Oropharyngeal Ulcers Research Articles

Paraneoplastic pemphigus occuring after radiotherapy for relapsed non-Hodgkin's lymphoma in a patient with common variable immunodeficiency

Paraneoplastic pemphigus (PNP) is a rare multisystem, autoimmune disease with prominent mucocutaneous manifestations, occurring most commonly in association with haematological malignancies. It is characterised by the presence of circulating autoantibodies against epithelial adhesion proteins. We report a 46-year-old woman with common variable immunodeficiency who developed paraneoplastic pemphigus after receiving radiotherapy for relapsed non-Hodgkin's lymphoma. Flaccid bullae covering approximately 70% of the skin, painful oropharyngeal ulceration and periocular erosions were prominent clinical features. Despite supportive treatment and attempts at disease control using high-dose corticosteroids and cyclophosphamide, the patient became increasingly debilitated, developed septic shock secondary to Pseudomonas aeruginosa septicaemia on two occasions and died of respiratory failure 6 weeks after presentation. We highlight the need to be aware of (PNP) and to perform appropriate immunological investigations. In addition, we emphasise the importance of a multidisciplinary approach to the management of such patients.

Three cases of Behcee's disease with oropharyngeal ulceration

Three cases of Behcee's disease with oropharyngeal ulceration

Estudio histopatológico de 19 biopsias cutáneas de pacientes con sida e histoplasmosis diseminada

Disseminated histoplasmosis (DH) frequently affects the skin and oropharyngeal tissues. Among 1,800 aids patients, 19 had DH, al1 with cutaneous or mucocutaneous lesions. The skin showed macules, umbilicated crusty and scaly papules, nodules and ulcers, while 42% of the DH patients had oropharyngeal ulcers. Skin biopsies showed a variety of inflammatory patterns such as superficial and deep perivascular and nodular and diffuse dermatitis, pustular folliculitis and small vessel vasculitis with leucocytoclastic PMN. In five patients, cutaneous nerves were surrounded by the inflammatory reaction and numerous fungi within macrophages or Schwann cells were seen within the endoneural and perineural tissues. The main differential diagnoses were leucocytoclastic vasculitis, cryptococosis, mucosal aftae, paracoccidioidomycosis and diffuse cutaneous leishmaniasis. Clinicians, laboratory workers and pathologists should be aware of DH in HIV+ patients with maculo-papular cutaneous rash or mouth ulcers, since a prompt diagnosis allows a treatment to control the disease. Skin biopsy of these lesions is a rapid, safe and reliable method of diagnosis. H. capsulaturn has the ability to penetrate into cutaneous nerves in some of these patients.

Prevalence of HIV type 1 infection, associated clinical features and mortality among hospitalized children in Dar es Salaam, Tanzania.

The aim of this study was to determine the prevalence of HIV-1 infection, the clinical spectrum of HIV-1-associated conditions and HIV-1-associated mortality among children hospitalized in the medical paediatric wards at Muhimbili Medical Centre (MMC), Dar es Salaam, Tanzania. All children admitted to the medical paediatric wards of MMC between August 1995 and January 1996 were eligible for the study. Testing for HIV antibodies was done using 2 consecutive enzyme linked immunosorbent assays (ELISAs). ELISA-reactive samples from children aged 18 months and below were further tested by a recently developed heat-denatured p24 antigen assay. The prevalence of HIV-1 infection among the 2015 children studied was 19.2%. When present for 14 days or more, fever, cough, diarrhoea, ear discharge, oral ulcers and skin rash were all significantly more common in HIV-1-infected than in HIV-uninfected children (p < 0.001). In the multivariate analysis cough, ear discharge, oropharyngeal ulcers and skin rash were found to be the most important symptoms. Clinical signs found to be significantly associated with HIV-1 infection in the univariate analysis were wasting, stunting, hair changes, oral thrush, oropharyngeal ulcers, lymphadenopathy, lung consolidation and lung crepitations (p < 0.001). In the multivariate analysis, oral thrush, lung crepitations, cervical lymphadenopathy, wasting and inguinal lymphadenopathy were found to be the most important signs. The 3 most common diagnoses in HIV-1-infected children were acute respiratory infection (ARI) (39.4%), malnutrition (38.1%) and tuberculosis (19.3%), while in HIV-uninfected children they were malaria (47.0%), ARI (25.0%) and malnutrition (16.1%). The mortality rate was 21.4% in HIV-1-infected children and 8.4% in HIV-uninfected children (p < 0.001). In conclusion, the prevalence of HIV-1 infection among hospitalized children at the main hospital in Dar es Salaam was high and associated with high mortality. Many symptoms and signs are indicative of HIV-1 infection, but appropriate laboratory testing is required for diagnosis.

Thalidomide in Oncology: the Peril and the Promise

From 1956 through 1958, was released into the European and Canadian pharmaceutical markets as a rapid-acting, hangover-free sedative without danger of respiratory depression. In 1960, reports of peripheral neuropathy with chronic use began to surface. Growing evidence of severe infant limb defects (phocomelia) and internal organ deformities associated with maternal use of soon eclipsed this lesser concern. By late 1961, was withdrawn from the world market but not before more than 10,000 thalidomide babies were affected. Since that time, thalidomide’s ability to inhibit tumor necrosis factor-alpha (TNF-alpha) activity has been found to be beneficial for the treatment of erythema nodosum leprosum (ENL), chronic graft-vs-host disease (CGVHD), HIV-associated cachexia, oropharyngeal ulcers, and a variety of mucocutaneous disorders. Currently, is under study as an angiogenesis inhibitor, with the majority of human data in refractory multiple myeloma.

Thalidomide in Crohn's disease

Thalidomide has been shown to be effective in treating idiopathic oral and oropharyngeal apthous ulceration in HIV-1 positive patients.1 Inhibition of tumour necrosis factor-α (TNF-α) by thalidomide may be an important mechanism of action.2 TNF-α is thought to have an effect in Crohn's disease,3 and antibodies to TNF-α decrease the activity of Crohn's disease.4,5 We treated a patient with severe recurrent bleeding due to Crohn's disease with thalidomide.

Disseminated Histoplasma capsulatum infection presenting as genital ulcerations

Histoplasma capsulatum infection in adults is most often subclinical but can result in disseminated disease with weight loss, fever, hepatosplenomegaly, and oropharyngeal ulcerations. Genital ulceration as the presenting sign of the disease has been reported rarely. A 63-year-old woman presented with multiple vaginal ulcerations due to chronic disseminated H capsulatum infection. Initial diagnosis was made by Papanicolaou and Giemsa-stained vulvar smears. Ketoconazole therapy resulted in clearing of the lesions in 5 weeks. Chronic disseminated histoplasmosis is an insidious and potentially fatal disease that can present rarely as genital mucocutaneous ulcerations in women. Prompt presumptive diagnosis can be accomplished by examination of smears obtained by ulcer abrasion, permitting institution of appropriate therapy.

Safety of long-term administration of granulocyte colony-stimulating factor for severe chronic neutropenia.

When a new product with huge clinical potential explodes on the scene, the hope is that the benefits far outweigh the risks in long-term administration. After 10 years of clinical use, granulocyte colony-stimulating factor (G-CSF) has lived up to that promise so far. In the context of severe chronic neutropenia, more than 90% of patients have reaped big benefits in terms of improved quality of life and less infection, inflammation, antibiotic use, and hospitalization as well as oropharyngeal ulcers. With long-term use, toxic and adverse events have been catalogued but in general are not clinically troublesome and, aside from occasional adjustment of scheduling and dosing, seldom necessitate stopping therapy. Currently, the topic of intense focus is the phenomenon of malignant myeloid transformation in patients with congenital neutropenia who are receiving G-CSF. Data from the Severe Chronic Neutropenia International Registry have identified 23 of 249 patients with congenital neutropenia who have developed myelodysplasia or acute myelogenous leukemia (MDS/AML) giving a crude rate of about 9% with an average follow-up of 4.5 years. No cases of MDS/AML have occurred in 257 patients with cyclic or idiopathic neutropenia. A critical analysis of the incidence of transformation year by year showed a fairly uniform hazard rate of less than 2% per year, and the risk of MDS/AML after 5 to 6 years of therapy did not appear to be greater than during the first year of therapy. The transformation risk in the congenital cohort must also be viewed in the context of published reports of spontaneous myelodysplasia or acute myelogenous leukemia occurring in these patients in the pre-G-CSF era. Thus, the role of G-CSF in malignant conversion is still not clear and requires long-term vigilance and research. G-CSF is still deemed specific therapy for severe chronic neutropenia with a high margin of safety and should be the initial treatment for this family of disorders.

Nasal septal perforation and carotid cavernous aneurysm: Unusual manifestations of systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is a multisystem collagen vascular disorder defined by its clinical features and the presence of antibodies in the blood directed against one or more cell nuclei components. Typically, SLE affects the head and neck in the form of cutaneous malar erythema and oropharyngeal mucosal ulcerations. The uncommon findings of nasal septal perforations and restrictive central nervous system (CNS) aneurysmal formation as initial manifestations of SLE are detailed in this case report.

Zalcitabine-associated pharyngeal ulcers

Objective To report the development of giant pharyngeal ulcers in a series of HIV-positive patients after commencing zalcitabine therapy. We also present an algorithm for the clinical management of oral ulceration in HIV-infected individuals.Design and subjects Prospective review of patients who were prescribed zalcitabine, a nucleoside analogue reverse transcriptase inhibitor of HIV, at a single UK centre.Setting HIV out-patient clinic at the Chelsea and Westminster Hospital in London.Outcome measured Oro-pharyngeal ulceration associated with zalcitabine therapy.Results We report 3 cases of zalcitabine-associated oral toxicity which manifested as atypical giant pharyngeal ulcers. The subjects were all male homosexual non-smokers who were participating in the Medical Research Council Delta trial and had previously been randomised to receive didanosine or matching placebo in combination with zidovudine, before commencing open label zalcitabine.Conclusions Oral ulceration is a documented side-effect of zalcitabine, which may produce larger atypical lesions in some individuals. Possible predisposing factors and proposed mechanisms for zalcitabine-associated pharyngeal ulceration are discussed.

Thalidomide as Treatment of Refractory Aphthous Ulceration Related to Human Immunodeficiency Virus Infection

In recent years, thalidomide has been used for the treatment of a variety of ulcerative and immunologic conditions. Several previous reports have suggested that thalidomide therapy is beneficial for patients with aphthous ulceration related to human immunodeficiency virus (HIV) infection. We describe the use of thalidomide in 20 HIV-infected patients with oropharyngeal, esophageal, and rectal ulceration. Nineteen patients had a dramatic response to thalidomide therapy, with both subjective and objective abatement in the signs and symptoms of their ulcerative disease. The standard treatment course was 200 mg of thalidomide for 14 days (the drug was administered at night). Four patients required additional courses of treatment because symptoms recurred after thalidomide therapy was stopped. Side effects due to thalidomide included rash (5 patients), peripheral neuropathy (1 patient), and excessive fatigue (1 patient). There did not appear to be any adverse immunologic effects in thalidomide-treated patients. The mechanism of the effect of thalidomide is uncertain, although recent studies have suggested that thalidomide selectively inhibits the production of tumor necrosis factor alpha.

Hematopoietic growth factors for the treatment of severe chronic neutropenia.

Severe chronic neutropenia (SCN) is a rare but important cause of recurrent fevers, oropharyngeal ulcerations and severe infections. In three forms of SCN, i.e., congenital neutropenia (Kostmann's syndrome and related syndromes), idiopathic neutropenia (both childhood and adult), and cyclic neutropenia, it is now established that long-term treatment with the hematopoietic growth factor, recombinant human granulocyte colony stimulating factor (rHuG-CSF or Filgrastim), can elevate blood neutrophil counts to the normal range in most patients, with a concomitant reduction in infection-related events including fever, oral ulcerations, antibiotic use and symptoms of inflammation. Treatment with this growth factor causes an increase in the number and maturity of marrow cells of the neutrophilic series; other cell lines are largely unaffected. Marrow stimulation and expansion are reflected by the occurrence of bone pain early in therapy, as well as some increase in spleen size in most cases. Adverse effects of therapy are infrequent in both children and adults, and long-term treatment with daily or every-other-day s.c. injections of rHuG-CSF are well accepted. Because of the risk that some patients with chronic neutropenia may have or develop myelodysplasia and/or leukemia, careful pretreatment evaluations (blood, bone marrow and cytogenetics) and long-term observations are extremely important. An international registry for patients with SCN has been established to maintain records and further investigate these conditions.

High‐grade T‐cell lymphoma following treatment with cyclosporin A

A 47-year-old man with persistent severe oropharyngeal ulceration developed a high-grade T-cell lymphoma soon after commencing treatment with cyclosporin A. Using Southern blotting to identify T-cell beta-chain gene rearrangements, evidence of clonal restriction was found both in blood and lymph node DNA samples. Two BamH1 rearranged bands were demonstrated in both samples. In the blood a 16 Kb band predominated, with a weaker 28 kb band. In the lymph node sample this pattern was reversed. The findings suggest that a bi-clonal population of T-lymphocytes or clonal evolution of an existing T-cell monoclone had developed, and that cyclosporin contributed to the emergence of a high-grade T-cell lymphoma.

Don't forget carbenoxolone in AIDS-related oropharyngeal ulceration

Don't forget carbenoxolone in AIDS-related oropharyngeal ulceration

The gastrointestinal and hepatic manifestations of the acquired immunodeficiency syndrome.

Eighty-five patients with the acquired immunodeficiency syndrome (AIDS) were treated at Fairfield Infectious Diseases Hospital between April 1984 and June 1987. Sixty per cent of patients suffered gastrointestinal symptoms during the period of study, and in a further 15% of patients, abnormalities of the gastrointestinal tract were found incidentally. The principal manifestations were oropharyngeal ulceration, dysphagia/odynophagia, abdominal pain, diarrhoea, gastrointestinal bleeding, and perianal lesions. Opportunistic diseases involving all parts of the gastrointestinal system were encountered, the most prevalent being infections that were caused by Candida spp., cytomegalovirus, Mycobacterium avium-intracellulare and herpes simplex, and Kaposi's sarcoma. Abnormal liver-function test-results were found in 41 patients; most commonly, these were attributable to minor drug reactions, and cytomegalovirus or Myco. avium-intracellulare infection. Only one patient became jaundiced clinically. We conclude that involvement of the gastrointestinal tract is common in patients with AIDS, and that gastrointestinal lesions are an important cause of morbidity and mortality in these patients.

A EXPERIMENTAL STUDY OF OROPHARYNGEAL ULCERS DUE TO CANDIDA ALBICANS; HISTOLOGIC FINDINGS

In the previous paper the authors had reported on the histologic manifestation of deep-seated candidiasis, namely chronic ulcer ie the mouth and pharynx. In oder to extend these histitologic- findings, we had attempted to produce experimentally oropharyngeal ulcers in dogs using candida albicans. Dogs showed a strong resistance and it was necessary to use alloxan pretreatment in oder to induce ulcers using candida. In rabbits ulcers were induced easely using a strain of candida albicans which was isolated from a patient with necrotic ulcer and which was highly toxic. To induce the ulcer a suspeension of fluid containing candida must be injected submucously as super- ficial as posible. Thus the authers succeeded in producing ulcers in 45 cases.. The ulcers occured in 2 or 3 days after injection and lasted from 15 to 22 days (18 days on the average) and spontano- usly healed with scar formation. In the early stage, marked edema and slight infiltration with leucscytes occured in the tissue surrounding the injected area. Following the developement of the ulcers, at about the 10th day, fibroblasts and endothial-like cells appeared in the floor of the ulcers. The most notable finding was the formation of new blood vessels, which was so remarkable that it resembled angioma in some areas. This findigs was noted by the authors in the human biopsy specimens prviously described. Giantcells appeared following the 5th day and increased gradually until terminal stage of the ulcers. The candida on the 3-5 day were seen as spores with mycelia in the injected area and surroun- ding tissues, in the central area of which abcess formation could already be seen. By the 10-15th day spores and mycelia were no longer seen in tee granulation tissue. As control for this experiment, the authors used staphylococci and killed candida. In these experiments ulcers were produced, but they were small, inconstant in their occurrence, and healed rapidly. Their microscopic appearance was different from the foregoing findings. Thus the earlier picture may be considered as characteristic of deep seated ulcers caused by candida.

Pulmonary lesions in polyarteritis nodosa

Summary Two cases of an unusual pulmonary granulomatous lesion associated with polyarteritis nodosa are described. This type of pulmonary lesion may give rise to considerable difficulty in X-ray and clinical diagnosis. It is frequently associated with rapid onset of renal failure due to acute glomerulo-nephritis and is accompanied by oro-pharyngeal and respiratory tract ulceration. The lesions appear to result from a pan-vasculitis rather than from extra-vascular collagen degeneration. A further 3 cases are described of polyarteritis associated with pulmonary hypertension. These cases add further evidence to the view that polyarteritis nodosa is a disease of varied ˦tiology.

COLLEGE OF PHYSICIANS OF PHILADELPHIA, SECTION OF OTOLARYNGOLOGY, AND NEW YORK ACADEMY OF MEDICINE, SECTION OF OTOLARYNGOLOGY

Some Interrelationships of Modern Hematology and Otolaryngology. Dr. Thomas Fitz-Hugh Jr. A brief review is presented of the more important hematologic disorders affecting the nose, oropharynx, larynx and auditory apparatus. Lingual mucosal strophulous glossitis and dysphagia in connection with anemia respond favorably to treatment directed toward the special type of anemia and the underlying cause. In the Plummer-Vinson syndrome curious veils of mucous membrane partly obstruct the lower part of the pharynx. Here, too, treatment directed to the disease is more effective than esophageal instrumentation. Aplastic anemia is frequently associated with oropharyngeal ulceration, necrosis and bleeding, and any severe anemia may produce vertigo, tinnitus and even deafness. In leukemia, lymphosarcoma and Hodgkin's disease, not only the oral mucosa but also the tonsils and cervical nodes may present ulcerative and necrotic lesions. Epistaxis and gingival bleeding may occur, and in cases of mediastinal involvement recurrent laryngeal nerve palsy has been observed.