IntroductionErectile dysfunction (ED) is common in men with systemic sclerosis (SSc) but the demographics, risk factors and treatment coverage for ED are not well known.MethodThis study was carried out prospectively in the multinational EULAR Scleroderma Trial and Research database by amending the electronic data-entry system with the International Index of Erectile Function-5 and items related to ED risk factors and treatment. Centres participating in this EULAR Scleroderma Trial and Research substudy were asked to recruit patients consecutively.ResultsOf the 130 men studied, only 23 (17.7%) had a normal International Index of Erectile Function-5 score. Thirty-eight per cent of all participants had severe ED (International Index of Erectile Function-5 score ≤ 7). Men with ED were significantly older than subjects without ED (54.8 years vs. 43.3 years, P < 0.001) and more frequently had simultaneous non-SSc-related risk factors such as alcohol consumption. In 82% of SSc patients, the onset of ED was after the manifestation of the first non-Raynaud's symptom (median delay 4.1 years). ED was associated with severe cutaneous, muscular or renal involvement of SSc, elevated pulmonary pressures and restrictive lung disease. ED was treated in only 27.8% of men. The most common treatment was sildenafil, whose efficacy is not established in ED of SSc patients.ConclusionsSevere ED is a common and early problem in men with SSc. Physicians should address modifiable risk factors actively. More research into the pathophysiology, longitudinal development, treatment and psychosocial impact of ED is needed.
Psoriatic arthritis (PsA) is a chronic inflammatory musculoskeletal disease affecting up to 30% of psoriasis vulgaris (PsV) cases and approximately 0.25% to 1% of the general population. To identify common susceptibility loci, we performed a meta-analysis of three imputed genome-wide association studies (GWAS) on psoriasis, stratified for PsA. A total of 1,160,703 SNPs were analyzed in the discovery set consisting of 535 PsA cases and 3,432 controls from Germany, the United States and Canada. We followed up two SNPs in 1,931 PsA cases and 6,785 controls comprising six independent replication panels from Germany, Estonia, the United States and Canada. In the combined analysis, a genome-wide significant association was detected at 2p16 near the REL locus encoding c-Rel (rs13017599, P=1.18×10−8, OR=1.27, 95% CI=1.18–1.35). The rs13017599 polymorphism is known to associate with rheumatoid arthritis (RA), and another SNP near REL (rs702873) was recently implicated in PsV susceptibility. However, conditional analysis indicated that rs13017599, rather than rs702873, accounts for the PsA association at REL. We hypothesize that c-Rel, as a member of the Rel/NF-κB family, is associated with PsA in the context of disease pathways that involve other identified PsA and PsV susceptibility genes including TNIP1, TNFAIP3 and NFκBIA.
We present a case of allergic contact dermatitis in an 18-month-old boy caused by type-IV allergy to mercapto mix and mercaptobenzothiazole as components of the elastic border of diapers. Allergic contact dermatitis should be included in the differential diagnosis of diaper dermatitis, especially in difficult-to-treat cases or atypical clinical presentation.
Reticular erythematous mucinosis (REM) syndrome primarily affects young women; the skin lesions usually appear on the chest and upper back. REM is diagnosed on the basis of the clinical picture and histological findings. REM syndrome is often associated with lupus erythematosus tumidus. Both diseases respond well to treatment with chloroquin. Topical tacrolimus and the use of a pulsed dye laser have fewer side effects and have also proved to be effective.
AnamneseEin dreijähriges Mädchen stellte sich mit mehreren, seit 3 Wochen bestehenden Knoten am Rücken, Hals und Gesicht in unserer Ambulanz vor. 3 Monate zuvor wurde extern eine Pneumonie sowie vor 2 Wochen eine fieberhafte Bronchitis diagnostiziert und behandelt.
HautbefundTemporal rechts, paravertebral beidseits und zervikal finden sich multiple, bis zu 1 3 1,5 cm große, relativ scharf begrenzte, derbe, gut verschiebliche hautfarbene und symptomlose Nodi. Nuchal, angulär und zervikal sind kleine indolente Lymphknoten palpabel (Abbildung 1a, b).
Histologie und ImmunhistochemieHistologisch zeigt sich unter unauffälliger Epidermis unter Freilassung eines schmalen Grenzbereichs ein zelldichtes Infiltrat, das teils knotenförmig, teils diffus das gesamte Korium bis zum subkutanen Fettgewebe durchsetzt. Das Infiltrat besteht aus überwiegend mittelgroßen lymphatischen, teils lymphoblastären Zellen mit hyperchromatischen Kernen sowie fokal prominenten Nukleolen. Insgesamt erhöhte mitotische Aktivität der lymphatischen Zellen. Immunhistochemisch färben die Zellen überwiegend positiv für CD 79 (Abbildung 2).
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