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Objective: To improve the diagnosis of laryngeal disease in patients with gastroesophageal reflux disease (GERD)., Material and Methods: 171 patients (89 (52%) men, 82 (48%) women) with non-erosive (39 (23%) people) and erosive (132 (77%) people) forms of GERD were examined. None of the patients made active complaints about the condition of the larynx and pharynx. The examination included a questionnaire, examination by an otorhinolaryngologist, and videoendolaryngoscopy., Results: The survey revealed the presence of symptoms of otorhinolaryngological pathology in 74% of patients. Videolaryngoscopy revealed no laryngeal pathology in 55 (32%) patients, and signs of GERD-associated laryngitis were detected in 116 (68%) patients. The most common pathology was pachydermia in interarytenoid region - in 89 people, laryngeal granuloma was detected in 7 patients, leukoplakia - in 2 patients, chronic edematous polypous laryngitis - in 2 patients, benign laryngeal formations - in 9 patients. In patients with GERD (erosive and non-erosive forms), an asymptomatic course of chronic laryngitis associated with this pathology was observed in 44% of cases. In 52% of patients, pachydermia in interarytenoid region was diagnosed, of which every second had pronounced hyperplasia and folding of the mucous membrane in interarytenoid region, which must be differentiated from laryngeal cancer (in this case, patients should be under the dynamic supervision of an otorhinolaryngologist). Other precancerous formations of the larynx (granulomas and leukoplakia) were found in 5% of patients. Benign neoplasms of the larynx (polyps and cysts) were also detected in 5% of cases., Conclusions: Despite the high frequency of detection of otorhinolaryngological diseases in the examined patients with gastroesophageal reflux disease, none of the patients presented complaints related to the pathology of the larynx, which indicates the need for active detection of this pathology in this category of patients by questioning, questioning, as well as examination by an otorhinolaryngologist.

Background: The diagnosis of vocal cord leukoplakia mainly relies on laryngoscopy. The morphology of vocal cord leukoplakia under laryngoscope is closely related to the pathological nature of leukoplakia. The specific manifestations associated with high-risk vocal cord leukoplakia remain to be explored., Objective: To investigate the characteristics of low-risk and high-risk vocal cord leukoplakia under conventional white light imaging (WLI) laryngoscopy and its correlations with narrow band imaging (NBI) laryngoscopy., Methods: One hundred and seventy-five cases of vocal cord leukoplakia were divided into low-risk and high-risk groups. The characteristics of low-risk and high-risk vocal cord leukoplakia under WLI laryngoscopy and its correlations with NBI laryngoscopy were analyzed., Results: Logistic regression analysis showed that thickness and hyperemia were independent factors ( p  < .05). Hyperemia had a strong consistency with the visualization of spots under NBI laryngoscopy (kappa = 0.758). The sign of hyperemia and the NBI classification had equivalent diagnostic efficacy for predicting the risk of cancerization of vocal cord leukoplakia., Conclusion: The sign of hyperemia under WLI laryngoscopy is significantly correlated with the visualized spots under NBI laryngoscopy. Hyperemia is an important feature for predicting malignant potential of vocal cord leukoplakia.

A 53-year-old female patient with acute myeloid leukemia developed severe chronic graft vs host disease (cGVHD) of the oral mucosa after allogeneic hematopoietic stem cell transplantation with leukoplakia and relapsing oral squamous cell carcinoma (SCC) of the tongue. cGVHD needed long-lasting immunosuppressive therapy; SCC was treated with radiation and surgery. Acute myeloid leukemia remained in complete remission. The patient developed a myositis with pain of all muscles as well as paraparesis with elevated creatine kinase and C-reactive protein and detection of antiskeletal muscle autoantibodies 3500 days after hematopoietic stem cell transplantation. No other clinical features of chronic GVHD were apparent at this time. Symptoms disappeared after treatment with corticosteroids but relapsed while tapering. Weekly therapy with the B-cell-depleting antibody rituximab was started and administered for 6 weeks. Symptoms disappeared again but partly returned after some weeks, so therapy with azathioprine was started. During therapy with azathioprine slow tapering of corticosteroids was possible and clinical symptoms remained absent. Here we present a case report and review of the literature on alloimmune myositis as paraneoplastic complication of an oral SCC of the tongue after severe chronic GVHD or as late manifestation of chronic GVHD itself., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Backgound: Pepsin immunohistochemical (IHC) staining is a promising diagnostic approach of laryngopharyngeal reflux (LPR). The interarytenoid mucosa has been proved to be an effective biopsy area. Objectives: To investigate whether positive result of pepsin IHC staining in laryngeal lesions can predict LPR. Methods: The study included 136 patients with laryngeal cancer or vocal cord leukoplakia. 24 h multichannel intraluminal impedance-pH (MII-pH) was performed before operation, and pepsin IHC staining was performed on pathological sections after operation. The results of the two methods were compared. Results: Among the 136 patients, 101 with at least one LPR event were regarded as MII-pH positive group, and another 35 were negative. The positive rate of pepsin IHC staining was 93.1% in MII-pH positive group and 54.1% in MII-pH negative group ( p  < .05). If the MII-pH results were used as a reference, the sensitivity and specificity of pepsin IHC staining in the diagnosis of LPR were 93.1 and 45.7%, respectively. The consistency of pepsin IHC and MII-pH was moderate (Kappa value = 0.452). Conclusions: The sensitivity of pepsin IHC staining in laryngeal lesions for diagnosing LPR is satisfactory. The existence of false negative of MII-pH may be the main reason for the low specificity.

Objectives: Previous evidence suggested Helicobacter pylori (H. pylori) promoted tumorigenesis by inducing inflammation in gastric premalignant disease. Whether this organism affected the development of head and neck precancerous lesion remains unknown. We performed this study to assess the prognostic impact of H. pylori infection on patients with laryngeal precancerous lesion., Methods: In this prospective study, 41 enrolled patients with laryngeal precancerous lesion were arranged into H. pylori-positive group (n=16) and H. pylori-negative group (n=25). Two poor outcomes were recurrence and malignant transformation. Prognostic impact of H. pylori infection was analyzed using the Univariate Cox proportional hazard model and Cochran-Mantel-Haenszel test. We also analyzed the prognostic value of systemic inflammation-based markers., Results: During a median follow-up of 32.9 months, recurrence developed in 8 patients in the H. pylori positive group and 5 patients in the H. pylori negative group (50.0% vs. 20.0%, P=0.026, Log-rank test). Malignant transformation developed in 4 patients in the H. pylori positive group and no patients in the H. pylori negative group (25.0% vs. 0.0%, P=0.005, Log-rank test). H. pylori positive group had a significant higher malignant transformation rate after stratifying on the observed covariates. There was no significant difference of recurrence rate in two groups stratified with laryngopharyngeal reflux, size of lesion, and pathological type (Cochran-Mantel-Haenszel test). The basal levels of lymphocyte counts and neutrophils to lymphocytes ratio were significantly different in patients with H. pylori infection. Moreover, the receiver operating characteristic curve analysis revealed a predictive value of lymphocyte counts and neutrophils to lymphocytes ratio for poor outcomes (areas under the curve, 0.658 and 0.651, respectively)., Conclusion: The data demonstrate a malignant transformation-relevant role of H. pylori in laryngeal precancerous lesion. H. pylori infection combined with laryngopharyngeal reflux, size of lesion, and pathological type might relate to recurrence of laryngeal precancerous lesion. And changes of lymphocyte counts and neutrophils to lymphocytes ratio associated with H. pylori infection might deserve as predictive indicators for poor prognosis of laryngeal precancerous lesion., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Objective: In laryngology practice, vocal fold leukoplakia is frequently evaluated by suspension laryngoscopy and biopsy examination upon the patient's complaints of hoarseness and dysphonia. The purpose of the present study is to investigate and analyze risk factors, diagnosis, treatment, and follow-up results of cases with Candida leukoplakia., Study Design: Retrospective case control study., Setting: Tertiary medical center., Subjects and Methods: Patients with a diagnosis of vocal fold leukoplakia who underwent direct laryngoscopy and biopsy between 2007 and 2017 and diagnosed as candida or noncandida in their histopathology were assigned into 2 groups. Then they were compared in terms of their demographic characteristics, predisposing factors, diagnosis, treatment, and follow-up results., Results: Of the 289 vocal fold leukoplakia cases, 36 were candida, and 253 were noncandida. The mean age of the patients with Candida leukoplakia was 60.86 years. As for the age groups, the largest group (26.1%) was in the seventh decade ( P < .001). The use of inhaled corticosteroids was a significant risk factor ( P < .001). For their medical therapy, the patients were administered fluconazole 200 mg per day for 3 weeks, and the treatment yielded successful results in 91.66% of them. In 5 of the patients, candida leukoplakia and superficial epithelial dysplasia were observed, and no malignant transformation was observed during a mean follow-up of 28 ± 13 months., Conclusion: Candidiasis causing vocal fold leukoplakia is rare, and we report the findings of the largest published case series to date. Eliminating predisposing factors and administrating oral fluconazole 200 mg for 3 weeks are sufficient for medical treatment.

Competing Interests: Competing interests: None declared.

Oral leukoplakia and erythroleukoplakia are common oral potentially malignant disorders diagnosed in the oral cavity. The specific outcome of these lesions remains to be elucidated, as their malignant transformation rate exhibits great variation. The ability to predict which of those potentially malignant lesions are likely to progress to cancer would be vital to guide their future clinical management. The present study reported two patients with tongue squamous cell carcinoma: Case study 1 was diagnosed with a simultaneous leukoplakia and case study 2 developed an erythroleukoplakia following the primary tumor treatment. Whole genome copy number alterations were analyzed using array comparative genomic hybridization. The present study determined more genomic imbalances in the tissues from leukoplakia and erythroleukoplakia compared with their respective tumors. The present study also identified in tumor and potentially malignant lesions common alterations of chromosomal regions and genes, including FBXL5, UGT2B15, UGT2B28, KANSL1, GSTT1 and DUSP22, being some of these typical aberrations described in oral cancer and others are linked to chemoradioresistance. Several putative genes associated with hallmarks of malignancy that may have an important role in predicting the progression of leukoplakia and erythroleukoplakia to squamous cell carcinoma, namely gains in BNIPL, MCL1, STAG2, CSPP1 and ZNRF3 genes were also identified.

Conclusion This study indicates that IL-10 promoter polymorphism variants, smoking, and alcohol consumption increase the risk of recurrence and canceration in vocal leukoplakia. Objective This prospective, clinical trial was performed to evaluate the association of interleukin (IL)-10 promoter polymorphism variants and canceration and recurrence rates in vocal leukoplakia (a pre-cancerous laryngeal carcinoma lesion) over a 2-year period. Participants and method Sixty-one post-operative patients with vocal leukoplakia were enrolled in this prospective, observational study and genotyped for the IL-10 promoter gene (IL-10-1082 A/G, -819 T/C and -592 A/C) using pyrosequencing, and responded to a 2-year follow-up survey. Recurrence and canceration rates were used to evaluate the association between the genotype variants and the clinical outcome. Results There was an increased canceration rate in the variant genotype group compared to that in the normal genotype group in the 2-year follow-up period (18.4% vs 0%, p-value = 0.038). Compared with the non-smoker group, the smoker group had a higher recurrence rate of vocal leukoplakia (29.3% vs 5%, p-value =0.044). Likewise, the recurrence rate in the alcohol consumption group was also higher (30.6% vs 8%, p-value =0.034). The percentage of cancerization in the alcohol consumption group was significantly higher than that in the non-alcohol consumption group (19.4% vs 0%, p-value =0.035).

Objective: To evaluate the relationship between pepsin induced by laryngopharyngeal reflux and laryngeal carcinoma., Methods: Patients with vocal cord leukoplakia(n=18) and laryngeal carcinoma(n=21) encountered in Nanfang Hospital between December 2012 and April 2014 were included and sixteen healthy volunteers were recruited as control. Laryngeal biopsy specimens were taken from the patients with laryngeal carcinoma, or vocal cord leukoplakia and control subjects for the immunohistochemical study of pepsin. The correlation between pepsin expression and reflux events of 24 hour multichannel intraluminal impedance-pH monitoring (MII-pH) was analyzed., Results: The patients with laryngeal carcinoma showed the highest expression of pepsin, followed by the patients with vocal cord leukoplakia and control subjects, with significant difference among the three groups (in strong positive expression, the constituent ratio of each group are 0/16、1/18 and 4/21, P<0.01). The presence of pepsin was associated with upright and total laryngopharyngeal acid reflux (P<0.05), including acid reflux episodes, the percentage of times that the pH was below four, the percentages of acid reflux time and average acid removal time. There was a significant correlation between the pepsin level and the esophageal acid reflux parameters (P<0.05) except supine the percentage of time that the pH was below four., Conclusions: Pepsin expression in laryngeal tissue increases in patients with vocal cord leukoplakia and laryngeal carcinoma, contributing to the development of laryngopharyngeal carcinogenesis.

The Acoustic Voice Quality Index (AVQI) is a relatively new clinical method to quantify dysphonia severity. Since it partially relies on continuous speech, its performance may vary with voice-related phonetic differences and thus across languages. The present investigation therefore assessed the AVQI's performance in English, Dutch, German, and French. Fifty subjects were recorded reading sentences in the four languages, as well as producing a sustained vowel. These recordings were later edited to calculate the AVQI. The samples were also perceptually rated on overall dysphonia severity by three experienced voice clinicians. The AVQI's cross-linguistic concurrent validity and diagnostic precision were assessed. The results support earlier data, and confirm good cross-linguistic validity and diagnostic accuracy. Although no statistical differences were observed between languages, the AVQI performed better in English and German and less well in French. These results validate the AVQI as a potentially robust and objective dysphonia severity measure across languages.

Objective: To investigate the correlation between clinicopathological characteristics of vocal fold leukoplakia and laryngopharyngeal reflux (LRP)., Methods: One hundred and thirty-eight patients with vocal leukoplakia who received laryngeal microsurgeries under suspension laryngoscope were recruited in this study. Reflux symptom index (RSI) and reflux finding score (RFS) were measured. The clinical features, pathological characteristics were collected., Results: There were 129 males and 9 females with a mean age of (53.4 ± 11.5) years. The mean age of patients with severe dysplasia/carcinoma and invasive carcinoma was significantly older than that of inflammation/hyperplasia (F = 6.773, P < 0.05) . In 138 patients, 85 patients (61.6%) were pathologically diagnosed as chronic inflammation or squamous epithelium hyperplasia, 18 patients (13.0%) as mild dysplasia, 10 patients (7.2%) as moderate dysplasia, 15 patients (10.9%) as severe dysplasia/carcinoma in situ and 10 patients (7.2%) as invasive carcinoma. One hundred and twenty-nine patients (93.5%) complained of hoarseness with clinical courses between 7 days and 20 years. 68.5% vocal folds with inflammation/hyperplasia showed nearly normal mucosal waves, and 72.7% to 80.0% vocal folds with moderate or more severe dysplasia showed severe reduced mucosal waves. A correlation existed between the severity of pathological type and the decreased extent of mucosal waves (rank correlation coefficient was 0.427, P < 0.001). The prevalence of LPR diagnosed with RSI > 13 or RFS > 7 was 54.3 %, while the positive rate of RSI was 21.7% and that of RFS was 43.5%. There was no significant differences of the positive rates of RSI and RFS among different pathological types (χ² values 3.537 and 7.619 respectively with both P > 0.05). According to RSI evaluation, the most common reflux symptoms were hoarseness (94.9%), throat clearing (68.8%) and globus sensation (62.3%). The most common RFS findings were posterior commissure hypertrophy (79.7%), thick endolaryngeal mucus (77.5%) and vocal fold edema (70.3%)., Conclusions: The vocal fold leukoplakia consisted of various pathological types including benign, dysplasia and malignancy. There was a correlation between pathological types and the extent of mucosal waves. About half of the patients with vocal fold leukoplakia existed LPR using RFS and RSI evaluation, but no significant difference of LPR positive rates were observed among different pathological types.

Objective: To explore the significance of laryngopharyngeal reflux (LPR) and gastroesophageal reflux (GER) in patients with vocal cord leukoplakia and early glottic cancer., Methods: Patients with vocal cord leukoplakia and early glottic cancer encountered in Nanfang Hospital between December 2012 to January 2014 were included in this study. Ambulatory 24 hour multichannel intraluminal impedance-pH monitoring (MII-pH) was applied to obtain LPR and GER events, as well as the reflux properties of substances. Tobacco and alcohol history was also evaluated. Sixteen healthy volunteers were recruited as normal controls., Results: There were 26.3% (5/19) LPR patients in glottic cancer group, 35.3% (6/17) LPR patients in vocal cord leukoplakia group and 12.5% (2/16) LPR volunteers in normal controls. There was no statistically significant difference in the positive rate of LPR between early glottic cancer patients and normal controls as well as between vocal cord leukoplakia patients and normal controls (P > 0.05). There was statistically significance in numbers of acid reflux events, time of acid exposure, and time of acid clearance between vocal cord leukoplakia patients and normal controls as well as between glottic cancer patients and normal controls (P < 0.05). GER was found in 26.3% (5/19) patients in glottic cancer group and 23.5% (4/17) patients in vocal cord leukoplakia group and 6.3% (1/16) volunteer in normal controls. There was no statistically significant difference in the positive rate of GER between early glottic cancer patients and normal controls as well as between vocal cord leukoplakia patients and normal controls (P > 0.05). However, there was statistically significance in DeMeester scores between glottic cancer patients and normal controls (P < 0.05), while no statistically significance between vocal cord leukoplakia patients and normal controls (P > 0.05). Reflux events were dominated by acid and weakly acidic reflux in upright position. Weakly alkaline reflux events in upright position, acid reflux events in supine position, and weakly alkaline reflux events in supine position in vocal cord leukoplakia patients were significantly more than those in normal controls (P < 0.05). No statistically significant difference existed in positions and contents between early glottic cancer patients and normal controls (P > 0.05). There was also no statistically significant correlation between happening LPR and GER, smoking and drinking in patients with vocal cord leukoplakia and early glottic cancer (P > 0.05)., Conclusions: Reflux events are more in vocal cord leukoplakia patients and early glottic cancer patients, however, the relationship between laryngopharyngeal reflux and canceration of the vocal cord is still needed to be investigated. The significance of mucosal injury induced by nonacid refluxes is needed to be further studies.

Treating patients with multiple oral leucoplakias (MOLs) who smoke is more difficult and complicated than treating those with single oral leucoplakia (SOL). Removing the aetiological factors causing MOL can effectively prevent its recurrence. The aim was to study the infection by and influences of Candida in smoking patients with MOLs. A retrospective study was conducted on 136 smoking patients who had clinicopathological OLs. Among these patients, 73 lesions in 31 patients were MOLs, while 105 patients had SOLs. All patients were treated by complete resection. All specimens were tested for epithelial dysplasia, and stained with periodic acid-Schiff reagent. The rate of MOL concurrence with candidal infection was higher than that of SOLs. The incidence of Candida associated with MOLs was higher for recurrent than for non-recurrent lesions. The disease-free time was shorter in MOL patients with candidal infection. Moreover, MOLs with candidal infection were more likely to have an increasing ratio to combine with epithelial dysplasia. Candida is an important risk factor in smoking patients with MOLs. Microscopic and fungal examinations of those lesions should permit a detailed diagnosis in such patients and for long-term predictive assessments., (© 2010 Blackwell Verlag GmbH.)

Objective: The objective of the present study was to assess the effect of tobacco chewing on buccal mucosa by using cytomorphometry., Study Design: We compared the cellular diameter (CD), nuclear diameter (ND) and the ratio of nuclear diameter to cellular diameter (N/C) of buccal mucosa squames of normal subjects (N) with buccal mucosa squames of tobacco users without lesion (A), with tobacco-lime lesion (B), leukoplakia (C), and oral squamous cell carcinoma (D). The study group consisted of 125 patients divided into five groups (N, A, B, C and D) between the ages of 21 and 75 years., Results: The mean of the cellular diameter (in micrometers) of group N, A, B, C, and D was 72.86+/-5.26, 68.30+/-3.02, 62.13+/-3.29, 57.75+/-4.66, 54.51+/-4.66 respectively (p<0.01). The mean of the nuclear diameter (in micrometers) of group N, A, B, C, and D was 8.70+/-1.30, 8.98+/-1.08, 9.06+/-0.83, 9.12+/-1.06, and 11.04+/-1.46 respectively (p<0.01). The mean of the ratio of nuclear diameter to cellular diameter of group N, A, B, C, and D was 0.11+/-2.00, 0.13+/-1.82, 0.14+/-1.35, 0.16+/-3.11, 0.21+/-4.51 respectively (p<0.01). Univariate analysis of variance (ANOVA) showed a significant group effect for cellular diameter, nuclear diameter and ratio of nuclear diameter to cellular diameter. Multiple comparison tests by Tukey-HSD procedure revealed a significant decrease in the mean cellular diameter, increase in the nuclear diameter and ratio of nuclear diameter to cellular diameter., Conclusions: Cytomorphometric changes could be the earliest indicators of cellular alterations. There is progressive decrease in cellular diameter, increase in nuclear diameter and increase in ratio of nuclear diameter to cellular diameter in smears from all tobacco users, as compared to normal subjects. This indicates that there could be cause-effect relationship between tobacco and quantitative alterations.

We present a case of a 45-year-old patient who reported bleeding hemorrhoids. He had grade II hemorrhoids and was found to have a circumferential leukoplakic lesion at the anal verge. The patient had no symptoms of leukoplakia. The hemorrhoids were ablated with an Ellman radiowave device. Histological findings of the incised lesion from the verge showed hyperkeratosis and acanthosis extending cephalad from the anal verge to the dentate line. Follow-up after 5 years revealed no evidence of dysplastic changes in the leukoplakic lesion. Because of the varied opinions regarding the fate of leukoplakia of the anal canal, patients should be followed carefully to detect any malignant transformation of the lesion.

We present a case of prostatic abscess and coexistent leukoplakia of the urethra in a 51-year-old man. He had been suffering from diabetes mellitus for 10 years and following cessation of high fewer, transurethral electrovaporesection was performed.

The main advantage of photodynamic therapy (PDT) in laryngology seems to be its non-invasiveness and the possibility of using it despite previous application of conventional methods. In the study, we gave PDT to two separate groups of patients, i.e. five patients with advanced tumour (four of them with local recurrence (squamous cell carcinoma) after surgery and radiotherapy and one with a primary hypopharyngeal tumour) and five patients with leucoplakia. In the first group delta-aminolaevulinic acid (ALA) was administered orally (dose 3 g), while in the second, an ointment containing 10% ALA was applied locally. In both groups prior to irradiation, the tissue level of protoporphyrin IX was verified using an adapted Xillix Life instrument. All treated lesions were irradiated with an argon-pumped dye laser (dose range 100-250 J/cm2, wavelength 635 nm). In the first group, partial response was obtained. In these advanced cases, diminution of cancerous ulcerations was observed. In the leucoplakia group, complete response was achieved in four out of five treated patients. On the basis of our preliminary results, PDT may be useful in eradicating premalignant lesions of the oral cavity and in the palliation of advanced lesions of the oropharynx and larynx.

Eighty syrian hamsters were divided into 4 equal groups. The right buccal pouches of hamsters in group I and group II were painted three times weekly with 0.5% DMBA dissolved in acetone, but the hamsters in group I received 0.45 g Taixian tablet daily by mouth. The animals in group III only received 0.45 g Taixian tablet daily and group IV was control group. After 9, 12 weeks, animals were killed with their pouches excised, and tumors were counted and measured. The results showed; 1. Comparing with group II, the malignant rate of group I was lower after 9 weeks and the tumor volume was smaller after 12 weeks (P < 0.01); 2. It was found histologically many inflammatory cells locating in the epithelial layer and lamina propria of group I after 9 weeks, while only a few inflammatory cells in group II. The high differentiated squamous cell carcinoma could be seen in group I and group II after 12 weeks, but no abnormal changes in cervical lymphnodes and organs (lung, liver, spleen, et al) of all animals in 4 groups. It suggests that Taixian tablet can restrain the development of oral carcinogenesis.

An increasing number of syndromes with palmoplantar keratoderma (PPK) with associated diseases are being identified, representing a wide spectrum of distinct entities. At present only one case report has described the combination of marked anogenital leukokeratosis with diffuse PPK evolving in a collodion baby. We report a patient with a diffuse, nonprogressive PPK in combination with an intermittently pruritic, slowly progressive anogenital leukokeratosis. Hyperkeratosis of the perineal area was most pronounced and extended to the distal portion of the anal mucosa. The opalescent lesion was also visualized at the margin of the major labia. Vulvar structures were not otherwise involved or dystrophic. There were no signs or symptoms of ectodermal dysplasia. Specifically, the nails were normal and showed no signs of pachyonychia congenita. Other differential diagnoses included dyskeratosis congenita and white sponge nevus, which may be associated with anogenital leukokeratosis, but a keratoderma is not associated with these entities. Keratin immunocytochemistry showed marked expression of suprabasal K17 and absence of K6 and K16. Further examination of the initial case described by Itin and Rufli demonstrated the same expression pattern and supports the contention that these two cases represent the same entity.

Materials and Methods: The research has been carried out with the cooperation of Department of Otorhinolaryngology of the "Ospedale S.S. Trinita" in Cagliari, on 294 patients suffering from oral malignant tumours between 1984 and 1994. This kind of disease was studied in relation to age, sex, histological type, and afterwards the examination results were compared to other researchers' studies. All the 294 patients were recorded in the operating theatre's registers. Oral malignant tumours amount to about 45% of the neoplastic systemic pathology. Use and misuse of alcohol, smoking, repeated blows resulting in pre-cancerous lesions including leukoplakia, might be considered the main causes of the origin of oral tumours., Results: Male patients (237) amount to 80.7% of the examined sample, while female ones (57) reach only 19.3%. The part most frequently involved is the tongue (97 cases, 33%), then the area of the lower lip (69 cases, 23.5%), and finally the lower part of the oral space (47 cases, 15.58%)., Conclusions: From the histological point of view, the squamous carcinoma is found more often in the tongue, in the lower oral space and in the lips, while the basal carcinoma in the lower lip rather then in the upper one. A remarkable increase in the squamous carcinoma and a grow in the areas of the tongue has been observed.

We describe a pediatric patient with dyskeratosis congenita, whose symptoms included abdominal pain, vomiting, dysphagia, and hematochezia. Gastrointestinal symptom are prominent in this rare genetic disorder.

Dyskeratosis congenita (DC) is a rare congenital X-linked disorder. The major clinical manifestations are abnormal skin pigmentation, nail dystrophy, and leukoplakia of mucosal membranes. About 50% of the patients develop bone marrow failure, which is partly responsible for the poor prognosis. Recombinant human granulocyte-macrophage colony-stimulating factor (rhGM-CSF) has been administered to some neutropenic patients with DC, but only a moderate stimulation of neutropoiesis has been observed. We report on a patient with DC treated with recombinant human granulocyte-colony-stimulating factor (rhG-CSF). This treatment resulted in a substantial dose-dependent increase in the neutrophil count.

A 37-year-old male presented with a cauliflower-like tumor in the right buccal mucosa, leukoplakia of the tongue and esophagus, and keratotic papules or plaques on the extremities including bilateral palms and soles, associated with borderline diabetes mellitus and a repeated history of infections. A multi-system keratinizing abnormality was suspected, although no distinct disease category has been found showing similar involvements.

Dyskeratosis congenita is a congenital multisystem disorder, characterized by skin pigmentation, dystrophic nails, and leukoplakia. Hematologic abnormalities progressing to severe pancytopenia play a significant role in the poor prognosis of afflicted patients. We report on a patient with dyskeratosis congenita and severe aplastic anemia, complicated by life threatening infection. The patient was treated with recombinant granulocyte-macrophage colony-stimulating factor. This therapy resulted in a moderate and transient improvement in absolute neutrophil counts. Current concepts regarding the pathogenesis and etiology of dyskeratosis congenita are discussed, while reviewing the available therapeutic options.

Dyskeratosis congenita is a multisystem disorder with an increased incidence of neoplasia and opportunistic infections. A case is reported as a cause of complete nasopharyngeal atresia.

Objective: This study was designed to assess the significance of HIV-related oral lesions in predicting the rate of progression to AIDS., Design: Cohorts were investigated prospectively, and oral examinations were performed by clinicians trained in the diagnosis of oral lesions., Setting: We studied three existing cohorts of homosexual and bisexual men in San Francisco, California, USA., Participants: Of the HIV-infected men who received standardized oral examinations (n = 791), 603 were eligible for analysis of baseline examinations and 448 for analysis of follow-up examinations., Main Outcome Measures: We determined time from presence of oral lesion at baseline or follow-up examination, or from participant self-reported history of the lesion, to diagnosis of AIDS., Results: Using proportional hazard regression and stratifying by CD4 lymphocyte count at the time of baseline oral examination, we found that the rate of development of AIDS was increased among men with hairy leukoplakia [relative hazard, 1.8; 95% confidence interval (CI), 1.2-2.7], oral candidiasis (relative hazard, 7.3; 95% CI, 3.1-17.3), and both lesions (relative hazard, 3.1; 95% CI, 1.6-6.1) compared with men with normal findings. On follow-up examination, stratifying for CD4 count, the rate of progression to AIDS was similar for those with hairy leukoplakia compared with those with oral candidiasis. The progression rate from oral candidiasis to AIDS was faster from presence on baseline examination than from presence on follow-up examination or from self-reported history of the lesion., Conclusion: The presence of oral candidiasis and/or hairy leukoplakia on baseline examination confers independent prognostic information and should be incorporated into HIV-staging schemes.

A 24-year-old Japanese man presented with dyskeratosis congenita (DC, Zinsser-Cole-Engman syndrome) complicated by non-cirrhotic portal hypertension, signet ring carcinoma of the rectum and Pneumocystis carinii pneumonia. At the age of 9 years, he was diagnosed as having DC on the basis of typical clinical manifestations including atrophic lingual papillae, hyperpigmentation of the skin, thrombocytopenia, and ophthalmological abnormalities. A few years later pancytopenia and splenomegaly developed. At 24 years, signet ring carcinoma of the rectum was detected but could not be resected because of the severity of the pancytopenia. Death was due to respiratory failure from P. carinii pneumonia. At autopsy the case illustrated several unique findings for DC, including non-cirrhotic portal hypertension, atrophy of frontal lobe and markedly slender folia of the cerebellum and superimposed infections with herpes zoster virus and P. carinii. Striking lymphocyte depletion and atrophy of lymphoid parenchyma in lymph nodes, tonsils, spleen, gastrointestinal tract, or thymus were seen histologically. The morphological picture supports the suggestion that there is a defect in the cell-mediated immune system in patients with DC, although immunoglobulin levels in the blood are normal. The cell-immune deficiency is a major factor in the poor prognosis.

A case of keratinizing desquamative squamous metaplasia (KDSM) of the upper urinary tract is presented showing a "goblet" sign. The relationship between KDSM, cholesteatoma, and leukoplakia is discussed and conservative management is recommended.

A patient who had vesical leukoplakia with unusual macroscopic and histological findings was studied. The mucous membrane of the urinary bladder in this patient showed a tumor-like proliferation similar to verrucous hyperplasia which is often found in the oral mucosa. We propose a new classification of vesical leukoplakia, dividing it into three groups, atrophic, hypertrophic and verrucous types. The atrophic type has a heavily keratinized, flat and thin type of epithelium with features resembling lichen sclerosis et atrophius. Histologically, it shows a variable degree of hyperkeratosis but no parakeratosis. The hypertrophic type has histological characteristics resembling features of leukoplakia in sites elsewhere in the body, which is marked by hyperkeratosis and irregular hyperplasia of the prickle cell layer with lengthening and abnormality in shape of rete pegs. The verrucous type has very rare features of coral shape, white color and verrucous proliferation. Histologically, this type shows severe hyperkeratosis, parakeratosis and elongation of the rete pegs, but no irregular invasion can be observed.

The pandemic, acquired immunodeficiency syndrome (AIDS) has been described in 40 nations throughout the world. This paper describes the wide spectrum of gastrointestinal tract manifestations seen in this syndrome, with particular attention to the epidemiology, etiology, and measurement of these problems. Discussion of candidiasis, herpes simplex, "hairy" leukoplakia, Kaposi's sarcoma, cytomegalovirus, anal warts and carcinoma, chlamydial proctitis (LGV), coccidiosis, and mycobacterial diarrhea, as well as "gay bowel syndrome," demonstrates the complex management problems associated with this condition.

A case of dyskeratosis congenita is reported. This rare hereditary disease usually has the following progression: ectodermal dystrophy (reticular skin pigmentation, nail dystrophy, leukokeratosis of mucosal membranes), appearing in the first decade, followed in about 50% of these patients by a hematopoietic disorder resembling Fanconi's anemia, usually developing in the second or third decade. Carcinomas may occur in leukokeratotic areas in the third, fourth, or fifth decade. This patient's clinical course is interesting because the thrombocytopenia developed as an isolated symptom at the age of 5 years and preceded the skin anomalies by three years. The diagnosis of dyskeratosis congenita was made only after an evolution of five years. The diagnosis of dyskeratosis congenita--although it is a rare disease--should be considered in every child first seen with aplastic anemia or thrombocytopenia.

Pachyonychia congenita is a rare hereditary disorder characterized mainly by nail hypertrophy and dyskeratoses of skin and mucous membranes. A thorough literature survey since its first description in 1904 up to 1985 revealed 168 cases of pachyonychia congenita. There were no indications of any sex or ethnic group predilection. Based on this survey the following classification is suggested: type I (56.2% of cases), hyperkeratosis of nails, palmoplantar keratosis, follicular keratosis, and oral leukokeratosis; type II (24.9% of cases), clinical findings of type I plus bullae of palms and soles, palmar and plantar hyperhidrosis, natal or neonatal teeth, and steatocystoma multiplex; type III (11.7% of cases), clinical findings of types I and II plus angular cheilosis, corneal dyskeratosis, and cataracts; and type IV (7.2% of cases), clinical findings of types I, II, and III plus laryngeal lesions, hoarseness, mental retardation, hair anomalies, and alopecia.

The article reports on a 4-year-old girl with multiple congenital malformations. Ophthalmological examination revealed microphthalmia, bilateral colobomas of iris, leukoma of the right eye, convergent strabismus in the left eye. An oval salmon-coloured area was seen at the fundus of the left eye where the retinal vessesl proceed into the depth. There was no vascular infundibulum. A papilla-like cavity was seen at the bottom left of the coloboma (possibly a circulus zinnii, also called circulus vasculosus nervi optici, may be present). Both papillae are connected with each other by means of a vein. This patient, therefore, presented an extremely rare case of malformation with two papillae in one coloboma.