Aylin Kalayciyan

An update on Behçet's disease

Behçets disease (Adamantiades‐Behçets 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‐Behçets 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.

Experimental Koebner phenomenon in patients with psoriasis.

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.

A retrospective analysis of patients with pemphigus vulgaris associated with pregnancy

SIR, Interferon alfa (IFN-a) therapy is highly effective in patients with chronic myelogenous leukaemia and other malignant diseases. Most side-effects, such as chills, malaise, myalgia and fatigue, are transient and tend to disappear with ongoing therapy, while others, such as anorexia, neurological and mood disorders, are dose-related and need dose adjustment or withdrawal of IFN-a. Induction of immunemediated disease, including thyroid disease, rheumatoid arthritis, systemic lupus erythematosus, vasculitis, thrombocytopenia, autoimmune haemolytic anaemia, and insulin-dependent diabetes mellitus, has also been described. Moreover, other immunological entities, such as myasthenia gravis, vitiligo, lichen planus, Henoch–Schönlein purpura and Sjögren syndrome, have been reported after IFN-a therapy for chronic viral hepatitis or multiple sclerosis (MS). Here, we report on the first case of systemic sclerosis (SSc) developing after therapy with IFN-a for chronic myelogenous leukaemia. In October 1998, a 52-year-old-woman without a personal or a familiar history of autoimmune disease, was diagnosed as having chronic myelogenous leukaemia, with a karyotype 46,XX,t(9;22), bcr ⁄ abl positive in all the metaphases. She was given hydroxyurea 20 mg kg day for 2 months and then IFN-a 2a to a maximum daily dose of 10 · 10 U. In June 1999, cytogenic evaluation showed a normalization of the karyotype in up to 33% of metaphases. Meanwhile, treatment was reduced to 6 · 10 U day for the appraisal of arthralgias, ataxia and depression. In June 2000 a further evaluation revealed the loss of efficacy of therapy (Ph chromosome positive in all the analysable metaphases). In the same period, the patient experienced fever, dyspnoea and oedema of the extremities. Laboratory findings were normal, except for an increased erythrocyte sedimentation rate (ESR, 50 mm in the first hour). Chest X-ray and computed tomography (CT) of the lung demonstrated the presence of pulmonary vascular congestion. Cardiac function, evaluated by electrocardiogram and Doppler echocardiography, was normal. The possible diagnosis of congestive heart failure was discarded and the patient’s symptoms were ascribed to a leak capillary syndrome secondary to interferon therapy; interferon therapy was interrupted. Peripheral oedema and respiratory symptoms improved on treatment with diuretics. Nevertheless, 3 months later, in November 2000 she experienced dyspnoea, oedema of both the upper and lower limbs and progressive skin thickening of the hands and wrists. A further echocardiographic evaluation revealed a normal ejection fraction (70%) and minimal tricuspid regurgitation with increased pulmonary artery pressure (34 mmHg). CT of the chest showed a picture of diffuse parenchymal and interstitial fibrosis with multiple areas of ground-glass attenuation. Pulmonary function tests demonstrated the presence of reduced volumes with an impaired diffusion capacity for carbon monoxide (DLCO, 46% predicted). Oesophagogastroduodenoscopy showed a total absence of peristalsis. Laboratory examination indicated: elevated ESR (80 mm in the first hour), impaired renal function (creatinine, 1Æ8 mg dL) with mild proteinuria (72 mg 24 h) and positive antisclero-70 antibodies. Her complete major histocompatibility phenotype was HLA-DRB1*11; 07, DQA*05;0201, DQB*03;02, A28, A24, B35, B13, Bw4, Bw6, Cw4. Capillaroscopy findings were consistent with scleroderma, showing multiple microhaemorrhages, dilation, distortion and rarefaction of capillaries. No skin biopsy was performed. A diagnosis of localized SSc was put forward, according to LeRoy et al. The patient was given loop diuretics, cyclophosphamide 100 mg day, prostanoids (iloprost, 2 ng kg min for 8 h for five consecutive days and then thrice monthly), steroids (methylprednisolone 750 mg intravenously for 3 days and then prednisone 25 mg day). In the following months the patient’s clinical condition improved, scleroedema decreased, the skin thickening partially receded and the signs of alveolitis were no longer detectable at a further CT evaluation of the lung (October 2001). Therapy has been gradually tapered and the patient is now receiving cyclophosphamide 50 mg day, prednisone 12Æ5 mg day, iloprost 2 ng kg min for 8 h every 3 weeks and hydroxyurea 10 mg kg on every alternate day. The patient’s main clinical and laboratory findings, before and after 10 months of therapy, are summarized in Table 1. Several authors showed that IFN-a therapy for malignancies, chronic viral hepatitis or MS may trigger the formation of autoantibodies directed toward various substrates, such as thyroid, platelets, erythrocytes, pancreas, parietal cells and nuclei. Their occurrence is sometimes, but not necessarily, coupled with the onset or the exacerbation of autoimmune diseases. In most cases endocrine disorders are described, but rheumatic and collagen–vascular diseases have been observed as well. Nevertheless, to date, no reports of SSc after interferon therapy have been made. However, Wandl et al. noticed that patients who develop antinuclear antibodies may experience Raynaud’s phenomenon, one of the diagnostic findings in SSc. Moreover, Black et al. pointed out that IFN-a therapy in the treatment of scleroderma may be deleterious, exacerbating life-threatening symptoms and precipitating the lung deterioration. As far as our patient is concerned, the laboratory findings, typical vascular alterations and ⁄ or clinical signs of autoimmunity are ascribable to interferon therapy. The patient had neither a personal nor a familiar history of autoimmunity, and a clear temporal relation between IFN-a administration and the occurrence of initial symptoms can be seen. Besides, she did receive IFN-a continuously for approximately 2 years. Fattovich et al. indeed noticed that de novo immuneBritish Journal of Dermatology 2002; 147: 385–410.

Stiff skin syndrome: a case report.

Abstract: 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.

Familial atrophia maculosa varioliformis cutis

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.

Psoriasis, enteropathy and antigliadin antibodies

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. Papules in multiple pyogenic granuloma tend to be grouped in a localized area, 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. 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. Unilateral naevoid telangiectasia consists of numerous thread-like telangiectases predominantly affecting the third and fourth cervical dermatomes. In generalized essential telangiectasia, telangiectases appear first on the lower extremities and progress gradually and symmetrically to the trunk and arms. 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.

Milia in regressing plaques of mycosis fungoides: provoked by topical nitrogen mustard or not?

Objective  To report three cases of mycosis fungoides with milia formation in the regressing lesions.

Red fluorescence on the teeth of psoriatic patients.

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