30–74 years). Nineteen suffered from cutaneous MM and one had a uveal MM (excluded from further analysis). The primary cutaneous MM occurred on the trunk (6), on the upper limb/shoulder (4), or on the lower limb (9). MM type was superficial spreading (13), nodular (2), acrolentiginous (1), lentigo maligna-type (1) or unclassified (2). Tumour thickness varied from 0.33 mm to 9.5 mm (median 2.0 mm). Ulceration was seen in four, and spontaneous regression in two MM patients. Invasiveness into blood or lymphatic vessels occurred in seven MM patients. The largest period from primary diagnosis to recurrence was 25.1 years with a median of 13.9 years. Metastatic spread was loco-regional (12 patients) or distant (7). Four patients were survivors and three of these had in-transit metastases only. Overall survival was 14.7 ± 6.6 years. Statistical analysis could not identify factors significantly associated with late recurrence. Conclusions Late recurrence is a clinical sign of melanoma dormancy. We conclude that late recurrences argue for a lifelong follow-up of melanoma patients. [ABSTRACT FROM AUTHOR]

Occupational skin diseases (OSD) are among the most frequent occupational diseases (OD). Compared to other occupations, bakers, confectioners and employees in the catering trades are at a high risk of developing OSD. In January 1999, the interdisciplinary Skin Disease Prevention Program in the Baking, Hotel and Catering Industries (SDPP) conducted by the Department of Dermatology and Allergology at the Friedrich Schiller University, Jena, in cooperation with the Research Center for Applied System Safety and Industrial Medicine, Erfurt, and the technical experts at the Social Insurance for the Food Industry and Related Professions (Berufsgenossenschaft für Nahrungsmittel und Gaststätten – (BGN)), Erfurt, was initiated. Following detailed analysis of the occupational exposure of the employees and their personal occupational disease history, the patients’ diagnosis and therapy was re-evaluated and supplemented if necessary. Individual skin care and protection regimes were demonstrated and practically trained. Skin care and protection products were supplied. Skin-care and protection seminars were offered to volunteering participants. From January to December 1999, 29 affected employees were examined in the OSD clinic. 22 employees (76%) suffered from irritant contact dermatitis. The follow-up data of 11 employees are available. In 8 employees (73%), the skin disease improved or disappeared. Moreover, in 1 employee (9%), the skin condition was stabilized even though he continued working. In only 2 employees (18%) did the skin condition worsen. These preliminary results showed that most of the OSD were due to lack of or unsuitable skin care and protection. The program will be extended to cover a larger number of food industry employees with OSD. [ABSTRACT FROM AUTHOR]

Summary: Pyoderma gangrenosum (PG) is a rare, treatable skin disorder that causes ulcers ‐ usually a single one but sometimes more. It is not contagious. Often it is diagnosed by ruling out other diseases with similar symptoms, and it can be misdiagnosed. The aim of this study, from Germany, was to establish a scoring system that includes symptoms and features that are specific to PG, that could easily be used in clinics to diagnose the disorder. The scoring system, called PARACELSUS, was tested on 60 participants with previously confirmed PG, and a control group of 50 patients with leg ulcers that are not caused by PG. The criteria included in the scoring system relate to how the ulcer looks, how it progresses and which drugs help clear it. The authors conclude that, based on their tests, the PARACELSUS score represents an easily implementable, effective and accurate diagnostic tool for PG. Linked Article: Jockenhöfer et al. Br J Dermatol 2019; 180:615–620 [ABSTRACT FROM AUTHOR]

Purpose: Uveal melanoma (UM) is an orphan cancer of high unmet medical need. Current patterns of care and surveillance remain unclear as they are situated in an interdisciplinary setting., Methods: A questionnaire addressing the patterns of care and surveillance in the management of patients with uveal melanoma was distributed to 70 skin cancer centers in Austria, Germany and Switzerland. Frequency distributions of responses for each item of the questionnaire were calculated., Results: 44 of 70 (62.9%) skin cancer centers completed the questionnaire. Thirty-nine hospitals were located in Germany (88.6%), three in Switzerland (6.8%) and two in Austria (4.5%). The majority (68.2%) represented university hospitals. Most patients with metastatic disease were treated in certified skin cancer centers (70.7%, 29/41). Besides, the majority of patients with UM were referred to the respective skin cancer center by ophthalmologists (87.2%, 34/39). Treatment and organization of follow-up of patients varied across the different centers. 35.1% (14/37) of the centers stated to not perform any screening measures., Conclusion: Treatment patterns of patients with uveal melanoma in Germany, Austria and Switzerland remain extremely heterogeneous. A guideline for the treatment and surveillance is urgently needed.

Facial rejuvenation is gaining immense popularity among patients and clinicians. Botulinum toxins derived from bacteria are well-tolerated options as minimally invasive interventions for facial rejuvenation or other aesthetic procedures. These products have revolutionized aesthetic treatments. Several types of botulinum toxins (BoNT) are available. Currently type A and B are clinically used and only BoNT‑A products are approved for use for cosmetic indications in the Germany and the United States. Each product is unique in terms of its composition. Understanding the various BoNT‑A products is essential in choosing the optimal treatment for our patients. In this article we discuss different BoNT‑A products used for aesthetic intervention.

Background: Melanoma incidence rates rise as people age, but the impact of aging on distant metastasis is unclear., Objective: To investigate how timing, pattern, and extent of distant metastasis is influenced by age., Methods: Analysis of a single-center cohort of 1457 patients of the German Central Malignant Melanoma Registry with prospectively documented follow-up. Findings were compared with those for 1682 patients from 5 different institutions. All patients presented initially with stage IA to IIC and developed distant metastasis in their further disease course., Results: The number of metastatic sites decreased with increasing age at melanoma diagnosis (P < .001). The rate of stage M1d disease decreased from 50.2% in patients aged 50 years or younger to 30.1% in patients older than 70 years, and the rate of stage M1b disease increased from 5.8% to 21.5%. The rate of lung metastases remained stable in all investigated age groups (P = .54). Distant metastases occurred earlier and were more synchronized in patients older than 70 years than in patients aged 50 years or younger. An age-dependent decrease in metastatic sites and stable rate of lung metastasis were found and confirmed by data on the multi-institutional cohort., Limitations: The study was not population based., Conclusion: Pattern, timing, and extent of distant metastasis change as people age. These findings may be considered when treating patients with melanoma of different ages., (Copyright © 2019 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Background: Advanced cutaneous squamous cell carcinoma (aSCC) is an area of unmet medical need and no treatment standards are established. Recently, an anti-PD-1 inhibitor received FDA breakthrough therapy designation. The aim of the study was to describe the clinical course, therapeutic management and prognosis of aSCC under real-life conditions., Patients and Methods: In a retrospective study performed in 24 German and Austrian hospitals and doctor's offices, patient and tumour characteristics of patients diagnosed with aSCC between January 1, 2010 and December 31, 2011 and their disease course was documented. Advanced SCC comprised either locally advanced SCCs (laSCC) or metastatic SCCs (mSCC) with any kind of metastatic spread., Results: Data of 190 patients with aSCC were analysed. Median age at time of diagnosis of aSCC was 78 years. LaSCC was diagnosed in 76 patients (40%), 114 patients (60%) had mSCC. Once diagnosed with laSCC, most patients (59%) did not receive any therapy, whereas in 92% of mSCC patients at least one type of therapy was performed. Only 32 patients (29 mSCC, 3 laSCC) received systemic antitumour therapies, mostly EGFR inhibitor-based regimens. Mean duration of response was short (17-months laSCC patients, 3-months mSCC patients). Only 2 patients achieved a complete response, 27% had a partial response, 43% disease stabilisation. At diagnosis of aSCC, ECOG status was 0-1 in most patients. Non-malignant comorbidities influenced the decision on SCC-specific therapy in 39 patients (21%)., Conclusions: Our data show the high medical need for efficient and tolerable antitumour therapies and demonstrate that despite older age and comorbidities, most patients can be expected to be fit for treatment. This study provides a historical context for emerging aSCC treatments., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

Background: Acral lentiginous melanoma (ALM) is one of the four major subtypes in cutaneous melanoma (CM). Although ALM has a poorer prognosis than other CM subtypes, the prognostic factors associated with ALM have only been verified in small-sized cohorts because of the low incidence of ALM worldwide., Objectives: To investigate the clinical characteristics of ALM and to evaluate their prognostic values based on a large dataset from the Central Malignant Melanoma Registry (CMMR) of the German Dermatologic Society., Methods: The Kaplan-Meier method was used to estimate the potential influence of clinical and histological parameters on ALM disease-specific survival (DSS) curves, which were compared using the log-rank test. A Cox proportional hazards model was used to identify independent prognostic factors for DSS., Results: In total, 2050 patients with ALM were identified from 58 949 patients with CM recorded by the CMMR with follow-up data. In multivariate analyses, age (P = 0·006), ulceration (P = 0·013), tumour thickness (P < 0·001) and tumour spread (P < 0·001) turned out to be significant prognostic factors for DSS in ALM whereas sex, nevus association and level of invasion were not independent factors., Conclusions: ALM has the same prognostic factors as other subtypes of melanoma. Unfavourable prognosis probably derives from the delay in diagnosis in comparison with other melanoma subtypes., (© 2017 British Association of Dermatologists.)

The history of medical and veterinary mycology in general has been reviewed in the excellent monography by G.C. Ainsworth (1905-1998) published in 1986. Here, we will focus on German-speaking mycology and their outstanding personalities. We will start with the early years when medical mycology was in its infancy. Microscopy was a most valuable tool for the identification of fungi followed by cultivation and staining methods. Human pathologies became linked to fungi. After World War I, medical mycology flourished as an integral part of dermatology at universities and in private institutes. The development was interrupted by World War II, which divided Germany. In both parts of Germany, medical mycology had to be re-established. After re-unification the two different medical societies joined together. The development of DMyK (Deutschsprachigen Mykologischen Gesellschaft - Mykologie) is illustrated. Important personalities and some of their achievements are mentioned. Mycology has attracted other fields of medicine including internal medicine, pediatrics, microbiology, and veterinary medicine.

Background: Atypical fibroxanthoma is a rare mesenchymal tumor of the head and neck region., Methods: We analyzed the files of 3 large dermatology hospitals from the years 2001 to 2013 in southeast Germany., Results: We identified 53 patients (56 tumors) with a male predominance. The mean age was 78.0 years ± 8.3 years. Mohs surgery was performed in all cases resulting in complete remission in 45 patients. Five patients had a relapse within 2 years, and 4 developed metastases. None of the tumors with a safety margin of ≥2 cm relapsed. The majority of cases were found in the Dresden region., Conclusion: Atypical fibroxanthoma demonstrates an uneven geographic distribution in southeast Germany that demands further investigations. Mohs surgery with ≥2 cm safety margins is the treatment of choice. A regular follow-up is recommended., (© 2014 Wiley Periodicals, Inc.)

Wax models in dermatology were used to a large extent until the 1930s as three-dimensional models to illustrate various pathologic conditions and in particular cutaneous signs of disease. As an example, a young woman who was struck by lightning and developed Lichtenberg figures is presented. Lichtenberg figures are a fernlike pattern on skin, characteristic of lightning. Such a wax model housed in the German Hygiene Museum Dresden, Germany, illustrates the significance of wax models and how, even today, they can play an important role in medical education., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Background: Due to the increasing problem of antibiotic resistance in gram-negative pathogens, the Commission for Hospital Hygiene and Infection Prevention (KRINKO) decided to establish a new clinically oriented definition of multi-resistance. Gram-negative pathogens with a multidrug-resistance (MRGN) are divided into those with resistance to three (3MRGN) or four (4MRGN) antibiotic groups., Patients and Methods: In this multicenter study which was done in ten dermatological wound clinics, the bacteriological swabs from up to 100 patients with chronic leg ulcers per center were analyzed according to the current classification KRINKO and evaluated., Results: Overall, the results of 970 patients (553 women, 417 men) could be evaluated. We found 681 gram-positive and 1155 gram-negative bacteria. Pseudomonas aeruginosa was with a detection-rate of 31.1% the most frequent gram-negative pathogen, followed by Proteus mirabilis with 13.7% and various enterobacteria with 28.6%. According to the current KRINKO classification,eight patients with 4MRGN and 34 patients with 3MRGN could be identified., Conclusions: Our results demonstrate the current spectrum of bacteria in patients with chronic leg ulcers with a variety of gram-negative pathogens, some of which are classified as multi-drug resistant. As a clinical consequence some of the patients require individualized preventive measures and therapy.

Acquired melanocytic naevi (MN) are considered a risk factor for melanoma. Exposure to ultraviolet light (UV) is the major environmental factor for MN. UV protection is most critical in pre-school children. This 3-year interventional longitudinal study examined 395 3-year-old children attending daycare centres (DCC) in Dresden, Germany. Photo-skin type, eye and hair colour were recorded. DCC were randomly assigned to a control group and a behavioural intervention group. All children had a regular naevus check-up, including digital objective analysis with Dell'Eva-Burroni Dermoscopy Melanoma Image Processing Software (DB-MIPS) technology. Parents of children in the intervention group received additional guidance for sun-protection. The mean total MN counts of both groups at the start of the study period were 7.19 ± 4.55 (intervention) and 6.84 ± 4.63 (control), respectively. There was a significant increase in MN counts for both groups (mean 12.5 and 13.8). Subgroup analysis for skin type, eye colour, and hair colour did not demonstrate a significant influence on MN counts. The DB-MIPS integrated classifier revealed no risky lesions while analysing their patterns. Intervention did not reduce the number of newly acquired MN. MN counts in pre-school children were approximately 5 times higher than expected from previous large studies in Germany. This is the first study in pre-school children using objective digital image analysis of pigmented lesions. No atypical lesions were observed. New approaches to UV protection in pre-school children are now required.

Background: In almost every chronic wound different bacteria species can be detected., Patients and Methods: Retrospective, multicenter evaluation of bacterial swab results from 2010 and 2011 in patients with chronic leg ulcer treated in 10 dermatologic wound care centers from 5 regions in Germany., Results: Data of 970 patients were analyzed. Staphylococcus aureus was detected in 47.6 % of the patients, 8.6% of these were methicillin-resistant (MRSA). Pseudomonas aeruginosa was found in 31.1 %, enterobacteria in 28.6 % and Proteus mirabilis in 13.7 % of the patients. The regional comparison showed a significant south-north gradient for S. aureus, P. aeruginosa, and enterobacteria. Moreover, a highly significant west-east gradient for MRSA was found with detection rates of 13.5 % in the west to 4.0 % in the east. Furthermore, nationally there was a significant negative correlation between S. aureus and P. aeruginosa or P. mirabilis, respectively, as well as a positive correlation of P. aeruginosa with respect to wound size and duration. In addition to climatic and therapeutic conditions, different regional health care structures are discussed as potentially relevant reasons for these significantly different regional detection rates., Conclusions: Our data show the regional variability of the spectrum of currently detected bacteria in patients with chronic leg ulcers in Germany., (© The Authors | Journal compilation © Blackwell Verlag GmbH, Berlin.)

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.

Background: Phlebologic diseases have become extremely common and have major socio-economic impact. However, the percentage of dermatologists working in phlebology appears to be decreasing according to the data of the German Society of Phlebology (DGP)., Methods: To investigate the reasons for this development, we--on behalf of the DGP--sent a questionnaire to 120 German Departments of Dermatology in autumn 2012., Results: In 76 returned questionnaires, the number of physicians with additional fellowship training in phlebology averaged 1.5; the average number of those who fulfill the criteria for training fellows in phlebology was 0.9. In 71.1 % of the departments there was a phlebologist. A special phlebologic outpatient clinic existed in 73.7 % of the departments. Sonography with Doppler (89.5 %) and duplex (86.8 %) was used as the most frequent diagnostic tool. For therapy, compression (94.7 %), sclerotherapy (liquid 78.9 %, foam 63.2 %, catheter 18.4 %), endoluminal thermic procedures (radio wave 28.9 %, laser 17.1 %) and surgery (especially crossectomy and stripping 67.1 %, phlebectomy of tributaries 75 %) were used. The average number of treatments was very heterogenous in the different departments., Conclusions: Phlebology definitely plays an important role in dermatology. Most departments fulfill the formal criteria for the license to conduct advanced training in phlebology. A wide spectrum of phlebological diagnostic and therapeutic procedures is available.

Aesthetic medicine has become increasingly popular in the last two decades. The same trend has occurred in dermatology. Aesthetic dermatology prefers minimally invasive procedures. Nevertheless, even these procedures are not free of possible adverse effects. The spectrum of possible adverse effects, their management and prevention are discussed for four popular procedures in aesthetic dermatology, i.e. chemical peels, mesotherapy, botulinum toxin, and dermal fillers. Aesthetic procedures should only be performed by well-educated, well-trained medical doctors with an excellent medical background, never by lay persons.

The department of dermatology at the Hospital Dresden-Friedrichstadt (Dresden, Germany) was founded in 1874 as one of the oldest departments of dermatology in a municipal hospital in Germany. Dresden was the capital of Saxony and, as such, one of the most influential cultural centers of Germany. This particular situation is also reflected by the hospital's history itself.

Background: Early detection of cutaneous melanoma is the most important step to improve prognosis. In recent years objective techniques have been developed to improve diagnostics of pigmented lesions superior to the naked eye. Digital dermoscopy offers several advantages such as independence from the investigator, format ready for teledermatology and image storage, and comparability., Setting: In a routine setting including three centres in Europe, i.e. Lugano and Locarno in Switzerland, and Dresden in Germany, the usefulness of digital dermoscopy in daily routine has been evaluated., Patients and Methods: Digital dermoscopy was performed with the DB-MIPS system in 1308 patients, and 3544 pigmented lesions were evaluated. A total of 466 pigmented lesions were surgically removed and classified by histopathology. Among those 52 melanomas and 299 dysplastic, naevi could be identified. DB-MIPS system evaluated 35 variables of geometrics, colour, colour distribution and texture. Lesion identification is realized by clustering. Another important part of the analysis was to study the decision making, i.e. removal or follow-up of lesions. The tendency to follow-up was defined as the ratio of (number of suspicious lesions-removed lesions)/suspicious lesions. The interventism is defined as the ratio of the number of removed lesions to the number of suspicious lesions., Results: The DB-MIPS system was robust and easy in handling in daily routine. The data analysis and digital imaging were realized in real time. Fifty-two melanomas could be identified including eight in situ melanomas and 33 T1a melanomas (thinner than 1 mm). Sensitivity of digital dermoscopy varied between 90% and 95% between the three centres. Specificity varied between 79.6% and 93.3%. The centres differed in the patient population, the mean diameter of pigmented lesions and decisions. Interventism varied between 36.3% and 76.0%, follow-up varied from 24% to 63%. Higher levels of clinician's false positive were associated with a higher percentage of interventism., Conclusions: Digital dermoscopy offers advantages for daily routine in detection of early melanoma. Sensitivity and specificity for early melanomas is high and thereby, the experienced dermatologist can be easily supported in daily routine of a pigment lesion clinic to improve diagnostics and hopefully prognosis in cutaneous melanoma.

Molds are quite more often suspected as pathogens by the public than by the medical care community. Molds may, however, cause serious medical problems, and mold infections can develop incognito. Among the mycoses caused by opportunistic molds, alternariosis and fusariosis together with aspergillosis are of particular importance. They are more common than other groups with pathological characteristics. The aim of our presentation is to demonstrate the important role of common molds as causative agents in skin and ear infections. The clinical picture, etiology and pathogenesis, diagnosis and treatment, and course and prognosis of cutaneous infections will be given. The spectrum of clinical symptoms ranges from eczemalike lesions to chronic erythematous, verrucous lesions of the skin or multiple acute infiltrations of the dermis, occasionally forming abscesses. The mycologic direct preparation of the specimens, particularly with optical brighteners, and a histological examination of a skin biopsy are strongly recommended. The outbreak of cutaneous infections is triggered by weakened host defense mechanisms. A review of the literature regarding immunosuppressed and immunocompetent patients will be given.

Objective: Chronic leg wounds can represent a major health problem and have a negative emotional impact on patients' lives. The Cardiff Wound Impact Schedule (CWIS) was developed by the Wound Healing Research Unit at University of Wales College of Medicine to assess the quality of life in patients with chronic wounds., Method: To perform the linguistic validation (translation) of the CWIS into German, French and US English as a first step in making the questionnaire available globally. The standard linguistic validation process employed by the Mapi Research Institute was used to translate the CWIS into French and German. An adjusted process was used for the US English translation., Results: The linguistic validation of the US English version was straightforward as it shares the same root language with the original UK version. The problems encountered in the French and German translations were mostly related to semantics and syntax., Conclusion: The CWIS is now available for further validation testing by the international community (the psychometric evaluation of each translated questionnaire). These translations will be useful to health-care providers undertaking formal quality-of-life assessments of patients with chronic leg wounds.

Objective: To examine the role of hemochromatosis (HFE) gene mutations, which are associated with porphyria cutanea tarda (PCT), in the therapeutic response to chloroquine., Design: We retrospectively analyzed a database (Excel version 2001 [Microsoft Excel, Redmond, Wash]; date range of search, 1985-1999) of chloroquine-treated patients with PCT on whether HFE mutations (C282Y and H63D) might have influenced the clinical response, urinary porphyrin excretion, liver enzyme activities, and serum iron markers. Serum samples and corresponding complete sets of data before and after therapy were available in 62 of 207 patients with PCT who were treated exclusively with chloroquine., Settings: Academic teaching hospital., Intervention: For treatment, low-dose chloroquine diphosphate, 125 to 250 mg twice weekly, was used during a median time of 16 months (range, 12-26 months)., Results: Of the 62 German patients with PCT, 37 (60%) carries HFE mutations. Chloroquine therapy was accompanied by clinical remission and reduced urinary porphyrin excretion (P<.001) in the 24 patients (39%) with HFE wild type as well as in 35 HFE heterozygous patients with PCT (56%). Decreases of serum iron markers following chloroquine therapy were limited to patients with PCT and HFE wild type. All patients homozygous for the C282Y mutation (3 [5%] of 62) had high serum iron, ferritin, and transferrin saturation and failed to respond to chloroquine treatment., Conclusions: The therapeutic response to chloroquine was not compromised by C282Y heterozygosity and compound heterozygosity of HFE mutations. Because HFE C282Y homozygotes (+/+) did not respond to chloroquine and a decrease in serum iron concentration was limited to patients with PCT and HFE wild type, phlebotomy should be first-line therapy in patients with PCT and HFE mutations.

Objective: The incidence of melanoma is increasing. Melanoma patients are at risk for the development of second neoplasias. Data for the new German Bundesländer are not available, but would be suitable to define frequency, site and type of secondary malignancies and conclusions for follow-up of melanoma patients., Design: Retrospective study at the Melanoma Outpatient Clinic of the Department of Dermatology and Allergology at the University of Jena (Germany) for patients seen between June 1966 and June 1999. To investigate the impact of second malignancies on survival a case-comparison study of mortality was performed. The log-rank test and chi2-test were used to investigate statistical significance. There were 554 patients with malignant melanoma, 237 male and 317 female, with an age at time of diagnosis between 17.0 and 90.1 years (mean 53.7 years). The mean follow-up was 5.6 years., Results: Sixty-one patients (11.0%) developed a second tumour. The total number of tumours was 83. Forty-five patients developed one, 16 developed > or = 2 second tumours. Basal cell carcinoma (BBC) was the most frequent neoplasia (17 patients, 22 tumours; mean age 64.9 years). A second melanoma was found in 15 patients, while two developed a third melanoma. The mean tumour thickness was 0.81 mm (in second or third melanomas) compared with 1.92 mm of primary melanomas. Seven female patients developed breast cancer (eight cancers; mean age 57.3 years). The other second tumours included skin cancer (eight), gastrointestinal tract tumours (four), genital cancers (19), brain tumours (two), lung cancer (two) and other tumours (six). The difference in survival of patients with second tumours was not statistically significant from age-, sex- and melanoma thickness-matched controls., Conclusions: Second malignancies were seen in 11.0% of melanoma patients. Most important are second skin tumours such as second melanomas and BCC, recommending follow-up by the dermatologist. In a group of patients with regular follow-up examinations, no negative impact of second tumours (BCC, melanoma, breast cancer) on overall survival could be detected.

Fibromyalgia has been reported to occur with high prevalence in systemic lupus erythematosus. Data on fibromyalgia in other subsets of lupus erythematosus are not available. Risk factors for fibromyalgia have not been defined. We investigated 60 patients with different subsets of lupus erythematosus for the presence of fibromyalgia, association with clinical and laboratory parameters and disease activity. Our data were compared with the multicentre lupus erythematosus registry at the Free University of Berlin. Ten out of 60 patients with more than 11 tender points and widespread pain for more than 3 months were classified as positive for fibromyalgia. All of them were female. Fibromyalgia-positive patients suffered significantly more often from headache, morning stiffness, diffuse alopecia, muscle pain, arthralgia, renal involvement, and disclosed peripheral blood cell cytopenia, rheumatoid factor, hypergammaglobulinaemia and intake of corticosteroids and azathioprine. Fibromyalgia was more frequent in systemic lupus than in other lupus subsets. Evaluation of fibromyalgia symptoms and lupus disease activity was performed in 30 patients in a 1-year (range 9-13 months) follow-up. These 30 patients consisted of 9 fibromyalgia-positive and 21 fibromyalgia-negative patients. Both groups were characterized by stable clinical features such as number of tender points and ECLAM index. Fibromyalgia did not show a correlation with lupus activity. We suggest that fibromyalgia and lupus erythematosus are distinct complaints. Patients with lupus are at risk of developing secondary fibromyalgia. The clinical features of fibromyalgia-positive patients may contribute to misinterpretation of lupus activity.

Monilethrix is a rare dominant hair disease characterized by beaded or moniliform hair which results from the periodic thinning of the hair shaft and shows a high propensity to excess weathering and fracturing. Several cases of monilethrix have been linked to the type II keratin gene cluster on chromosome 12q13 and causative heterozygous mutations of a highly conserved glutamic acid residue (Glu 410 Lys and Glu 410 Asp) in the helix termination motif of the type II hair keratin hHb6 have recently been identified in monilethrix patients of two unrelated families. In the present study, we have investigated two further unrelated monilethrix families as well as a single case. Affected members of one family and the single patient exhibited the prevalent hHb6 Glu 410 Lys mutation. In the second family, we identified in affected individuals a lysine substitution of the corresponding glutamic acid residue, Glu 403, in the type II hair keratin hHb1, suggesting that this site represents a mutational hotspot in these highly related type II hair keratins. Both hHb1 and hHb6 are largely coexpressed in cortical trichocytes of the hair shaft. This indicates that monilethrix is a disease of the hair cortex.

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.