BACKGROUND: Neurofibroma, the hallmark of neurofibromatosis, is a cutaneous or subcutaneous lesion, with a variable clinical presentation. Histologically, neurofibroma consists of proliferation of nerve derived cellular elements, together with an abundant, collagenous extracellular matrix. Specifically, neurofibroma has been shown to contain 30-50% collagen in its matrix. Objective 5. METHODS: We examined the expression of extracellular matrix genes (collagen, fibronectin, laminin), TGF-b mRNA and Ha-ras oncogene mRNA by using Northern and slot-blot hybridization and immunoperoxidase stains. Result: In Northern blot analysis, Ha-ras and TGF-b genes revealed respectively, 8.8kb and 2. 5kb sized mRNA transcripts in neurofibroma. These parameters were normal in the control. The expression of these genes were 1.9, 2.0 fold increased in neurofibroma. In slot-blot analysis, expression of type I collagen showed fibronectin genes to be 2,401+210, 540+43, respectively, in neurofibroma. So there were 3.7 fold, 2.1 fold, differences respectively, compared to the normal control. However, there were no significant changes of type IV collagen and laminin Bl mRNA levels between neurofibroma and normal skin tissues. Irnmunoperoxidase staining by rnonoclonal anti type IV collagen antibody in neurofibroma showed type IV collagen to be diffusely and weakly stained in tissue. On staining by monoclonal anti-laminin antibody, laminin was stained in a matrix and around vessels. CONCLUSION: The increased expression of extracellular matrix genes may suggest that there is a subpopulation of fibroic cells in neurofibroma which are stimulated by TGF-b. Ha-ras genes which might have accumulated with the differentiation of neural tissue may be related to the pathogenesis of neurofibroma tissue formation. Further studies are needed to determine whether the other factors are related to the pathogenesis of neurofibroma.
Blotting, Northern ; Collagen ; Collagen Type I ; Collagen Type IV ; Coloring Agents ; Extracellular Matrix* ; Fibronectins ; Genes, ras* ; Laminin ; Neurofibroma ; Neurofibromatoses* ; RNA, Messenger ; Skin

The Melkersson-Rosenthal syndrome consists of the associated of indings of facial nerve paralysis, facial swelling and scrotal tongue. We report a case of MRS where a 20 year old female had diffuse swelling on her face and lips for 10 years. This was also accompaiined with difficulty of facial expression and fissured tongue. The improved with intralesional injection: of triamcinolon acetonide 40mg for 3 consecutive weeks and 2 every other week.
Facial Expression ; Facial Nerve ; Facial Paralysis ; Female ; Humans ; Injections, Intralesional ; Lip ; Melkersson-Rosenthal Syndrome ; Tongue, Fissured ; Young Adult

No abstract available.
Lichen Planus, Oral* ; Mouth* ; Tacrolimus*

Lupus vulgaris is most prevalent on exposed parts, especially the face but can also develop on exetremities. Lupus vulgaris originates from tuberculosis elsewhere in the body by hematogenous, lymphatic, or contiguous spread. A 19-year-old male patient came to our department. The patient had had many recurrent oozing and verrucous plaques and crusts on the left foot for one year. A skin biopsy from the lesion on the left dorsum of the foot showed scattered well defined granulomas consisting of the epithelioid cell clusters with Langerhans and foreign body type giant cells in the mid dermis. Caseation necrosis was slight. There were no bacilli on AFB staining. The multi test CMI for tuberculin was highly positive. A chest X-ray did not show any abnormal findings. The presence of Mycobacterium tuberculosis DNA was demonstrated by polymerase chain reactions (PCR) for detection of mycobacterial DNA from a routinely prepared paraffin-embedded skin specimen. Herein we report a very atypical case of lupus vulgaris confirmed by PCR.
Biopsy ; Dermis ; DNA ; Epithelioid Cells ; Foot ; Giant Cells, Foreign-Body ; Granuloma ; Humans ; Lupus Vulgaris* ; Male ; Mycobacterium tuberculosis ; Necrosis ; Polymerase Chain Reaction ; Skin* ; Thorax ; Transplants* ; Tuberculin ; Tuberculosis ; Young Adult

A 26-year-old man with a one-year history of asthma and sinusitis presented with bilateral pleural effusions, patch basilar infiltrates on a chest x-ray and a pericardial effusion on an echocardiogram. The peripheral blood showed marked eosinophilia. An obstructive pattern was also observed during the pulmonary fuction test, which was responsive to bronchodilator inhalation. Nerve conduction studies showed right sural neuropathy. Thoracentesis yielded an acidotic exudative effusion with low glucose, low C3 and eosinophilia. An open lung biopsy revealed an eosinophilic interstitial pneumonitis associated with a necrotizing eosinophilic vasculitis, and granulomatous inflammation foci. In the literature, pleural effusions were reported in 29 percent of Churg-Strauss patients, but the number of effusions was low and their characteristics have not been well described. This report describes the characteristic findings of pleural fluid and its histologic features in a case of classical Churg-Strauss syndrome.
Adult ; Asthma ; Biopsy ; Churg-Strauss Syndrome* ; Eosinophilia ; Eosinophils ; Glucose ; Humans ; Inflammation ; Inhalation ; Lung ; Lung Diseases, Interstitial ; Neural Conduction ; Pericardial Effusion ; Pleural Effusion* ; Sinusitis ; Thorax ; Vasculitis

No abstract available.
Epidermal Cyst*

No abstract available.
Trichothiodystrophy Syndromes*

PURPOSE: We investigated reproducibility of the quantification of left ventricular volume and ejection fraction, and grading of myocardial wall motion and systolic thickening when we used gated myocardial SPECT and Cedars quantification software. MATERIALS AND METHODS: We performed gated myocardial SPECT in 33 consecutive patients twice in the same position after Tc-99m-MIBI SPECT. We used 16 frames per cycle for the gatingof sequential Tc-99m-MTBI SPECT. After reconstruction, we used Cedars quantitative gated SPECT and calculated ventricular volume and ejection fraction (EF), Wall motion was graded using 5 point score. Wall thickening was graded using 4 point score. Coefficient of variation for re-examination of volume and fraction were calculated. Kappa values (k-value) for assessing reproducibility of wall motion or wall thickening were calculated. RESULTS: Enddiastolic volumes (EDV) ranged from 58 mi to 248 ml (122 ml +/- 42 ml), endsystolic volumes (ESV) from 20 mi to 174 mi (65 ml +1- 39 ml), and EF from 20% to 68% (51% +/- 14%). Geometric mean of standard deviations of 33 patients was 5.0 ml for EDV, 3.9 ml for ESV and 1.9% for EF. Their average differences were not different from zero (p>0.05). k-value for wall motion using 2 consecutive images was 0.76 (confidence interval: 0.71-0.81). k-value was 0.87 (confidence interval:0.83-0.90) for assessment of wall thickening. CONCLUSION: We concluded that quantification of functional indices, assessment of wall motion and wall thickening using gated Tc-99m-MIBI SPECT was reproducible and we could use this method for the evaluation of short-acting drug effect.
Heart ; Humans ; Tomography, Emission-Computed, Single-Photon*

No abstract available.
Brain Injuries* ; Brain* ; Disability Evaluation* ; Humans ; Perfusion* ; Technetium Tc 99m Exametazime* ; Tomography, Emission-Computed, Single-Photon*