Gingival involvement in mucous membrane pemphigoid

A 60-year-old woman with clinical features of desquamative gingivitis had a history of painful, blistering gingival lesions for more than 2 years. There were no other accompanying mucosal or skin lesions. Clinical examination revealed erythematous and edematous gingiva with ulcerated areas and evidence of intact and ruptured bullae. White plaquelike lesions were also noted. Gingival manipulation caused epithelial desquamation. Light microscopic examination of biopsy specimens from the perilesional gingival tissue showed separation of the oral gingival epithelium and connective tissue at the margin of the collapsed bulla. A moderately intense inflammatory infiltrate was present in the connective tissue. Direct immunofluorescent microscopy revealed a continuous linear deposition of immunoglobulin G and C3 at the basement membrane zone. On the basis of clinical, histopathologic, and immunofluorescent findings, the diagnosis of mucous membrane pemphigoid was made.     

Stevens-Johnson syndrome and toxic epidermal necrolysis are severity variants of the same disease which differs from erythema multiforme

A new classification, based on the pattern and distribution of cutaneous lesions, separates erythema multiforme major from Stevens-Johnson syndrome. A retrospective re-classification of 76 cases supported the validity of that separation by demonstrating differing causes and pathology. Another prospective international case-control study found differing demographic characteristics and risk factors between erythema multiforme major on the one hand and Stevens-Johnson syndrome or toxic epidermal necrolysis on the other. Erythema multiforme major was mainly related to Herpes virus infection, while Stevens-Johnson syndrome and toxic epidermal necrolysis were associated with drug reactions.     

Stevens-Johnson syndrome associated with carvedilol therapy

Stevens-Johnson syndrome, related to carvedilol use, has not been previously reported as a serious adverse experience requiring hospitalization. We report this reaction in a 71-year-old man with stable ischemic cardiomyopathy.     

Epidemiology of erythema exsudativum multiforme majus, Stevens-Johnson syndrome, and toxic epidermal necrolysis in Germany (1990-1992): structure and results of a population-based registry

The severe skin reactions erythema exsudativum multiforme majus (EEM with mucosal involvement, EEMM), Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN) are difficult to study as they are very rare diseases with an incidence of about two cases per 1 million inhabitants per year. We report on the structure of a registry with the aim of ascertaining all hospitalized cases of EEMM, SJS, and TEN in western Germany and Berlin. The registry is structured as an intensive reporting system, regularly contacting more than 1500 departments including 100% of the burn units (n = 34), departments of pediatrics (n = 241), departments of dermatology (n = 106), and 100% of all internal medicine departments in hospitals with intensive care facilities or with more than 200 beds (n = 1161). With a coverage rate up to 95% based on the number of responding departments between April 1, 1990 and December 31, 1992, from a total of 767 reported cases 353 patients with EEMM, SJS, and TEN were finally included in the registry. Most of these patients were directly reported to the registry; only 2.54% (9 of 353) were primarily registered by the German spontaneous reporting systems. Assuming an average population of 64.5 million for western Germany and Berlin an incidence up to 1.89 per 1 million inhabitants per year could be calculated for SJS and TEN.     

Helicobacter pylori in mucous membrane of stomach and duodenum of patients with symptoms of ulceration and dyspepsia

The material consisted of samples of mucous membrane of stomach and duodenum obtained during endoscopy in patients with clinical symptoms of peptic ulcer of stomach or the duodenum or with dyspeptic problems. Samples were tested for presence of H. pylori by culture on brain-heart agar supplemented with 7% of horse blood. Direct test for urease production was also performed. Isolated strains were identified basing on morphology of growth, Gram-stained preparation, mobility of microorganism, production of oxidase, catalase and urease, and ability to agglutinate in immune goat serum for standard H. pylori strain. Out of tested 217 samples, positive result was obtained in 141 cases. Urease test was positive in 138 cases. Isolated strains were tested for susceptibility to 14 antimicrobials. They were all resistant to nalidixic acid and susceptible in 90-100% to cephalothin, furazolidone, gentamicin and ofloxacin.     

Gynecologic disorders in women with Ehlers-Danlos syndrome

OBJECTIVE: We characterized the population with Ehlers-Danlos syndrome with regard to genital prolapse, urinary incontinence, and other gynecologic disorders. METHODS: Forty-one adult women who had registered for a first-ever Ehlers-Danlos multidisciplinary clinic participated in the study. Each had a comprehensive standardized evaluation, including gynecologic history, physical examination, urodynamic testing, and physical therapy evaluation. Qualitative and quantitative data were analyzed to determine means for various gynecologic disorders of Ehlers-Danlos syndrome. RESULTS: The frequencies of incontinence complaints (59%), endometriosis (27%), dyspareunia (57%), and previous hysterectomy (44%) were higher than expected for a population with a mean age of 41 years. Incontinence could not be demonstrated objectively. Prolapse was diagnosed in 12 (29.3%). CONCLUSIONS: Careful attention should be paid to women with Ehlers-Danlos syndrome because of an association with many gynecologic complaints. Women with Ehlers-Danlos syndrome should be questioned regarding incontinence, genital prolapse, endometriosis, and dyspareunia.     

Tetracaine-lidocaine-phenylephrine topical anesthesia compared with lidocaine infiltration during repair of mucous membrane lacerations in children

When a rigorous methodological approach is utilized, a substantial majority of recent studies provide evidence for the familial transmission of schizophrenia. Although the absolute rates of schizophrenia among relatives of schizophrenics tend to be lower than those reported in the earlier studies due to the restrictiveness of contemporary definitions of schizophrenia, the risk to relatives compared to that of controls has remained quite consistent. This observation that relatives of schizophrenics have an elevated risk for schizophrenia compared to controls is consistent with theories of both genetic and environmental transmission. Twin studies of schizophrenia have consistently reported greater concordance rates for monozygotic than dizygotic twins. Although this indicates the importance of genetic factors, the less than 100% concordance for monozygotic twins observed in every study indicates that nongenetic factors also play a role in the etiology of schizophrenia. Further, adoption studies offer an opportunity to unconfound genes and environment. The findings of adoption studies confirm that there are genetic components for schizophrenia. Even though we have shown that family, twin, and adoption studies have provided strong evidence for the role of genetic factors in schizophrenia, the mode of transmission remains unclear. The results of mathematical modeling studies do not support the single gene model. There is somewhat more support for the multifactorial polygenic model, but the model has also been rejected in several studies. Thus, the pattern of inheritance of schizophrenia has eluded an unambiguous characterization. Genetic linkage analysis promised to clarify the mechanisms of transmission, but early positive reports were subsequently overturned and, to date, there are no consistently replicated positive linkage findings for schizophrenia. There is now a world-wide search for the location of the genes on specific chromosomes which are responsible for schizophrenia. The clinical implications of current work to the future of locating a schizophrenic gene or genes will be discussed.     

Meige's syndrome associated with basal ganglia and thalamic functional disorders

Magnetic resonance imaging (MRI) or single positron emission computed tomography (SPECT) or both were performed and the responses of surface electromyography (EMG) were examined in seven cases of Meige's syndrome. MRI or SPECT or both demonstrated lesions of the basal ganglia, the thalamus, or both in five of the cases. Surface EMG revealed abnormal burst discharges in the orbicularis oculi and a failure of reciprocal muscular activity between the frontalis and orbicularis oculi in all the cases. These findings suggest that voluntary motor control and reciprocal activity in the basal ganglia-thalamocortical circuits are impaired in Meige's syndrome. In addition, good responses were seen to clonazepam, tiapride and trihexyphenidyl in these cases. Therefore, we conclude that dopaminergic, cholinergic, and gamma-aminobutyric acid (GABA) ergic imbalances in the disorders of the basal ganglia and thalamus in Meige's syndrome cause control in the excitatory and inhibitory pathways to be lost, resulting in the failure of integration in reciprocal muscular activity and voluntary motor control. This failure subsequently causes the symptoms of Meige's syndrome.