Pathergy is the term used to describe hyper-reactivity of the skin that occurs in response to minimal trauma. A positive skin pathergy test (SPT), characterised by erythematous induration at the site of the needle stick with a small pustule containing sterile pus at its centre, is among the criteria required for a diagnosis of Behçet's disease (BD) and in certain population has been shown to be highly specific for this condition. Problems with standardising the induction manoeuvre for the SPT as well as the method of assessment of the response have limited the usefulness of the SPT in the clinical setting. Extensive investigation into histopathological and immunological aspects of pathergy has led to a number of hypotheses relating to the aetiology of the skin pathergy reaction and the disease itself, but the cause is considered to be unknown. Pathergy lesions, the development of new skin lesions or the aggravation of existing ones following trivial trauma, are also reported in pyoderma gangrenosum and has been noted in other neutrophilic dermatoses such as Sweet's syndrome. The response of such patient groups to the systematic application of the SPT has not been described. We propose that a new way of considering the pathergy reaction is to see it as an aberration of the skin's innate reactivity from a homeostatic reactive mode closely coupled to tissue healing to an abnormal destructive/inflammatory mode. Our understanding of BD and other similar conditions would profit by more detailed mechanistic knowledge of skin homeostasis to minimal trauma in both health and disease through a more structured and systematic use of the SPT.

Individual variability in skin reactivity and healing capacity after trauma are important clinical issues. The aims were to develop an in vivo, human wound model based on a standardised minimal skin injury and to demonstrate therapeutic effect of simple wound therapies in terms of morphological wound outcome with changes in skin blood perfusion as a quantified indicator of wound healing. In a series of experiments, wounds were induced on the normal forearm skin of volunteers using a blood collection lancet. This was well tolerated. Wounds were assessed by naked eye examination or laser Doppler perfusion imaging (LDPI) at baseline and at up to 6 further time points up to 96 h in control wounds and wounds treated by commonly used occlusive dressing options. Assessment by clinical observation with 10x magnification showed over 96 h a progression of erythema, surface crust, a new keratinisation layer and finally healed areas. LDPI quantifying wound erythema showed a peak at 24 h and near normal levels at 96 h. Inter-individual variability was evident but intra-individual variability was much less pronounced. Wounds treated with occlusion showed a statistically significant more rapid return to baseline blood perfusion as measured by LDPI compared to controls supported by favourable healing parameters in the clinical assessment. The paper exemplifies use of non-invasive, bioengineering technique for quantification of individual innate variability in skin reactivity, wound healing capacity and therapeutic effect in a well-tolerated in vivo, human, minimal skin trauma model.

A 55-year-old woman presented with multiple cutaneous leiomyomas and multiple uterine leiomyomas (fibroids). The clinical diagnosis of the autosomal dominant hereditary leiomyomatosis and renal cell cancer (HLRCC) syndrome was confirmed by identification of a fumarate hydratase gene mutation. This case highlights the need to consider the possibility of renal and uterine cancer in members of cutaneous leiomyomatosis families.

been under the treatment of sulfasalazine and he has remained asymptomatic. Conclusion Calciphylaxis may appear without renal failure and hyperparathyroidism. Long-term steroid using in RA patients may lead to calciphylaxis. Acquired protein S deficiency may contribute to this process. AB0094 PREVALENCE OF TWIN BIRTHS IN BEHÇET'S DISEASE (BD)