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Genital Ulcerations (genital + ulceration)
Selected AbstractsNumber VII Behçet's disease (Adamantiades syndrome)ORAL DISEASES, Issue 2 2006M Escudier Behçet's syndrome (BS; Adamantiades syndrome) is the association of the triple symptom complex of recurrent aphthous stomatitis (RAS) with genital ulceration, and eye disease (especially iridocyclitis) though a number of other systemic manifestations may also be seen. BS mainly affects young adult males, and there is an association with HLA-B5 and HLA-B51 (B5101). Features such as arthralgia and leucocytoclastic vasculitis suggest an immune-complex mediated basis, which is supported by finding circulating immune complexes and, although the antigen responsible is unidentified, heat shock proteins have been implicated. An inflammatory disorder, BS is now considered as a systemic vasculitis, characterised by a very wide spectrum of clinical features and by unpredictable exacerbations and remissions. [source] Behçet's disease and vitiligo in two brothers: coincidence or association?CLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 8 2009M. Borlu Summary Behçet's disease (BD) and vitiligo are disorders with unknown aetiology. We report on two brothers with ocular Behcet's disease who had advanced unilateral ophthalmic symptoms associated with vitiligo. The two brothers had recurrent oral and genital ulceration, uveitis and white patches on their skin. The most probable hypothesis for the aetiology of BD is that of an autoimmune reaction in genetically predisposed individuals, with vasculitis as the main pathological hallmark. Despite many years of research, the specific causes of vitiligo remain obscure, and the most advanced aetiological hypothesis remains that of autoimmunity. To our knowledge, this is the first reported case within the literature of BD associated with vitiligo. The existence of the two different disorders is noteworthy as they were observed in two brothers during the same period in their lives, with very similar clinical observations. [source] A case of Adamantiades-Behçet disease with ischemic optic neuritis (posterior optic neuropathy)JOURNAL DER DEUTSCHEN DERMATOLOGISCHEN GESELLSCHAFT, Issue 11 2007Satoko Shima Summary Adamantiades-Behçet disease (ABD) may present with cutaneous and ophthalmologic finings. A 29-year old woman complained of fever and general fatigue, along with erythema nodosum and vesiculo-pustular lesions on the legs, acneiform lesions, genital ulcerations and painful oral ulcers. She also complained of reduced visual acuity, visual disturbance and blurred vision in the left eye. Her left visual acuity was 6/20. Light reflex in the left eye was reduced. The relative afferent pupillary defect (RAPD) was positive in the left eye where a central scotoma was present. The vitreous was clear; the optic disc, macula, retina and iris were all normal. Uveitis was not observed. The patient was diagnosed with ischemic optic neuritis (posterior optic neuropathy) with ABD. Histopathological findings taken from a blister on the leg showed subepidermal bulla, dense dermal neutrophil infiltration, and extravasation of erythrocytes, suggesting leukocytoclastic vas-culitis. She was treated orally with high-dose corticosteroids (methylprednisolone 500 mg/d) for three days. Her general condition and ophthalmic symptoms resolved completely. Optic neuropathy with ABD is very rare; we know of two previous cases [1, 2] of ABD with ischemic posterior optic neuritis. [source] Study on association between polymorphism of HLA-DRB1 alleles and Behçet's diseaseJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 12 2009Y-B Shang Abstract Background, Behçet's disease (BD) is known to be associated with human leucocyte antigen (HLA)-B*51 in many ethnic groups. However, the association of HLA class II gene with BD has been described to be different according to different countries and regions. Objective, This study aims to investigate the association between polymorphism of HLA-DRB1 alleles and BD. Methods, Forty patients with BD and 100 healthy controls were typed for HLA-DRB1 alleles by the LABTypeTM SSO method. Results, The frequency of HLA-DRB1*14 was significantly higher in BD patients than in controls (P < 0.05), while the frequency of HLA-DRB1*15 was markedly lower in BD patients (P < 0.05). Regarding clinical manifestations, the frequency of HLA-DRB1*15 was significantly decreased in BD patients with genital ulcerations compared with controls (P < 0.05); the frequency of HLA-DRB1*14 was significantly increased in BD patients with erythema nodosum,like lesions and in BD patients with folliculitis-like lesions when compared to controls (P < 0.05, respectively). Moreover, the frequency of HLA-DRB1*14 was significantly increased in BD patients under 20 years of age at the onset of disease (P < 0.01), while the frequency of HLA-DRB1*15 was significantly decreased in them (P < 0.05), compared with controls. Conclusion, The results suggested that HLA-DRB1 alleles might play an important role in the onset and clinical manifestations of BD. [source] Behçet Disease in a Child,Emphasis on Cutaneous ManifestationsPEDIATRIC DERMATOLOGY, Issue 5 2007Vânia Oliveira de Carvalho M.D. Recurrent episodes of oral and genital ulcerations, skin lesions, and ocular manifestations are seen. The disease may also involve the central nervous system, gastrointestinal tract and, less frequently, the large vessels. In general, manifestations occur in the third or fourth decade of life and are not common in children. Therefore few data concerning this age group have been found in the literature. In this study we report a child with Behçet disease beginning at 1 year of age whose cutaneous manifestations were exuberant acne-like and folliculitis-like lesions, which were crucial for diagnostic confirmation. [source] |