A case of Klippel-Trenaunay syndrome involving only upper limbs.

The case of a 14-year-old girl suffering from Klippel-Trenaunay syndrome associated with multiple port-wine stain type vascular anomalies and varicose veins involving upper limbs is described. Finger deformations are common in Klippel-Trenaunay syndrome. In our patient, striking hypertrophy of soft tissues and overgrowth of bones were observed on both forearms and finger deformations were observed particularly on right thumb and index finger. Although lower limb involvement is very common amongst the patients with Klippel-Trenaunay syndrome, in our patient involvement of the lower limbs was not observed.

The effects of anastrozole on neonatal rat skin.

BACKGROUND: Anastrozole is a third-generation nonsteroidal aromatase inhibitor which is used in the treatment of breast cancers. Anastrozole has also been used in the treatment of dermatomyositis skin eruptions but its direct effects on skin have not been well documented. OBJECTIVE: To study the effects of anastrozole administration on neonatal rat skin. METHODS: Forty Sprague-Dawley female newborn rats were separated into two control groups and two experimental groups (n = 10). One day after birth the control group of newborn rats were given daily 0.02 ml saline subcutaneously for a period of 15 days. The first experimental group of rats were treated with 0.05 mg/100g/day anastrozole subcutaneously for 15 days whereas the second experimental group of rats were given 0.25 mg/100g/day anastrozole subcutaneously for 15 days. Histopathological assessments were made and compared with the control groups. RESULTS: Increased keratinization, strippling, hypertrophic epidermal cells and disorganization of the epidermal cells were observed in the first experimental group. In the second experimental group in addition to these pathologic findings acantholysis was observed. CONCLUSION: The administration of anastrazole in newborn rats showed considerable harmful effects.

Neurotological status in Behçet's disease and its ophthalmological correlates.

OBJECTIVE: To assess neurotological status and its ophthalmological correlates in Behçet's disease. STUDY DESIGN: A cross sectional study in which 17 patients with Behçet's disease and 13 healthy controls were included. PATIENTS AND METHODS: The patients were divided into two groups: those with and without end stage ocular involvement. Neurotological status was evaluated with audiological tests (pure tone and speech audiometry, tympanometry, short increment sensitivity index, tone decay, and evoked response audiometry), and Dix-Halpike positional testing. RESULTS: The positional test was normal. The audiological test results of the patients with and without end stage ocular involvement were not significantly different (p>0.05). A sloping audiogram with bilateral symmetric and mild sensorineural hearing loss was the main audiogram obtained. There was no relationship between the presence or absence of the end stage ocular involvement and the otological parameters studied excluding I-III interval on evoked response audiometry (p>0.05). CONCLUSION: Although the main underlying pathogenetic factor in Behçet's disease is the autoimmune vasculitis, the mechanisms involved in the pathogenesis of neurological and ocular damage may be different. Alternatively, the differential involvement of certain organ systems may not reflect the nature of the disease process itself, but rather the manner in which each organ responds to injury.

The significance of immunohistochemistry in the skin pathergy reaction of patients with Behçet's syndrome.

BACKGROUND: Behçet's syndrome is a chronic systemic immuno-inflammatory disorder affecting multiple organs with generalized vasculitis of arteries and veins. Although the aetiology is still unknown, endothelial dysfunction is one of the most prominent features in Behçet's syndrome. The skin pathergy reaction (SPR) is a non-specific hyperreactive lesion formation that is one of the major features and diagnostic criteria of the disease. It develops after 24-48 h at the site of the needle-prick, especially in the exacerbation period, and it is very similar to the erythematous papules or pustules that appear spontaneously in patients with Behçet's syndrome. Therefore, an investigation into the formation of the SPR lesion may contribute to the pathophysiology of skin lesions of this unique disorder. OBJECTIVE: To evaluate the immunological features of SPR formation by assessing the immunohistochemical staining of cell adhesion molecules and endothelial growth factor markers such as E-selectin, P-selectin and endoglin (CD 105). METHODS: Patients with Behçet's syndrome showing positive (n = 15) or negative (n = 10) SPR and 15 age- and sex-matched hospital-based healthy control subjects from a similar ethnic background were included in this study. Patients were divided into active and inactive stage by clinical findings and acute-phase reactant parameters including erythrocyte sedimentation rate (ESR) and neutrophil count. Punch biopsy specimens were obtained both from the lesion site on the forearms at 48 h and from normal skin approximately 5 cm adjacent to the SPR site. A biopsy was also obtained from the test application site in Behçet's syndrome patients with negative SPR and healthy volunteers. Biopsy specimens were then evaluated by immunohistochemical staining. RESULTS: Immunohistochemical examination demonstrated a mixed inflammatory cell infiltrate around the vessels and skin appendages that extended somewhat into the deep dermis. A positive segmental staining of E-selectin and P-selectin was noted in the endothelial cells of biopsies obtained from the patients with positive SPR. A positive segmental staining of CD 105 in the endothelial cells was also observed in the same group of patients. However, the immunostaining of the same markers was found to be negative in the biopsies obtained from normal skin of SPR-positive patients, SPR-negative patients and healthy control subjects. Both acute-phase reactant levels were significantly higher in the active stage than in inactive patients or healthy controls. CONCLUSION: Interaction of cellular adhesion molecules together with endothelial proliferation may play an important role in the formation of SPR lesions in patients with Behçet's syndrome. The involvement of the vascular endothelium in a large number of diseases including Behçet's syndrome supports the importance of vascular-specific adhesion molecules for their aetiopathogenesis.

Serum leptin concentration is increased in patients with Behçet's syndrome and is correlated with disease activity.

UNLABELLED: Background Behçet's syndrome is a systemic, relapsing immuno-inflammatory disease with a generalized vasculitis of the microvasculature endothelial dysfunction. Leptin, a recently discovered neuroendocrine hormone, is a metabolic peptide that appears to be involved. Serum proinflammatory cytokines upregulate leptin levels and leptin itself directly induces nitric oxide production from endothelial cells with its specific receptors. OBJECTIVES: To detect changes of serum leptin concentrations in patients with Behçet's syndrome compared with age- and sex-matched healthy volunteers by using enzyme-linked immunosorbent assay. We also investigated whether disease activity or the duration of Behçet's syndrome correlates with leptin concentration. METHODS: Thirty-five consecutive patients with Behçet's syndrome (41.2 +/- 8.4 years, 16 male, 19 female) and 20 age- and sex-matched healthy control subjects (40.4 +/- 10.91 years, nine male, 11 female) were included in this study. The body mass index (BMI) [weight (kg) height(-1) (m(2))] was calculated for subjects at study enrollment. We measured serum leptin with a leptin enzyme immunoassay kit, and acute-phase reactants, including erythrocyte sedimentation rate, alpha1-antitrypsin, alpha 2-macroglobulin and neutrophil count. The Mann-Whitney U-test was used for statistical analysis and P < 0.05 was considered significant. Values were expressed as mean +/- SD. RESULTS: The gender ratio, age and BMI were not substantially different among Behçet's patients and controls. The mean serum leptin concentrations in patients with Behçet's syndrome (16.8 +/- 7.49 ng mL(-1)) were significantly (P < 0.001) higher than in healthy control volunteers (7.5 +/- 2.77 ng mL(-1)). Active Behçet's patients had significantly (P = 0.001) higher leptin concentrations (20.5 +/- 7.99 ng mL(-1)) when compared with patients in inactive periods (12.8 +/- 4.43 ng mL(-1)). In addition, patients with longer disease duration (mean, 20.1 +/- 5.15 years) had also significantly (P = 0.013) higher leptin concentrations (20.2 +/- 8.52 ng mL(-1)) than those with shorter disease duration (13.4 +/- 4.52 ng mL(-1)) (mean, 7.4 +/- 3.29 years). All acute-phase reaction parameters were found to be significantly (for each, P < 0.01) increased in active disease. CONCLUSIONS: Leptin may have a role in modulating endothelial function and may be involved in mechanisms for vessel endothelium repair, during an exacerbation as well as in chronic disease.

Citalopram-induced photopigmentation.

Citalopram is one of the newer and most potent selective serotonin re-uptake inhibitor (SSRI) drugs. It has a well-established antidepressive action with a favorable adverse event profile. We present a fifty-year-old woman with diffuse photopigmentation who had been diagnosed as suffering from depression. The patient was given citalopram (40 mg/day) for her psychiatric condition and diffuse photopigmentation was noted thereafter. To our knowledge, such an adverse event has not been reported previously.

The prevalence of hepatitis C virus infection in patients with lichen planus in Gaziantep region of Turkey.

The purpose of this case-control study was to investigate the association between lichen planus (LP) and hepatitis C virus (HCV) infection in Gaziantep region of Turkey. Seventy-three patients with LP and a control group of patients (n: 73) with a dermatological disorder other than LP were detected for HCV infection using a third generation enzyme-linked immunosorbent assay (ELISA). A serological positivity for HCV was found in five of LP patients (6.84%), whereas it was positive for only one patient of the control group (1.36%). A statistically significant difference was found between LP and control groups (p < 0.05). We conclude that the coexistence of the two diseases is probably more than coincidental.