Epidemiology

HIV is estimated to have caused 65 million human infections worldwide with 25 million deaths since the beginning of the epidemic (8), according to the World Health Organization and the Joint AIDS United Nations program data as of the end of 2005. The epidemic has different characteristics in different geographic areas, but overall remains the fourth leading cause of death, with a staggering 95 of those occurring in young adults in the developing world, mostly in sub-Saharan Africa. The epidemic...

Autoimmune Inner Ear Disease TABLE 1 Epidemiology of AIED

Year Number of patients Mean age (range) Women Men Hughes et al. (3) Moscicki et al. (4) Rauch (5) Sismanis et al. (6) Lasak et al. (7) Harris et al. (8) Broughton et al. (9) Loveman (10) Cohen etal. (11) Matteson et al. (12) 44 (8-77) 1-80 47 (4-72) 51 (27-77) 50 (5-87) Abbreviation AIED, autoimmune inner ear disease. delicate inner ear tissues. In 1982, Harris injected keyhole limpet hemocyanin (KLH) systemically and into the inner ears of guinea pigs. Measurements of antibodies within the...

Neuromuscular Disorders

Many neuromuscular diseases cause dysphagia in fact, the presenting symptom is often dysphagia. This is not surprising, considering the complex neuromuscular coordination required to execute a normal swallow. Although stroke patients develop sudden dysphagia, patients with other degenerative and nondegenerative neuromuscular diseases have a more insidious onset of dysphagia. Motor neuron diseases causing bulbar palsy or pseudobulbar palsy usually develop a progressive dysphagia and dysarthria...

Syphilis

Syphilis is a sexually transmitted disease resulting from infection by the spirochete Treponema pallidum. It is the oldest documented venereal disease, dating back to the 1400s. A more detailed discussion of syphilis appears in Chapter 15. Epidemiology. The United States has seen cyclic rises in the incidence of syphilis since nearly eliminating the disease in the late 1950s. Since 1990, the rate of primary and secondary syphilis has decreased nearly 90 to 2.5 cases per 100,000 people. Of the...

Scleroderma

Scleroderma can involve limited or widespread changes in the skin, with a host of associated internal organ manifestations. In cases of limited skin involvement, the acronym CREST has been widely used to denote the characteristic features of calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias. In cases of widespread skin involvement, the term systemic sclerosis may be used. Additional features include fibrotic lung disease, pulmonary hypertension, and...

Differential Diagnosis

In NUG, the interdental papillae undergo necrosis beginning at the tips and extending toward the crest of the dental alveolar bone. As a result of the soft-tissue necrosis, the interdental gingival papillae exhibit a punched out blunted, crateriform area covered by a fibrinonecrotic membrane (Fig. 39). The gingiva are exquisitely sensitive and often exhibit spontaneous bleeding. A fetid odor accompanies the soft-tissue necrosis. There may be associated systemic symptoms of fever, malaise, and...

Allergy and Dermatitis

There is clear evidence that otitis media with effusion is highly related to an allergic diathesis. When this converts to chronic draining otitis media, the allergic component would seem to still be relevant, although direct evidence is scant (17-19). Therefore, the surgeon must consider allergy evaluation, based on a patient history of other allergic diatheses, especially of the unified respiratory epithelium. Patients with chronic draining ear and allergic rhinitis, chronic rhinosinusitis,...

Neoplasm

Although neoplasms are not a cause of acute or chronic pharyngitis, tumors arising in the oropharynx often present with signs and symptoms that most commonly indicate an infectious etiology. Patients treated for infectious pharyngitis, who do not improve, warrant further investigation to identify a possible neoplasm. Common presenting symptoms of oropharyngeal cancer include unilateral sore throat, dysphagia, odynophagia, weight loss, and otalgia. On physical exam, an asymmetric pharyngeal mass...

Diagnosis

Computed tomography (CT) of the paranasal sinuses may show hyperdensities with small calcifications that represent allergic mucin concretions. There may be adjacent hypodense opacification of the involved sinus with air-fluid levels representing retained mucus. More advanced disease can lead to loss of adjacent bony sinus margins and even erosion of the anterior skull base due to expansile pressure. There may be extensive polyps that accompany mucosal thickening. Magnetic resonance imaging...

Info

FIGURE 5 The technique of anterior ethmoid artery ligation via a Lynch incision. intractable epistaxis. Since that time, multiple reports utilizing this basic technique with various catheters and embolic materials have been reported. The use of this technique is seen in many areas including arteriovenous malformations, pseudoaneurysms, bleeding varices, shrinking of fibroids, and vascular tumors of the head and neck. Diagnostic angiography with embolization is commonly performed under local...

References

Ponikau JU, Sherris DA, Kern EB, et al. The diagnosis and incidence of allergic fungal sinusitis. Mayo Clin Proc 1999 74(9) 877-884. 2. Branovan DI. Pathophysiology of rhinosinusitis. In Rice DH, Schaefer SD, eds. Endoscopic Paranasal Sinus Surgery. 3rd ed. Philadelphia, PA Lippincott Williams & Wilkins, 2004 53-68. 3. Furguson BJ, Johnson JT. Infectious causes of rhinosinusitis. In Cummings CW, Flint PW, Haughey BH, eds. Cummings Otolaryngology-Head and Neck Surgery. Vol. 2. 4th ed....

Allergic rhinosinusitis

Allergic rhinosinusitis is a disorder expressed in the tissues of the nares and upper airway, manifested by local edema and inflammatory infiltration of the submucosa and mucosa, and associated with increased secretion of mucoid materials with accompanying inflammatory leukocytes. These processes lead to obstruction of airflow and of mucus drainage, and, therefore, may be complicated by postobstructive infectious complications. By definition, allergic disease is dependent on the host generation...

Definitions

Fungal sinusitis, when used loosely, can be a misleading term. It actually refers to a spectrum of fungal-associated diseases of the nose and paranasal sinuses, each with a unique presentation and management implications (Table 1). When communicating with TABLE 1 Fungal-Associated Diseases of the Nose and Paranasal Sinuses ( is used to indicate controversy) TABLE 1 Fungal-Associated Diseases of the Nose and Paranasal Sinuses ( is used to indicate controversy) topical nasal steroids, antifungals...

Infection Fungal

Candida albicans is a common organism found in the oral cavity flora that causes candidiasis in certain clinical situations. Candida has a capsule and forms true hyphae and pseudohyphae. It adheres to mucosal surfaces and is capable of superficial mucosal invasion. Factors that contribute to oral candida proliferation include uncontrolled diabetes mellitus, antibiotic therapy, and any condition that causes immunosuppression. Clinical features of candidiasis include white, cheesy...

Case Example

A 51-year-old woman was transferred from a small community emergency department for subspecialty evaluation. The patient had presented to that facility the previous night complaining of headache and fever for three days with a sudden loss of vision in her left eye. During our evaluation, the patient reported that one week earlier, she fell, striking the left side of her face and sustaining several small abrasions. She denied other facial trauma. Further history revealed a general malaise and...

Leukemia

AML is treated with intensive chemotherapy. The standard regimen is an anthracycline plus Ara-C (the three plus seven regimen). Following remission, consolidation, often with high-dose ARA-C, is administered. Depending on risk stratification, allogeneic or autologous transplant may be appropriate in selected patients. Acute promyelocytic leukemia (FAB M3) is treated with ATRA in addition to chemotherapy. Induction is followed by consolidation and then a maintenance regimen. There is no benefit...

Angioedema

Angioedema is an uncommon and usually self-limited swelling of the deep dermis. It can, however, be complicated by potentially serious adverse consequences, including death. Although IgE-mediated mechanisms are not the major cause of angioedema, they are among the few that can be clearly identified, and thus this topic will be discussed here. Angioedema occurs together with urticaria approximately 85 of the time in 15 of instances, it occurs alone. Angioedema (Fig. 1) is the abrupt and...

Treatment Lymphoma

The treatment of lymphoma depends primarily on the histologic subtype and the stage. HL is treated with chemotherapy, or for more limited-stage disease, combination chemotherapy and radiotherapy. In this situation, the dose of both radiation and chemotherapy is less than if either modality is used alone. The advantage is a reduction in toxicity of each modality used alone. In some cases of very limited disease without B symptoms, radiation alone may be used. The current standard chemotherapy...

Loco Regional Disease Nodal Assessment

After initial pathological assessment by punch or excisional biopsy, the next step is treatment of the primary site and staging of the regional lymph nodes. Treatment for the primary tumor consists of wide local excision. Local recurrence can be as high as 40 for primary melanomas that are not reexcised after biopsy (3). Melanoma-in-situ is treated by reexcision with at least 0.5-cm margins around the primary lesion or biopsy scar. When primary invasive melanomas are < 1 mm thick or Clark's...

Infection Viral

Adenovirus is the most common cause of viral pharyngitis. It is a double-stranded DNA virus. Serotypes 3,4, and 7 are frequently associated with viral pharyngitis. It is transmitted by either respiratory droplets or direct contact. School-aged children are most commonly affected. The classic presentation includes fever, sore throat, coryza, and red eyes. Adenovirus is cytolytic to the epithelial cells it invades and induces a localized inflammatory response in the surrounding...

Lymphoma Involving Specific Head and Neck Sites

Lymphoma Neck

Lymphoma involving the thyroid gland is rare, accounting for only 2 to 3 of all cases of lymphoma and less than 10 of thyroid malignancies. Women are affected more frequently than men are by a ratio of 2.7 1. The median age at presentation is over 60 years. The most common subtypes are DLBCL and follicular Grade-3 lymphoma, accounting for at least 80 of cases. A rare but interesting subtype is marginal zone B-cell lymphoma, which arises from MALT. In many cases, this lymphoma...

Malignant Melanoma

Morton John Wayne Cancer Institute at Saint John's Health Center, Santa Monica, California, U.S.A. Division of Otolaryngology, Head and Neck Oncology Program, Moores Cancer Center, University of California, San Diego, California, U.S.A. Clinical Manifestations 274 Loco-Regional Disease (Nodal Assessment) 278 Complications and Prognosis 281 Mucosal Melanomas Special Considerations 281

Idiopathic Diseases Sarcoidosis

Sarcoidosis is a chronic, idiopathic, granulomatous disease with a strong predilection for African American and Puerto Rican women in their third or fourth decades of life. Patients most often present with pulmonary symptoms, including shortness of breath, hemoptysis, nonproductive cough, and dyspnea on exertion however, the spectrum of patient presentation can range from asymptomatic to involvement of any organ system. When neurologic involvement is present, the term neurosarcoidosis has been...

Amyloidosis

Amyloidosis is a disease resulting from abnormal protein deposits in extracellular tissue. The disease can be localized or systemic and can be further subclassified as primary, secondary, and familial. In primary amyloidosis, the protein fibrils form without a known cause. Secondary amyloidosis results from multiple myeloma or other chronic diseases that generate an abnormal amount of protein breakdown. Familial amyloidosis refers to a hereditary version of the disorder. Presentation is...

Fungal Infections in the Immunocompromised Host

For the most part, the Mucorales are considered to be opportunistic pathogens. They require a breakdown in the immune defenses, particularly disease processes that lead to neutropenia or neutrophil dysfunction. Although neutrophil dysfunction induced by ketoacidosis underlies the majority of cases of human zygomycosis, neutropenia induced by bone marrow suppression during chemotherapy or immunosuppression induced following transplantation is causing a growing proportion of cases. Specifically,...

Rhinocerebral Mucormycosis

RCM represents one-third to one-half of all cases of Zygomycosis. The process originates in the nose and paranasal sinuses following inspiration of fungal spores. It is estimated that 70 of the cases of rhinocerebral zygomycosis occur in the setting of DKA (7). Disease starts with symptoms consistent with sinusitis. Low-grade fever, dull sinus pain, drainage, and soft-tissue swelling are initially seen, followed in a few days by double vision, increasing fever, and obtundation. Examination...

Systemic Sclerosis

Systemic sclerosis is a disorder of the small arteries, resulting in proliferation of fibrosis affecting the skin and multiple end organs. There are two subtypes of systemic sclerosis, diffuse and limited cutaneous. Diffuse cutaneous systemic sclerosis is characterized by rapid progression with skin involvement proximal to the elbows and knees. There is usually the early onset of Raynaud's phenomenon and early visceral involvement. Limited cutaneous systemic sclerosis is characterized by a...

Stomatitis

Herpangina

Stomatitis refers to an inflammatory process involving the mucous membrane of the mouth that may manifest itself through a variety of signs and symptoms including erythema, vesiculation, bulla formation, desquamation, sloughing, ulceration, pseudomembrane formation, and associated discomfort. Stomatitis may arise due to factors that may be of either local, isolated conditions or of systemic origin. For example, a solitary oral ulcer with a history of a recurrent pattern may be classified as...

Complications And Prognosis

Uveitis has numerous complications, which often cause visual loss. Treatment of uveitis can also contribute to these complications. Glaucoma may arise from a pressure response TABLE 2 Immunosuppressive Therapy in Uveitis, Cogan's Syndrome, and Sarcoidosis TABLE 2 Immunosuppressive Therapy in Uveitis, Cogan's Syndrome, and Sarcoidosis Retinopathy, nausea, rare neuromyopathy Nausea, mouth sores, hair loss, cytopenias, liver test abnormalities, rash, liver fibrosis, rare pneumonitis Nausea,...

Diagnosis And Treatment The Infectious Etiologies

Both the external ear and the middle ear are susceptible to infection and both can present with otorrhea. External-ear infections (acute otitis externa) are most often caused by irritation to the ear, either from manipulation (fingers, Q-tips, etc.) or from environmental factors (water, debris, etc.). External-ear infections will present with ear pain and drainage. In bacterial otitis externa, the discharge is typically purulent. The main pathogens are...

Mycobacterial Infections

Tuberculosis (TB, from Mycobacterium tuberculosis and or Mycobacterium bovis) is a major cause of morbidity and mortality in HIV disease. It usually presents as reactivation of a pulmonary primary focus, with a risk of 7 to 10 per year for HIV-infected persons regardless of CD4 lymphocyte count, versus 10 per lifetime for HIV-negative persons. There can be involvement of the lungs, central nervous system (CNS), or other organs, with rhinosinusitis, diffuse or localized (scrofula)...

Infectious Diseases

Hearing loss is an uncommon presenting symptom of a systemic infectious disease but may develop during the course of several viral and bacterial infections. Pathogens may infect the inner ear and auditory pathways in utero, resulting in congenital syndromes that often include deafness, or infection may be acquired after birth. As the fluids of the inner ear are not easily accessible for sampling, the implication of viral pathogens as the causes of sensorineural hearing loss has often depended...

Neck Vsh Investigation

Rhabdomyosarcoma is classified into three cell types embryonal, alveolar (which has botryoid and spindle cell variants), and pleomorphic. The embryonal subtype accounts for 60 to 70 of childhood cases and is the most common subtype. It is also the most common subtype presenting in the head and neck (20-22). Rhabdomyosarcomas are malignant tumors of skeletal muscle. They are part of the larger group of soft-tissue sarcomas. Sarcomas of bony origin are classified separately. Like most malignant...

Pernicious Anemia

Pernicious Anemia Parietal Cells

Pernicious anemia is associated with atrophic gastritis. The marked loss of gastric parietal cells results in a deficiency of intrinsic factor, which is required to transport Vitamin B12 across the intestinal mucosa. The resulting deficiency of Vitamin B12, which is necessary FIGURE 43 Pernicious anemia. This elderly female complained of a painful tongue. The mucosa of the anterior portion of the tongue was severely atrophic and totally devoid of lingual papillae. Posteriorly, the mucosa was...

Head and Neck

Oral ulcerations represent the most common finding of disease and are seen in over 95 of patients with Adamantiades-Behget's. They are required for diagnosis (Table 1), although some argue that in rare cases, oral ulcers need not be present for the diagnosis to be made. The oral ulcerations seen in Adamantiades-Behget' s may be present on the tongue, lips, gingival surfaces, buccal mucosa, soft palate, or posterior pharynx (Fig. 1). In general, they are shallow and painful, resolving in...

Treatment

Treatment of SS is complicated by its unique multiorgan involvement. Timely organ-specific treatment can effectively increase patient comfort and reduce potential damage to the corneas and teeth. Systemic complications of SS are secondary to autoimmune processes and potentially life-threatening, and are discussed below. Ocular management of KCS has three goals (i) replacing the aqueous tear film and restoring normal tear function through the use of preservative-free artificial tears (ii)...

Systemic Lupus Erythematosus

FIGURE 2 Rheumatoid nodule on left vocal fold with reactive changes on right vocal fold. SLE is a common autoimmune connective-tissue disease affecting 1 in 1000. It is much more prevalent in young females, with a female-to-male incidence of 9 1. It affects many organ systems. Skin rash is a very common presentation, typically appearing in the malar areas following sun exposure. Oral ulcerations develop in 40 of patients. Other systemic manifestations include myocarditis, nephritis,...

FIGURE 16 Oral hairy leukoplakia associated with EBV Source From

Leukoplakia

(PCR) for CMV viral load in serum can be used, but the clinical utility of the test is not yet clear. Whether treatment is beneficial for oral ulcers caused by CMV is not clear. Ulcerative esophagitis-causing symptoms can be treated with IV antivirals such as ganciclovir, foscarnet, or cidofovir, followed by oral maintenance, usually with oral valganciclovir. Epstein-Barr Virus and Oral Hairy Leukoplakia. OHL has been encountered since the early epidemic and has been strongly linked to...

TABLE 5 Extraglandular Manifestations in Primary SS

Hashimoto's thyroiditis Graves' disease Interstitial nephritis renal tubular acidosis Autoimmune hepatitis primary biliary cirrhosis Non-Hodgkin's lymphoma (extraglandular or glandular) Abbreviation SS, Sjogren's syndrome. ocular surface of both eyes is observed through the slit lamp using the cobalt blue filter. The amount of time between the last blink and the break-up of the fluorescein-stained precorneal tear film is determined by noting the first blue spot that appears on the surface of...

Crohns Disease and Ulcerative Colitis

Department of Medicine, Division of Gastroenterology, Inflammatory Bowel Disease Center, Mount Sinai School of Medicine, New York, New York, U.S.A. Clinical Manifestations 299 Crohn's disease (CD) and ulcerative colitis, collectively part of the inflammatory bowel diseases (IBD), are both diseases of the gastrointestinal tract that arise in genetically susceptible individuals. Both CD and ulcerative colitis, though clinically different entities, are thought to be part of a spectrum of diseases...

Cutaneous Bullous Diseases

Many cutaneous disorders have associated esophageal involvement. The more common of these disorders include epidermolysis bullosa, cicatricial pemphigoid (CP), lichen planus, and pemphigus vulgaris. Epidermolysis bullosa is a relatively rare cutaneous disease mediated by circulating IgG antibodies directed against type VII collagen. Clinically, patients develop intradermal blistering lesions with scarring at sites of trauma (hands and feet are most common). The proximal third of the esophagus,...

Mucous Membrane Pemphigoid

MMP is a chronic autoimmune mucocutaneous disease in which autoantibodies directed at structural proteins of the hemidesmosome destroy the epithelial-connective tissue attachment at the level of the basement membrane, producing a subepithelial separation (24,25). The protein targets of the autoantibodies include BP-1, BP-2, and laminin-5 (epiligrin), all components of the epithelial anchoring apparatus. MMP is a generalized term for a group of closely related disease processes (26). The term...

FIGURE 1 Linear gingival erythema Source Courtesy of the International AIDS SocietyUSA From Refs 3 4

Also known as HIV gingivitis, a fiery red linear discoloration of the gingival margin can be seen in HIV disease, even without significant plaque formation (Fig. 1). Patients complain of spontaneous bleeding or are asymptomatic. Referral to an HIV dental specialist is recommended and usually involves debridement, local care, and systemic antibiotics. Necrotizing ulcerative periodontitis and stomatitis. Rarely encountered in the asymptomatic HIV-infected individual,...

Cytologic Testing

Demonstration of fungal elements from cytologic preparations (i.e., sputa, inflammatory fluid aspirates from abscesses or sinusitis infection, and genitourinary and gynecologic specimens) may be difficult, due to the difficulty in extracting fungal elements from invaded tissues (8). Cultures of blood and cerebrospinal fluid (CSF) are negative. CSF, if inadvertently examined, may show an increased opening pressure, modest neutrophilic pleocytosis, normal or slightly elevated protein levels, or...

F

FIGURE 4 Erythematous form of oral candidiasis of the palate. Source Courtesy of the International AIDS Society-U.S.A. From Refs. 3, 4, 11. FIGURE 4 Erythematous form of oral candidiasis of the palate. Source Courtesy of the International AIDS Society-U.S.A. From Refs. 3, 4, 11. FIGURE 6 Erythematous candidiasis on the dorsal tongue surface. Source Courtesy of the International AIDS Society-U.S. A. From Refs. 3, 4, 11. FIGURE 6 Erythematous candidiasis on the dorsal tongue surface. Source...