PLATELET FUNCTION IN BEHÇET'S DISEASE

The coagulopathy observed in Behçet's disease has defied explanation. No pathology in concentration or function of coagulation parameters has been demonstrated. We have studied platelet adhesion function in 13 patients with established Behçet's disease, 5 with venous thrombosis and 8 with no involvement of veins, as well as in 30 age and sex matched healthy controls. Platelets derived from 10 of the 13 Behçet's patients exhibited significantly increased adhesion as compared to the controls (p<0.01). The platelets of 11 of the 13 tested patients showed significantly increased aggregation in response to adenosine diphosphate (ADP) and epinephrine incomparison to those from controls (p<0.01). However, no correlation between disturbed platelet function and the clinical occurrence of the thrombotic phenomenon was noted. It is suggested that the thrombotic tendency may be related to platelet abnormality while the generation of the actual process is dependent on other factors which await elucidation.     

ANTIACTIVATOR IN PLASMA OF PATIENTS WITH BEHÇET'S DISEASE

In the present study, it was confirmed for the first time that the plasminogen activator activity in the euglobulin fraction could be determined by the Testzym method using chromozyme S-2251. Antiactivators in plasma of patients with Behçet's disease, who showed decreased blood fibrinolytic activity, could be precipitated together with the activator in the euglobulin fraction. It was confirmed that the antiactivator was inhibited by dextran sulfate.     

BEHÇET'S DISEASE LEUCOCYTE INHIBITORY FACTOR (LIF) IN RESPONSE TO SKIN EXTRACT

This is a study of leucocyte inhibitory factor (LIF) in patients with Behçet's disease using an extract from a reactive (pathergic) lesion as an antigen and, as a control, an extract from normal skin of a normal subject. The purpose is to search for the possible existence of an antigen in the skin that may explain the development of this reactive lesion following a trivial trauma. The LIF was tested in 9 patients with the disease; in 7 both during remission and exacerbation of symptoms, and in 9 healthy matched controls. The results failed to indicate any existence of an antigen in the skin of the tested patients.     

LYMPHOCYTE TRANSFORMATION USING STREPTOCOCCAL ANTIGENS AND SERUM ANTISTREPTOKINASE TITERS IN BEHÇET DISEASE

The etiology of Behçet disease is still unknown. Some investigators have suggested that streptococci may play a role in the pathogenesis of Behçet disease. High antistreptolysin-O (ASLO) and antistreptokinase (ASK) titers were not detected in out-patients with Behçet disease. In our studies a considerably high percentage of blast cells was observed in lymphocyte transformation tests using streptolysin-O antigen, streptokinase-streptodornase (Varidase), Streptococcus nonhemolyticus, Streptococcus viridans, and Streptococcus pyogenes as the antigens in the cases of Behçet disease. Serum ASLO and ASK titers did not indicate significant relations among these results. The percentage of blast cells in the studies was not high in any case of Behçet disease, but it seemed that a cell-mediated immune reaction to streptococci might play a role in the pathogenesis of the disease.     

JUVENILE BEHÇET'S DISEASE AMONG 1784 TURKISH BEHÇET'S PATIENTS

Background. Behçet's disease is a chronic, relapsing disease, about which information on its clinical course in juveniles is only available from small groups of patients. Materials and Methods. Patients suffering from Behçet's disease who had their first lesion at or before the age of 16 were evaluated in terms of: age at onset, mucocutaneous signs, and findings related to systemic involvement. Ninety-five patients, evaluated as having juvenile Behçet's disease (JBD), were detected among 1784 Turkish Behçet's patients. The mean age of these 95 patients (51 boys or men, 44 girls or women) who had JBD was 26.8 ± 7.1 years. Results. The difference between sexes in terms of age at onset, development period of second lesions, and systemic involvement was not found to be significant in JBD. Patients were divided into two groups, one showing severe disease (N = 27) and the other mild disease (N = 68). There was no significant difference between the two groups with respect to age, age at onset, and sex distribution. The interval between the development of the first and second lesions was shorter in the patient group with severe disease (P < 0.001) and the development of second lesion was most frequently seen in the first 5 years (P < 0.05). Systemic involvement developed also in a shorter time in the group with the severe disease (P < 0.01) and was most frequently encountered during the first 5 years (P < 0.05). Conversely, patients with the mild disease developed systemic involvement more frequently after 6 years or later. Conclusions. Severe Behçet's disease in children and juveniles shows no age or sex predilection, but leads to an earlier recurrence and more severe systemic signs than the mild form.     

BEHÇET'S DISEASE: CLINICAL STUDY OF JORDANIAN PATIENTS

Background. Behçet's disease is a chronic, recurrent, inflammatory disorder characterized by the triad of oral and genital ulcers and ocular lesions. The etiology is unknown. This is the first report giving details of the clinical manifestations of Behçet's disease in Jordanian patients. Methods. Twenty patients with Behçet's disease were studied to determine the clinical pattern in the North of Jordan. The patients were seen in Princess Basma Teaching Hospital in North Jordan. They presented to various clinics and underwent full clinical examination. Data for each patient on all features of Behçet's disease were recorded on a standard form. A comparison was made between Behçet's disease in Jordan and other countries in the region. Results. Of the 20 patients, 14 were men and six women, giving a ratio of 2.3:1. Their ages ranged from 14 to 58 years. All had mouth ulcers, 65% genital ulcers, 65% ocular involvement, 55% joint involvement, 35% skin lesions, 20% vascular lesions, and 5% gastrointestinal involvement. The oral ulcers were the first manifestation of the disease process in 70% of our patients. Skin lesions, genital ulcers, and involvement of the central nervous system and the pulmonary system were less frequent in our study. Conclusions. Although it is difficult to obtain figures as to the incidence and prevalence of Behçet's disease in Jordan, the clinical manifestations, with a few exceptions, are similar to those in other countries in the region.     

CIRCULATING IMMUNE COMPLEXES IN VARIOUS FORMS OF BEHÇET'S DISEASE

Background. The etiology of Behget's disease (BD) is uncertain but there is strong evidence that the immune system is implicated in its pathogenesis. Methods. We assessed circulating immune complexes (CIC) in peripheral blood of 34 patients with BD, forming eight clinical groups, using a laser nephelometer to obtain more insight in the pathogenesis of different clinical forms of bd. Twenty healthy controls and eight patients with recurrent oral ulcerations were also included in the study. Results. Levels of CIC were significantly higher in patients (1.83 ± 0.93 μg/mL) than in controls (0.84 ± 0.51 μg/mL; P < 0.001). High titers were found in the groups of patients with erythema nodosum (3.14 ± 0.44 μg/mL), neurologic manifestations (2.9 ± 0.58 μg/mL), and ocular manifestations (2.34 ± 0.93 μg/mL). Compared to patients with recurrent oral ulcerations (1.91 ± 0.77 μg/mL), the mean value of CIC in patients with BD did not differ significantly, but the groups of patients having erythema nodosum, positive pathergy, and neurologic manifestations had significantly higher levels (P < 0.05) and the group of patients at the mild end of the spectrum (group 8) had a significantly lower level (1.09 ± 0.41 μg/mL) (P < 0.05). Only the groups having erythema nodosum, positive pathergy, and neurologic manifestations had significantly higher levels of CIC when compared to other groups lacking these clinical features, whereas group 8 had a significantly lower level (P < 0.05) when compared to all other groups. Conclusion. Our results show that CIC may be involved in the pathogenesis of BD, especially in those clinical forms of the disease with erythema nodosum, neurologic manifestations, and ocular manifestations. Patients at the mild end of the BD spectrum do not show significant changes in CIC levels compared to healthy control subjects. We can, therefore, suggest that in BD CIC may be implicated more in the pathogenesis of some features than of others.     

X-RAY EMISSION MICROANALYSIS OF THE DENSITY OF HAIR PROTEINS IN KWASHIORKOR

SUMMARY. Hair samples were obtained from the scalps of 5 infants with kwashiorkor and 5 matched control infants on a balanced diet.
The greatest diameters of the hairs were measured in their natural state, ethanol, xylol, water and air. The diameters of the kwashiorkor hairs were 8 to 22% larger in water than in air while the diameters of the control hairs were 5 to 8% larger. This indicates that the stability of the cross links between the polypeptide chains of hair proteins is reduced in kwashiorkor.
The mass per unit area of cryostat sections of hair was estimated by electron probe x-ray emission microanalysis. No difference was demonstrable between the density of proteins in kwashiorkor and control hair. It is concluded that the soft texture of kwashiorkor hair is not associated with a reduction in the density of its proteins.     

ULTRASTRUCTURAL ASPECTS OF DERMATITIS HERPETIFORMIS (DUHRING''S DISEASE)

Summary.— Biopsies of clinically normal skin and erythematous and recent vesicular lesions of 3 patients with dermatitis herpetiformis (DH) were studied by electron microscopy.
The early events at the dermo–epidermal junctional zone were the presence of glycogen and lipid particles in the basal cells, rarefaction of half-desmosomes, and small duplications of the basal lamina.
In some areas of the dermo-epidermal junction of the erythematous skin separation between epithelial basal cells and basal lamina was observed. In the vesicles fibrin, with its typical cross-banding pattern, was seen.
The early changes observed in the epithelial germinative cells were interpreted as evidence of disturbance in dermo-epidermal interaction, possibly caused by immunoglobulin deposition at this level. The abnormal synthesis of half-desmosomes by the basal cells could be the cause of supra-laminar dis-junction.