Background: Eosinophilic fasciitis (EF) is a rare fibrosing disorder associated with peripheral eosinophilia and scleroderma-like induration of the distal extremities which affects substantially quality of life. Although the disease has been described 30 years ago, the etiology and pathomechanisms are still obscure, and consensus for therapy is lacking. Numerous case reports of patients with EF exist but series are scarce. Patients and Methods: Eleven patients with EF from the Department of Dermatology, Kantonsspital Aarau, the University Hospital Basel and the Outpatient Clinic of Dermatology, Triemli Hospital Zurich, Switzerland, were retrospectively studied. Results: In 4 patients the initial diagnosis was not recognized by the referring nondermatologists. The median age was 55 years, excluding the youngest patient ever diagnosed with EF (age = 1 year). All patients showed an induration of the skin, which led to painful contractures in the joints in 3 cases. All but 2 patients demonstrated edema. A slight predominance of the upper extremities was observed. Sclerodactyly was noticed in 1 patient. Three patients reported an initial trauma at the affected site. Two patients were tested positive for borreliosis. One patient subsequently developed aplastic anemia and Hashimoto thyroiditis. Visceral or extracutaneous involvement was absent. Eight patients had a full or partial recovery under corticosteroids whereas in 2, improvement could be achieved only with cyclosporine, azathioprine or cyclophosphamide. Conclusions: The diagnosis of EF can be established by clinical, laboratory and histological findings. In general, corticosteroids are highly efficacious in EF and only a minority of patients need other immunosuppressive or cytostatic drugs.

Background: Scarce data exist concerning dermatological consultations within departments of internal medicine. To date, no survey has been carried out in Switzerland to elucidate this issue. The aim of this study was to analyze the spectrum of skin diseases internists are confronted with and to study their diagnostic accuracy in cutaneous diseases. In addition, we wanted to evaluate the motivation for dermatologists to cooperate closely with internists. Patients and Methods: The study included patients with dermatological problems treated at the Department of Internal Medicine at the Kantonsspital Aarau, Switzerland. All patients had been referred to the Department of Dermatology for examination between 1999 and 2001. Patient data were analyzed demographically, by referral modus, diagnoses and therapy. To evaluate the knowledge of internists and dermatologists in cutaneous medicine, 15 clinical slides of common dermatoses with a patient history were shown and asked for diagnostic suggestions to 32 internists of the Kantonsspital Aarau and to 13 dermatologists of the University Hospital Basel, Switzerland. Results: 1,290 patients were referred to the Department of Dermatology. 1,737 dermatological diagnoses were made including 348 different dermatoses. Eczema was the single most common diagnosis (12.6%), followed by actinic and bowenoid precancerosis (6.2%), drug eruption (4.2%), verrucae (4%) and mycosis (3.8%). The top ten diagnoses accounted for 41.7% of all skin-related diagnoses. Infection-related dermatoses were most common (20.5%) followed by different types of eczema (12.6%), malignant cutaneous tumors and malignant visceral conditions (11.2%). Local therapy was prescribed in 64.2% and systemic therapy in 22.6% of the patients. 15.9% did not receive specific therapy because the consultation request was only a diagnostic one. 146 skin biopsies were performed (11.3%). Systemic diseases with cutaneous manifestations accounted for 15.7%. In general, these conditions were not commonly seen by dermatologists in daily practice. The internists recognized 51.1% of the cutaneous manifestations during examination and 49% when presented with slides. Conclusions: Internists are confronted with a different spectrum of cutaneous diseases compared with dermatologists. Due to the broad spectrum of skin diseases, it is a challenging task for internists to recognize dermatoses. Our study elucidates that patients, internists and dermatologists may profit from a close cooperation.

The presence and distribution of mitotic figures is an important discriminatory parameter in the assessment of melanocytic lesions. We evaluated the number and distribution of mitotic figures in 353 randomly collected melanocytic nevi of various subtypes by hematoxylin and eosin (H&E) staining and immunohistochemically with the 2 mitotic markers Phospho-Histone H3 Ser28 (PHH3) and MPM2. At least 1 mitotic figure was present in 19.5%, 31.3%, and 42.8% of H&E-, PHH3-, and MPM2-stained lesions, respectively. In common compound nevi, the mean number of dermal mitoses amounted to 0.024/mm dermal surface area in the H&E staining (PHH3: 0.061; MPM2: 0.087) and to 0.175/mm in Spitz nevi (PHH3: 0.325; MPM2: 0.45). Nevi exhibiting mitotic figures were significantly more frequent in the youngest age group (0-20 years) than in patients older than 50 years (P < 0.0001). In the upper half of the dermis, mitotic activity was roughly 3 times as frequent as compared with the lower half. Clusters of mitotic figures within the dermis were not observed. Mitotic activity in obviously benign melanocytic nevi is not rare even in the deep dermal part. More than 2 mitotic figures per lesion can usually be explained either by the nevus subtype, young patient age, traumatization, or inflammation. PHH3 and MPM2 are a valuable diagnostic adjunct in the evaluation of melanocytic tumors allowing more sensitive and faster recognition of mitotic figures and their distribution.

Objective: Pulmonary arterial hypertension (PAH) is a severe complication of systemic sclerosis (SSc). In this longitudinal study, we aimed to identify factors associated with an unfavourable outcome in SSc patients with early PAH (SSc-PAH) from the DETECT cohort. Methods Conclusion:More than 40% of early-diagnosed SSc-PAH patients had disease progression during a short follow-up time, with male gender, functional capacity, and pulmonary function tests at PAH diagnosis being associated with progression. This suggests that even mild PAH should be considered a high-risk complication of SSc. Abstract word count: 192

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About 70% of patients with rheumatoid arthritis (RA) in any clinical cohort are seropositive.1 In early RA cohorts, barely 50% are seropositive indicating that a diagnosis of RA can be established without serological markers. However, there is evidence that seropositive and seronegative RA behave differently in short term studies: van Dongen et al2 showed that in anti citrulline peptide (CCP) positive patients, early MTX therapy postponed diagnosis of RA and prevented erosions statistically significantly better compared to placebo, which difference could not be seen in CCP negative patients. Few long-term studies have re-evaluated patients who have received a diagnosis of seronegative RA. In a 23-year follow-up study, Jäntti et al3;4 showed that psoriatic arthritis and mild spondylarthropaties are common in these patients. Early RA is an emergency case; patients have to be treated without delay to prevent damage, functional deterioration, work disability, and other consequences of inflammatory joint diseases. As diagnosis of RA is based on consensus criteria, patients with inflammatory joint diseases that mimic early RA can be included in early RA cohorts. Only careful follow-up of patients over many years and re-evaluation of diagnosis in all patients will reveal diagnoses behind symptoms that were classified as seronegative RA. We present seronegative cases who were classified and treated as early rheumatoid arthritis and were later revealed various diagnoses. The objective of this session is to remind of the necessity to re-evaluate diagnosis of patients with persistent seronegative “RA”. Sometimes wrong diagnosis may be a reason for poor response to anti rheumatic medications. References Sokka T, Kautiainen H, Pincus T, Toloza S, da Rocha Castelar PG, Lazovskis J et al. Disparities in rheumatoid arthritis disease activity according to gross domestic product in 25 countries in the QUEST-RA database. Ann Rheum Dis 2009; 68(11):1666-1672. van Dongen H, van Aken J, Lard LR, Ronday H.K, Hulsmans H.M.J., Speyer I et al. Efficacy of methotrexate treatment in patients with probable rheumatoid arthritis: A double-blind, randomized, placebo-controlled trial. Arthritis Rheum 2007; 56:1424-1432. Kaarela K, Jantti JK, Lehtinen K. Unclassified early arthritis is often spondyloarthritis. J Rheumatol 2003; 30(6):1393. Jantti JK, Kaarela K, Lehtinen KE. Seronegative oligoarthritis: a 23-year follow-up study. Clin Rheumatol 2002; 21(5):353-356. Disclosure of Interest None Declared