Behçet's disease (Adamantiades-Behçet's disease, ABD) is a multisystemic inflammatory disease, the pathogenesis of which is still a mystery. Many questions are still to be answered and the available diverse data need to be brought together to be compared and analysed. There is at least consensus on the effect of possible, but currently unknown, environmental triggering factor(s) against a background of genetic susceptibility. The possible aetiological factors form a broad spectrum, with infectious agents being the most probable ones. Whatever the stimulus is, the target tissue seems to be the small blood vessels, with various consequences of either vasculitis and/or thrombosis in many organ systems. The endothelium seems to be the primary target in this disease; however, it may just be the subject of the bizarre behaviour of the immune system. The diverse existing data could be interpreted in favour of either explanation. A similar confusion exists about the thrombotic tendency in Adamantiades-Behçet's disease, in terms of whether a primary hypercoagulability is present or whether it is secondary to inflammation. Recent interesting immunological data promise a way out of the existing dilemma. These findings will be outlined within the context of possible hypotheses and attention will be paid to further investigations that are needed.

Background: The Koebner phenomenon is defined as ‘the development of psoriasis at sites of traumatized skin’. The ‘all-or-none principle’ means that, if psoriasis occurs in one area of injury, all injured areas develop psoriasis or vice versa. The aim is to demonstrate the concordance of patients with the all-or-none principle when a standard method of trauma is employed. Methods: Sixty-two patients with psoriasis were enrolled in the study. Demographic data and disease characteristics were recorded. The medial aspects of both forearms, devoid of lesions, were pricked using two sets of five 30-gauge needles at an angle of 30°, with 2-cm intervals. On days 14 and 28, the patients’ forearms were checked for the presence of a typical psoriatic plaque of white scales on an erythematous papule. Results: On day 28, 45 patients (72.5%) had a negative Koebner response in all prick sites whereas 1 patient (1.6%) had psoriatic papules in 10 out of 10 prick sites. The rest of the patients (n = 16, 25.8%) had between 1 and 9 papules in number. Conclusion: Using standard methods of trauma, it is possible to induce psoriasis lesions as a Koebner response but this response is not always in concordance with the all-or-none principle previously described.

During periods of smoking, patients with Behçet's disease have less oral aphthae than in abstinence. To elucidate this observation, human keratinocytes and dermal microvascular endothelial cells (HMEC-1) were incubated with serum of 20 patients with Behçet's disease and 20 healthy controls for 4 hours. Maximum non-toxic concentrations were determined and the cells were further treated with 6 microM nicotine, 3.3% cigarette smoke extract (CES), 100 microM biochanin A, and 6.25/12.5 microM pyrrolidine dithiocarbamate alone and in combinations for 24 hours. Serum IL-8 levels of patients were significantly lower than those of controls. However, after 4 hours incubation with patients' sera, IL-8 release by both cell types was markedly increased when compared with the corresponding serum levels. The levels of IL-6 and vascular endothelial growth factor (VEGF) release were after 4 hours similar with the corresponding levels in serum. IL-1 was not detected. Nicotine significantly decreased IL-8 and -6 release by HMEC-1 maintained in both patients' and controls' sera, but only IL-6 release by keratinocytes maintained in patients' sera. VEGF release by both cells was markedly increased after nicotine treatment in either serum. CES significantly decreased IL-8 release and increased production of VEGF in keratinocytes maintained in patients' serum. The phytoestrogen biochanin A alone and in combination with nicotine further decreased the secretion of IL-8, -6, and VEGF in all experimental settings. Our data support a specific anti-inflammatory effect of nicotine on keratinocytes and endothelial cells maintained in the serum of patients with Behçet's disease. Moreover, biochanin A is likely to exhibit similar and even more profound results than nicotine.

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Stiff skin syndrome (SSS) is a disease similar to scleroderma with an unknown etiology. Stone-hard areas of skin are observed from birth or in early childhood. In this article we describe a 15-year-old girl with skin hardening and limitation of movement. We diagnosed the case as SSS, of which we have not encountered a similar report in the Turkish literature.

Atrophia maculosa varioliformis cutis (AVMC) was first described by Heidingsfeld in 1918, as a rarely reported form of idiopathic macular atrophy on the cheek (1). It is characterized, clinically, by shallow, sharply demarcated depressions in various shapes. Extrahepatic biliary atresia (2) and pachydermodactyly (3) have been the only conditions associated with AMVC reported in the past 80 years. Although keratosis pilaris is a common skin disorder, it is related to other idiopathic atrophic conditions considered in the differential diagnosis of AMVC, namely keratosis pilaris atrophicans (4). However, the two associations may be coincidental. We observed a patient with keratosis pilaris, and her brother and an unrelated young man, whose findings led to a diagnosis of AMVC.

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cyclical morphological changes from red papules to pigmented macules and finally cleared spontaneously; and (iii) histologically, there was prominent dilation of the superficial plexus, which was laden with red blood cells without vessel proliferation. We believe it appropriate to group these two cases in the category of telangiectasia. The clinical and histological findings justify the term ERPT as the lesions are not real angiomas, although they have the appearance of angiomas. A possible characteristic feature of ERPT may be that pathological hyperpigmentation of the basement membrane zone usually occurs ahead of the clinical demonstration of pigmented macules that form later in the course of the disease.Many skin disorders may assume an angioma-like appearance. Unlike our series, eruptive pseudoangiomatosis (EPA) generally resolves in no longer than 15 days, without a relapsing tendency. Our cases share the same pathological feature of large dilated blood vessels with EPA. However, EPA usually presents as plump hobnail-shaped endothelial cells that were lacking in our cases. 2 Papules in multiple pyogenic granuloma tend to be grouped in a localized area, 3 which is quite different from the random distribution in our cases. Pathologically, like our series, spider angioma, angiokeratoma, telangiectases and bacillary angiomatosis are characterized by dilation of blood vessels in the dermis. 1 However, our cases differ from these disorders in that eruptions were recurrent and showed both evolution and spontaneous resolution.Our patients presented typical pathological changes of telangiectasia, yet it is difficult to categorize them into any variant of telangiectasia that is recorded in the present literature. Telangiectasia in cutis marmorata telangiectatica congenita, hereditary haemorrhagic telangiectasia, ataxia-telangiectasia and hereditary benign telangiectasia occurs as a component of inherited disorders or congenital entities. 4-7 Unilateral naevoid telangiectasia consists of numerous thread-like telangiectases predominantly affecting the third and fourth cervical dermatomes. 8 In generalized essential telangiectasia, telangiectases appear first on the lower extremities and progress gradually and symmetrically to the trunk and arms. 9 Finally, our cases can be distinguished from telangiectasia macularis eruptiva perstans because of their normal mast cell count.We hypothesize that the lesions may result either from direct cumulative damage caused by ultraviolet radiation or from sunlight-induced antigen complex binding to the endothelium. The lesions represent a new type of telangiectasia, and we believe that the term ERPT is the most appropriate denomination for this entity.