The utility of immunoglobulin A/complement 3 and immunoglobulin G/immunoglobulin M ratios in the assessment of disease activation in patients with Behçet disease.

OBJECTIVE: Pathogenesis of Behçet disease (BD) has not yet been clearly revealed and there is no ideal test for the estimation of disease activation at present. This study aimed to assess the efficiencies of IgG/IgM and IgA/C3 ratios in determining activation of BD. METHOD: This retrospective cohort study consisted of 140 patients with BD. Patients were divided into two groups: (1) active BD (n = 89) and (2) inactive BD (n = 51) and were compared in terms of demographic features, clinical characteristics and laboratory test results. IgA/C3 and IgG/IgM ratios were compared according to organ system involvement; receiver operating characteristic (ROC) curve analysis was performed in order to assess the performance of IgA/C3 and IgG/IgM ratios in determining patient disease status. RESULTS: Significantly higher levels of erythrocyte sedimentation rate, C-reactive protein, IgA, G, C4, IgA/C3, IgG/IgM ratios (p = .007 for IgA and p < .001 for others) and significantly lower levels of IgM and C3 were observed in patients with active BD (p < .001). The IgG/IgM ratio was significantly higher in patients with vascular involvement (p = .017) and the IgA/C3 ratio was significantly higher in patients with arthritis (p = .007). Cut-off values of 0.019 (70.8% sensitivity, 62% specificity) and 7.08 (84.3% sensitivity, 80% specificity) were determined for IgA/C3 and IgG/IgM ratios, respectively. CONCLUSION: IgA/C3 and IgG/IgM ratios may be used as additional parameters for the assessment of BD status.

C-type lectin domain family 12, member A: A common denominator in Behçet's syndrome and acute gouty arthritis.

C-type lectin domain family 12, member A (CLEC12A) is a C-type lectin-like pattern recognition receptor capable of recognizing monosodium urate crystals. Monosodium urate crystals, the causative agents of gout are also among the danger-associated molecular patterns reflecting cellular injury/cell death. In response to monosodium urate crystals, CLEC12A effectively inhibits granulocyte and monocyte/macrophage functions and hence acts as a negative regulator of inflammation. Behçet's syndrome and gout are autoinflammatory disorders sharing certain pathological (neutrophilic inflammation), clinical (exaggerated response to monosodium urate crystals) and therapeutic (colchicine) features. We propose the hypothesis that decreased expression of CLEC12A is a common denominator in the hyperinflammatory responses observed in Behçet's syndrome and gout. Major lines of evidence supporting this hypothesis are: (1) Downregulation/deficiency of CLEC12A is associated with hyperinflammatory responses. (2) CLEC12A polymorphisms with functional and clinical implications have been documented in other inflammatory diseases. (3) Colchicine, a fundamental therapeutic agent used both in Behçet's syndrome and gout is shown to oppose the downregulation of CLEC12A. (4) Behçet's syndrome and gout are characterized by a hyperinflammatory response to monosodium urate crystals and other than gout, Behçet's syndrome is the only inflammatory condition exhibiting this exaggerated response. (5) Genomewide linkage and association studies of Behçet's syndrome collectively point to 12p12-13, the chromosomal region harboring CLEC12A. (6) Patients with severe forms of Behçet's syndrome underexpress CLEC12A with respect to patients with mild forms of the disease. If supported by well-designed, rigorous experiments, the forementioned hypothesis pertinent to CLEC12A will carry important implications for therapy, designing experimental models, and uncovering immunopathogenic mechanisms in Behçet's syndrome and gout.

Isotretinoin treatment in nodulocystic acne with and without polycystic ovary syndrome: efficacy and determinants of relapse.

BACKGROUND: Isotretinoin is the most efficacious long-lasting treatment for acne; however, some factors, including polycystic ovary syndrome (PCOS), patient age, family history, and type and number of acne lesions, may lead to treatment resistance or relapse following treatment. The aim of this study was to compare the efficacy and permanence of systemic isotretinoin (SI) in nodulocystic acne patients with and without PCOS and to evaluate the factors associated with relapse during the first and second post-treatment years. METHODS AND MATERIALS: The study included 96 female patients with nodulocystic acne. SI 0.5-1 mg/kg/dl was given, with a total cumulative dose of 120-150 mg/kg. Response to treatment and relapse during the first and second post-treatment years were evaluated. RESULTS: In all, the 50 non-PCOS and 46 PCOS acne patients were similar. SI was similarly efficacious in both groups. In total, eight patients relapsed during the first post-treatment year, versus 16 during the second. Relapse during the first year was associated with the number of nodules at the start of treatment and the number of papulopustular lesions at the end of treatment, whereas PCOS, patient age, and the number of nodules at the start of treatment were associated relapse during the second year. CONCLUSION: Regardless of its association with PCOS, SI was effective in the treatment of nodulocystic acne. The factors associated with relapse during the 1(st) and 2(nd) post-treatment years differed, except for the number of nodules at the start of treatment.

Does Behcet's disease associate with neuropathic pain syndrome and impaired well-being?

Previously peripheral neuropathy signs have been reported in inflammatory chronic diseases but the presence of neuropathic pain syndrome (NPS) in Behcet's disease (BD) is unclear. The aim of this study was to investigate the association of BD with NPS and impaired quality of life and sleep quality. A total of 111 patients diagnosed as BD and 52 healthy controls were included. Pain severity was assessed by visual analogue scale (VAS) in rest and during activity. The NPS was diagnosed according to the Leeds Assessment of Neuropathic Symptoms and Signs (LANSS) index. The well-being and sleep disturbances of the groups were evaluated with Psychological General Well-Being (PGWB) Scale and Pittsburg Sleep Quality Index (PSQI). Although there were no one with NPS in healthy controls, the proportion of NPS in patients with BD was 19.8 % (p = 0.001). The VAS scores both in activity and at rest were higher in BD (p < 0.001). There was statistically significant decrease in total PGWB score in BD patients compared to healthy controls (p < 0.001). And significant increase in LANSS score was observed in patients with BD compared to healthy controls (p = 0.000). The total LANSS scores showed significant positive correlation with PSQI scores (r = 0.322) and negative correlation with total PGWB scores (r = -0.672) in patients with BD. We observed a positive correlation between LANSS and VAS (rest and activity) scores (r = 0.44, r = 0.42 respectively). The NPS seems to be associated with BD which should be taken into consideration in patients with neuropathic signs. The quality of life (QoL) and quality of sleep of the patients with BD were found to be impaired and this may be due to the presence of NPS.

Association of hypermobility and ingrown nails.

Ingrown nail (onychocryptosis) is a common condition with severe pain and various associated morbidities. Although some underlying factors are identified, its etiology remains largely unknown. Generalized joint hypermobility (GJH) is a common entity with clinical features that might prone affected individuals to ingrown nails. Herein, we investigated the incidence of GJH in patients with ingrown nails to determine possible association between hypermobility and ingrown nail formation. Patients 16-50 years of age who were undergoing treatment for ingrown nails at the dermatology clinic were consecutively enrolled into the study. Patients with known rheumatic diseases or orthopedic foot disorders were excluded. All patients were in a pain-free period at the time of examination. The control group was comprised of age- and sex-matched healthy subjects without a history of ingrown nail. Assessment of GJH was made according to Beighton criteria. Local hypermobility was evaluated by measurement of range of motion using a goniometer. Thirty-nine patients (male/female, 17/22, mean age 31.9 ± 11.3 years) and 32 healthy subjects (male/female 12/20, mean age 31.7 ± 10.4 years) were included. Patients with ingrown toe nails were more likely to have GJH compared to healthy subjects (35.9 vs. 9.4 %, p = 0.009). Toes with ingrown nails had significantly smaller maximum dorsiflexion angles (p < 0.001) compared to toes of healthy subjects. Ingrown nail formation may be associated with GJH. However, when examined locally, there is a limited range of motion in the affected toe rather than hypermobility, which could be due to the degenerative process facilitated by the hypermobility.

Behçet disease in children.

PURPOSE: To investigate the incidence and clinical characteristics of Behçet disease in children. METHODS: The authors retrospectively reviewed the charts of 3382 patients with Behçet disease from October 1986 to December 2005 at Ankara University Medical School Behçet Unit and/or Atmaca private clinic. RESULTS: 110 children were diagnosed with Behçet disease. The 110 children represented 3.3% of the total number of Behçet patients that were reviewed. 62.7% were girls and 37.3% were boys. The mean age at initial manifestation was 11.63 ± 3.46 years and at diagnosis was 14.15 ± 2.13 years. Ocular involvement was found in 30.9% of the children. Of these, 61.8% were bilateral and 38.2% were unilateral. Anterior uveitis was found in 18 eyes (32.8%), posterior uveitis in 24 eyes (43.6%), and panuveitis in 13 eyes (23.6%). CONCLUSION: Behçet disease should be considered in the differential diagnosis of childhood uveitis, particularly in endemic areas. The collaboration of pediatricians, rheumatologists, dermatologists, ophthalmologists, and other specialists when necessary is required in the diagnosis and management of children with Behçet disease.

Evaluation of clinical findings according to sex in 2313 Turkish patients with Behçet's disease.

BACKGROUND: Behçet's disease is a multisystem disease featuring mucocutaneous, ocular, articular, vascular, intestinal, urogenital, and neurologic involvement and occurs with a high prevalence in the Mediterranean including Turkey. Higher incidence of severe clinical course and systemic involvement is observed in male patients. OBJECTIVE: To determine the influence of sex on the clinical course of Behçet' s disease. METHODS: We retrospectively evaluated the clinical findings of 2313 Behçet patients followed up at the multidisciplinary Behçet's Disease Center at Ankara University. RESULTS: The male/female patient ratio was 1.03. Oral aphthae was seen in all patients. In male Behçet patients, the prevalence of mucocutaneous lesions and systemic manifestations was as follows: 85.6% genital aphthae, 45.5% erythema nodosum, 59.5% papulopustular lesions, 17.5% thrombophlebitis, 38.1% ocular involvement, 11.3% articular involvement, 11.7% vascular involvement, 3.3% neurologic involvement, 1.4% gastrointestinal involvement, and 1.8% pulmonary involvement. In female Behçet patients, the prevalence of manifestations were as follows: 91% genital aphthae, 49.8% erythema nodosum, 48.3% papulopustular lesions, 3.5% thrombophlebitis, 19.8% ocular involvement, 11.8% articular involvement, 2.1% vascular involvement, 1.3% neurologic involvement, 1.4% gastrointestinal involvement, and 0.03% pulmonary involvement. CONCLUSIONS: Only genital aphthae and erythema nodosum were more frequent in females. On the other hand papulopustular eruptions, thrombophlebitis, ocular, neurologic, pulmonary and vascular involvement were more frequent in males. While female patients had the best prognosis, male patients had a worse overall prognosis than females.

Upper gastrointestinal findings in oral lichen planus.

BACKGROUND/AIMS: Lichen planus is an inflammatory disease of the skin and mucous membranes. Oral mucosa is known to be frequently affected by the disease, but it has also been observed that gastrointestinal mucosas are sometimes involved. METHODS: In this study, the upper gastrointestinal tract was investigated endoscopically and histopathologically in 20 patients with oral lichen planus. RESULTS: Endoscopy determined antral gastritis in seven patients, esophagitis in four, bulbitis in three, chronic duodenal ulcer in one and esophageal sphincter dysfunction in one patient. Lichen planus-like changes on the esophageal mucosa at histopathological examination were found in one patient. Findings in the other patients were as follows: chronic atrophic gastritis (nine), helicobacter pylori infection (nine), esophagitis (two), bulbitis (two) and erosive gastritis (one). CONCLUSION: Patients with Lichen planus should be evaluated for possible gastro-intestinal involvement with endoscop.