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The aim of this study was to evaluate a possible relation between clinical disease activity determined by a scale validated and most widely used acute phase response parameters in patients with Behcet's Disease (BD). One hundred patients with BD were included in this study. The demographic properties including age, sex, and disease durations were recorded. BD Current Activity Form (BDCAF), which scores the history of clinical features that have been present during the four weeks prior to the day of assessment, was used for the evaluation of disease activity. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels were evaluated as acute phase response. The independent samples t test and Pearson's correlation test were performed for the statistical analysis. There were 60 male and 40 female patients with an age range of 19-51 years. The disease durations of the patients were between 12 and 240 months. In the comparison of the laboratory parameters of the patients with or without the components of BDCAF, we found significantly higher levels of ESR and CRP in patients with erythema and arthralgia-arthritis versus without these involvements. There were significant positive correlations between ESR-CRP levels and patient's perception of disease activity (P = 0.012 and P < 0.05, respectively), clinician's overall perception of disease activity (P < 0.005), and total BDCAF score (P = 0.008 and P < 0.000, respectively). In conclusion, the presence of a newly-developed erythema nodosum, superficial thrombophlebitis, or joint involvement may be associated with higher levels of ESR and CRP. Also, correlations between these indices and general disease activity scores may contribute the overall disease activity perception in BD., (© 2013 The International Society of Dermatology.)

Background: Lipoid proteinosis (LP) is a very rare genodermatosis. The literature on LP consists of case reports only. As we have observed 14 LP patients belonging to nine different families in the last 15 years in our practice, we decided to review all reported Turkish LP patients in this 15-year period, and noted 37 diagnosed cases. The reasons for this relatively large number of cases, the clinical features of the patients, and the associations of LP with other clinical conditions are described in this article., Methods: Fourteen LP patients followed in our university clinic in Istanbul were scrutinized with regard to their demographic and clinical features. Diagnoses were established using clinical features, with histopathologic confirmation in 13 cases., Results: All but one of the patients had a history of consanguinity, or at least a marriage of parents from the same village. Typical cutaneous signs of LP and hoarseness of the voice were observed in all patients. Two patients of the same pedigree had insulin-dependent diabetes mellitus (IDDM), two patients from two different pedigrees had short stature, one patient had multinodular toxic goiter, and one patient had celiac disease., Conclusion: LP is not rare in Turkey as consanguineous marriage is still a social problem, especially in some rural areas. The disease is not limited to a particular geographic region in Turkey. Short stature was observed in two cases from two different families, an association not reported previously; the association of LP with IDDM in one pedigree was thought to be coincidental.