Patient characteristics in Behçet disease: a retrospective analysis of 213 Turkish patients during 2001-4.

BACKGROUND: Behçet disease (BD) is a chronic, inflammatory, multisystemic vasculitic disorder with a wide spectrum of clinical presentations. The highest prevalence is seen in Turkey. Specific diagnostic tools are yet to be discovered; thus, the diagnosis relies on physicians being acquainted with the symptoms and signs of the disease. OBJECTIVE: To investigate the epidemiologic characteristics of BD and to emphasize the typical clinical and laboratory characteristics. METHODS: This was a retrospective analysis of all the BD patients attending the Ankara Numune Education and Research Hospital throughout the years 2001-4. Diagnosis of BD was made according to the International Study Group criteria. A total of 213 patients were evaluated with respect to family history, clinical features, pathergy test, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), antistreptolysin O, and ferritin levels. When assessing disease activity, the active clinical manifestations on the day of the examination were taken into account. Correlations were analyzed between sex and age distribution, age of onset, disease duration, and family history; and between family history and age of onset, pathergy, clinical manifestations, and laboratory parameters. Correlations were also evaluated between pathergy positivity and clinical manifestations, and laboratory parameters. Correlations between activity scores and age of onset, duration, sex, family history, and laboratory data were also analyzed. RESULTS: The female : male ratio was 1.04, and the mean age of onset was 27 years. Family history did not affect age of onset or disease severity. Men presented with more active disease, and there was a weak but positive correlation between disease activity and CRP. No correlation was observed between duration and age of disease onset. The most common clinical presentations were oral aphthous lesions, genital ulcers, and skin lesions. Men more commonly presented with papulopustular lesions, pathergy positivity, and vascular, eye, and renal involvement, and women presented with arthritis/arthralgia more commonly than men. Vascular lesions, ESR, and CRP showed significant relationships with pathergy reaction. Eye involvement was not affected by age of onset. CONCLUSIONS: We believe our results indicate that the pathogenesis of BD is multifactorial. Hormonal factors seem to be of some influence, while genetic background and environmental factors seem to be the major contributors. Infections seem to be among the triggering environmental factors. Predisposing genes may affect the influence of environmental factors. Prevalence studies should be carried out periodically in those countries with a high prevalence of BD to keep up with the changing dynamics of the disease, which may also shed light on the as-yet unknown pathogenesis of BD.

Acquired aquagenic papulotranslucent acrokeratoderma: report of two cases.

Aquagenic papulotranslucent acrokeratoderma (APA) is an acquired, unusual condition characterized by bilateral, symmetric, translucent-to-white papules located on the hands and feet. These lesions are accentuated following a short period of water exposure. Since its first description in 1973 as hereditary papulotranslucent acrokeratoderma, only a few cases of APA have been reported. We report two cases of teenage girls with APA. Both patients responded well to 5% salicylic acid ointment but recurrence of lesions still occurred. The close temporal proximity of presentation of these two cases may indicate that APA is not as rare as previously thought.

Bone mineral density and bone turnover in patients with psoriatic arthritis.

Psoriasis is a common inflammatory skin disease, and conflicting data have been published about osteoporosis and bone turnover markers in patients with psoriatic arthritis. The aim of this study was to assess bone mineral density (BMD) and bone turnover markers in psoriatic patients with and without peripheral arthritis and to investigate the relationship between clinical parameters and markers of bone turnover. Forty-seven patients (24 women, 23 men) with psoriasis were included to the study. Demographic data and clinical characteristics were recorded. Erythrocyte sedimentation rate and C-reactive protein were assessed as disease activity parameters. BMD was determined for lumbar spine and total hip by dual X-ray absorptiometry (DXA). Serum Ca, P, alkalen phosphatase (ALP), and serum type I collagen cross-linked C telopeptide (CTX) were measured as bone turnover markers in all patients. The patients were divided into two groups according to their peripheral arthritis status. The clinical and laboratory variables, as well as bone mass status of the groups, were compared with each other. Eighteen patients had peripheral arthritis. All the female patients were premenopausal. None of the patients had radiologically assessed axial involvement. There was no significant difference between the BMD levels of psoriatic patients with and without arthropathy. One patient (5%) had osteoporosis, and nine (50%) patients had osteopenia in arthritic group, while eight (27.5%) patients had osteopenia in patients without arthritis. Serum CTX, ALP, Ca, and P levels were not significantly different in arthritic than in non-arthritic patients (p > 0.05). In patients with psoriatic arthritis, the duration of arthritis was negatively correlated with BMD values of lumbar spine and total femur and serum CTX levels, suggesting an association of increased demineralization with the duration of joint disease. In conclusion, psoriatic patients with peripheral arthritis with longer duration of joint disease may be at a risk for osteoporosis, which can require preventative treatment efforts.

Peripheral neuropathy in Behçet disease: an electroneurophysiological study.

The aim of this study was to determine the peripheral nerve involvement electrophysiologically in Behçet patients without clinically evident neurological signs and symptoms. Sixty-three patients who fulfilled the International Study Group Classification Criteria for Behçet's disease (BD) and 49 healthy control subjects were enrolled to the study. Conventional electrophysiological studies of peripheral nerves including F latencies were performed to all subjects. Thirty-one male and 32 female Behçet patients with a mean age of 33.6+/-11.1 years and (22 male and 27 female healthy control subjects with a mean age of 35.8+/-9.9 years were included to the study. All but four of the patients were active. In the BD group, electrophysiologically diagnosed neuropathy was detected in nine (14.28%) patients. One (1.58%) patient had sensorimotor polyneuropathy, one patient (1.58%) had sural and ulnar sensorimotor neuropathy, three (4.75%) patients had median and one patient (1.58%) had ulnar sensorimotor neuropathy. Sural nerve sensorial action potential was unobtainable in two (3.17%) patients and prolonged F latencies were observed in two (3.17%) patients. In the control group only one subject (2.4%) had low sural sensorial conduction velocity. The frequency of neuropathy was higher in the patients with BD when compared with the control subjects. Sensory nerves were affected more prominently than motor nerves. There was no relationship between the clinical and laboratory characteristics of the patients and the electrophysiologic findings. No significant difference was observed between the clinical parameters of the patients with and without electrophysiologically detected neuropathy, except the levels of disease duration (8.8+/-5.1 vs 5.28+/-4.3 years, respectively, p<0.05). In conclusion, Behçet patients may have subclinical peripheral nerve involvement. Conventional electrophysiologic nerve conduction studies including F responses are recommended in routine examination to diagnose early neuropathy in Behçet patients without evident neurologic symptoms.

Mycosis fungoides mimicking nevoid hyperkeratosis of the nipple and areola in an adolescent.

Mycosis fungoides is one of the great imitators in dermatology; it can mimic many dermatoses. Nevoid hyperkeratosis of the nipple and areola is a rare idiopathic disease with typical clinical features of verrucous thickening and brownish discoloration of the nipple, areola or both. Here, a 16-year-old male patient with mycosis fungoides mimicking nevoid hyperkeratosis of the nipple and areola has been reported. To our knowledge, this is the first atypical MF patient to have presented with a NHNA-like lesion. Although the clinical appearance of nevoid hyperkeratosis of the nipple and areola is highly characteristic for diagnosis, histopathological examination is recommended, especially in cases with atypical features such as unexpected age, male gender and unilateral location.

Prevalence of metabolic syndrome in Behçet disease: a case-control study in Turkey.

BACKGROUND: Chronic inflammatory diseases such as psoriasis, rheumatoid arthritis, and inflammatory bowel diseases have been reported to be associated with the development of metabolic syndrome (MetS), which is characterized by central obesity, elevated triglycerides (TG), reduced high-density lipoproteins (HDL), impaired fasting blood glucose (FBG), and hypertension. Behçet disease (BD) is a chronic, immuno-inflammatory disease with multisystemic involvement. OBJECTIVE: The aim of this study was to investigate the prevalence and risk factors for MetS in patients with BD. METHODS: All patients had BD according to the criteria of the International Study Group. Diagnosis of MetS was established according to National Cholesterol Education Program Adult Treatment Panel III (NCEP ATP III) criteria. Mean waist circumference, body mass index (BMI), FBG, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), total cholesterol, HDL, very low-density lipoprotein (VLDL), low-density lipoprotein (LDL), TG, systolic BP, and diastolic BP were measured and analyzed. RESULTS: A total of 86 patients and 72 healthy controls were included. MetS was detected in 35.4 % of patients and 20 % of controls (p = 0.04). Patients with BD had a 2.67-fold higher risk for MetS than healthy controls (p < 0.05). Significant risk factors for developing MetS according to multivariate analyses were BD, age, and BMI. Age at onset of the disease, duration of disease, BMI, gastrointestinal system involvement, and neurological involvement were correlated with increased MetS risk (p < 0.05). MetS tended to increase with age and the duration of the disease and was higher in women under the age of 40 years compared with healthy controls in the same age group. CONCLUSION: All BD patients should be closely monitored for hypertension, hyperlipidemia, and diabetes mellitus to avoid MetS development.

Coeliac disease-associated antibodies in psoriasis.

BACKGROUND: The possible relationship between psoriasis and coeliac disease (CD) has been attributed to the common pathogenic mechanisms of the two diseases and the presence of antigliadin antibodies in patients has been reported to increase the incidence of CD. OBJECTIVE: The aim of this report was to study CD-associated antibodies serum antigliadin antibody immunoglobulin (Ig)A, IgG, anti-endomysial antibody IgA and anti-transglutaminase antibody IgA and to demonstrate whether there is an increase in the frequency of those markers of CD in patients with psoriasis. METHODS: Serum antigliadin antibody IgG and IgA, antiendomysial antibody IgA and anti-transglutaminase antibody IgA were studied in 37 (19 males) patients with psoriasis and 50 (23 males) healthy controls. Upper gastrointestinal endoscopy and duodenal biopsies were performed in patients with at least one positive marker. RESULTS: Antigliadin IgA was statistically higher in the psoriasis group than in the controls (p<0.05). Serological markers were found positive in 6 patients with psoriasis and 1 person from the control group. Upper gastrointestinal endoscopy was performed in all these persons, with biopsies collected from the duodenum. The diagnosis of CD was reported in only one patient with psoriasis following the pathological examination of the biopsies. Whereas one person of the control group was found to be positive for antigliadin antibody IgA, pathological examination of the duodenal biopsies obtain from this patient were found to be normal. CONCLUSION: Antigliadin IgA prominently increases in patients diagnosed with psoriasis. Patients with psoriasis should be investigated for latent CD and should be followed up.

Flare-up of pustular psoriasis with fluoxetine: possibility of a serotoninergic influence?

In this article, we report two cases of pustular psoriasis flaring up after fluoxetine administration. A 21-year-old male patient with localized pustular psoriasis became erythrodermic following commencement of fluoxetine. Even though the lesions were unresponsive to cyclosporine A (Cyc A) treatment, dramatic resolution was observed with discontinuation of fluoxetine. A 44-year-old female patient with pustular psoriasis who was on Cyc A and acitretin therapy was given fluoxetine for her psychiatric symptoms. In the following 5 days, her lesions flared. Owing to previous experience, fluoxetine was stopped. Her lesions improved dramatically in the following 3 days. Exacerbation of psoriasis with antidepressant therapy has been rarely described. An extensive review of the literature revealed four such cases, all of which were seen after the use of selective serotonin reuptake inhibitors (SSRI). A serotoninergic influence in the etiopathogenesis of psoriasis may be possible together with a pharmacogenetic difference in the drug metabolism of these patients. Considering the two patients we presented and the patients previously reported in the literature, aggravation of pustular psoriasis by SSRI should be borne in mind.

Serum lipoprotein (a) levels and Behçet's disease: is there an association?

BACKGROUND: Behçet's disease (BD) is a multisystemic inflammatory disorder found in individuals with a particular genetic background. Hemostatic studies in BD support an imbalance towards a prothrombotic state at different levels. Lipoprotein (a) (Lp(a)) has atherogenic and thrombogenic properties. It is mostly under genetic regulation. We investigated the possible relationship between Lp(a) and BD. METHODS: Forty patients diagnosed with BD and 40 healthy controls were enrolled. The clinical characteristics of the patients were recorded. Serum total cholesterol, high-density lipoprotein (HDL), very-low-density lipoprotein (VLDL), low-density lipoprotein (LDL), apolipoprotein A1, apolipoprotein B, and Lp(a) levels of the two groups were assessed and compared statistically. RESULTS: All patients (100%) had oral aphthous ulcers. Thirty (75%) had genital ulceration, 37 (92.5%) had either erythema nodosum or papulopustular lesions, and 10 (25%) had eye involvement. Twelve (30%) had a positive pathergy test. Four (10%) had vascular involvement. The Lp(a) level of the patient population was 19.6+/-18.8 mg/dL. This level was higher than that of the controls, but not statistically significant. The Lp(a) levels of the four patients with vascular complications were within normal limits. CONCLUSIONS: Lp(a) is of interest, as it is a genetically determined parameter that was found to be high in BD patients in our study group. The levels were independent of thrombotic complications, perhaps suggesting a different role for this lipoprotein in the etiopathogenesis of BD. Further studies with a larger number of patients are essential to discover the exact role of Lp(a) in BD.

Contact dermatitis due to Allivum sativum and Ranunculus illyricus: two cases.

Plants are of relevance to dermatology for both their adverse and beneficial effects on skin and skin disorders respectively. Virtually all cultures worldwide have relied historically, or continue to rely on medicinal plants for medical care. As alternative herbal remedies are becoming more widely used there is an increase in phytocontact dermatitis. Here we document two patients who developed contact dermatitis due to Allivum sativum, and Ranunculus illyricus after applying to the skin in order to relieve the rheumatological joint pain.