A case of sporadic Bazex-Dupré-Christol syndrome presenting with scarring folliculitis of the scalp.
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Biomedical subjects
Publications and source records attributed to F G Bechara.
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BACKGROUND: Although the human beta-defensins hBDs today seem to have diverse functional activities in innate antimicrobial immunity, a few reports also indicated an altered expression of these antimicrobial peptides (AMPs) in tissues of cancers such as oral squamous cell carcinoma. The present work was aimed on the study of hBD gene expression in basal cell carcinoma (BCC) which is the most common cancer in humans. METHODS: Twenty-two non-ulcerated BCCs (12 nodular type, 10 superficial type) have been analysed for the presence of hBD (1-3) mRNA by quantitative real-time RT-PCR. As controls, non-lesional skin specimens of BCC patients as well as samples of healthy subjects were assessed by RT-PCR. RESULTS: hBD-1 levels in healthy controls and non-lesional skin of BCC patients were significantly (P < 0.05) higher than the levels observed in tumour tissue. Moreover, BCCs showed significantly (P < 0.05) increased mRNA expression of hBD-2 as compared to controls. There was no significant (P > 0.05) difference between lesional mRNA levels for hBD-3 and those levels observed in controls. The mRNA expression of hBDs (1-3) found in nodular and superficial BCCs did not significantly (P > 0.05) differ. CONCLUSION: The gene expression patterns of hBD-1 and hBD-2 are for the first time shown to be significantly altered in non-ulcerated BCCs as compared to intra-individual and inter-individual controls, respectively. The present findings may indicate that beside the antimicrobial activity of AMPs, hBDs may also play a role in the pathogenesis of BCC. However, functional and immunohistological studies investigating hBDs in patients with BCC are needed to confirm our data.
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BACKGROUND/AIMS: It has recently been proposed that the refractive index (RI) measured by means of optical coherence tomography (OCT) may be a valid measure for hydration of skin. In this pilot study, using OCT in vivo, we aimed to investigate the interday variability of RI measurements and acute changes of RI following the application of a moisturizer. METHODS: Twenty healthy Caucasian volunteers were investigated on their forearms using a commercially available OCT system (SkinDex 300, ISIS optronics GmbH, Mannheim, Germany) fitted with an integrated algorithm for the evaluation of the RI. The interday repeatability of the OCT method was determined performing symmetrical measurements on both forearms on day 1, 5, 9, and 13. In order to investigate the acute effect of a moisturizer on RI, OCT assessments were performed before and 10 min after the application of an aqueous lotion with a lipophilic phase. As a control, the contralateral site was investigated in the same way, except for the use of distilled water instead of the lotion. RESULTS: Assessments of interday variability revealed insignificant (P>0.05) variances between the four measurement times as expressed in very small repeatability coefficients (right arm: 0.039; left arm 0.053) and small coefficients of variance (right arm: 1.02%; left arm: 1.38%). With regard to the RIs measured over time, we could not observe significant (P>0.05) differences between the two symmetrical anatomic sites (mean+/-SD of RI: 1.3893+/-0.0142 (right arm); 1.3875+/-0.0192 (left arm)). The acute effect of the moisturizer was indicated by a significant decrease of the RI 5 min after the application of the lotion (1.399+/-0.01 vs. 1.387+/-0.02; difference between means: 0.012; P=0.033; 95% confidence interval: 0.001-0.0023). However the control site treated with distilled water did not show significant differences between the two measurement times (1.387+/-0.013 vs. 1.391+/-0.023; difference between means: -0.004; P=0.57; 95% confidence interval: -0.019-0.011). CONCLUSIONS: In this pilot study, we have demonstrated that RI evaluation via OCT is a promising technique that may be used for the assessment of skin hydration in vivo. However, the direct comparison of OCT with standard methods, ideally such as nuclear magnetic resonance spectroscopy, is necessary.
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BACKGROUND: Optical coherence tomography (OCT) is a new and promising diagnostic technique for investigation of skin tumours. We describe a method that makes evaluation and definition of specific morphologic structures of skin tumours via OCT more accurate. MATERIALS AND METHOD: We investigated three patients with basal cell carcinoma and three patients with melanocytic nevi. Three-dimensional (3D) images were obtained from these skin tumours via OCT according to previously applied marks, which were tattooed with special histological marking dye after excision of the tumours. Corresponding to these marks, we investigated serial histological sections (haematoxylin&eosin staining). RESULTS: We could prove similar morphological structures both in OCT and histology. Due to tissue deformation, the compared measurements of structures like cell nests or epidermal thickness were slightly deviated. However, by this method we could prove similar tissue formations in OCT and histology. CONCLUSIONS: Due to the deformation by histological processing and slightly different sectioning levels, the comparison of histological pictures and OCT images seems difficult. Nevertheless, in two cases it was possible to demonstrate the same morphological structures with OCT imaging and histological investigation. Our method could play an important role for further evaluation of OCT images. We estimate better evaluation of OCT imaging using a 3D reconstruction method.
BACKGROUND: Cutaneous microcirculation shows a distinctive spatiotemporal inhomogeneity. Therefore provocation tests are necessary to receive significant and reproducible data. The present study investigated the effect of localized cutaneous vascular alteration, like in psoriasis vulgaris, on different parameters of reactive hyperemia (peak capillary blood cell velocity [pCBV], postocclusive reactive hyperemia [PRH%], and time to peak capillary blood cell velocity [tpCBV]). MATERIAL AND METHODS: Psoriatic plaques and normal skin on the contralateral side of 20 patients with psoriasis vulgaris were examined by Laser Doppler Anemometry. Capillary blood cell velocity was measured before suprasystolic occlusion and during postocclusive hyperemia. RESULTS: Compared to normal skin psoriatic plaques showed a significant increase of peak capillary blood cell velocity (pCBV, normal skin: 0.89+/-0.23 mm/s, psoriatic plaque: 2.03+/-0.94 mm/s), resting capillary blood cell velocity (rCBV, normal skin: 0.43+/-0.12 mm/s psoriatic plaque: 0.72+/-0.20 mm/s) and postocclusive reactive hyperemia (PRH%, normal skin: 107%, psoriatic plaque: 180%). The time to peak capillary blood cell velocity (tpCBV) during reactive hyperemia did not change significantly. CONCLUSION: In reactive hyperemia, changing of pCBV and PRH% combined with unaltered tCBV indicate a dysfunction of cutaneous microcirculation. In contrast acute closure of upper and lower arterial extremity show a changed tCBV with stable pCBV and PRH%.
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BACKGROUND: Linear scleroderma "en coup de sabre" (LSCS) usually affects one side of the face and head in the frontoparietal area with band-like indurated skin lesions. The disease may be associated with facial hemiatrophy. Various ophthalmological and neurological abnormalities have been observed in patients with LSCS. We describe an unusual case of LSC. CASE PRESENTATION: A 23 year old woman presented bilateral LSCS and facial atrophy. The patient had epileptic seizures as well as oculomotor and facial nerve palsy on the left side which also had pronounced skin involvement. Clinical features of different stages of the disease are presented. CONCLUSIONS: The findings of the presented patient with bilateral LSCS and facial atrophy provide further evidence for a neurological etiology of the disease and may also indicate that classic progressive facial hemiatrophy (Parry-Romberg syndrome) and LSCS actually represent different spectra of the same disease.
BACKGROUND AND OBJECTIVE: Mummies have an important place in the study of archaeology and paleopathology because they are so well preserved. For the first time skin samples of six 2300-1600 year old bog bodies from North Germany were examined by histology, transmission electron microscopy and immunohistology. METHODS: For histology the samples were stained with H&E and van Gieson elastic stain. Fixation and embedding in epoxy followed for the transmission electron microscopy. Specific antibodies directed to type IV collagen and S-100 were used. RESULTS: Histologically it was possible to observe collagen bundles in the dermis, with a density similar to recently stained samples. Epidermis was not preserved. The electron microscopy showed collagen fibrils with a diameter of 45-110 nm and the characteristic axial periodicity. Throughout the dermis, a number of spores of bacteria with a diameter of 0.83 +/- 0.051 micron and an electron dense core were found. No activity against the used antibodies could be detected. CONCLUSIONS: Histology and electron microscopy demonstrate the excellent conservation of the dermal collagen in the bog. In contrast to ice mummies like "Otzi" and mummies from Egypt, no cellular elements could be found in the skin of bog bodies.