Inflammatory Autoimmune

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Pemphigus. Pemphigus is a rare disease that affects mucosal membranes. Although the term pemphigus may be erroneously used interchangeably with the condition bullous pemphigoid, it is a separate entity warranting a separate discussion. Pemphigus is characterized by vesicular lesions and bulla. There are multiple subtypes, with pemphigus vulgaris and pemphigus foliaceus being the most common (7). Other subtypes include pemphigus vegetans, pemphigus erythematosus, pemphigus herpetiformis, paraneoplastic pemphigus, drug-induced pemphigus, and IgA pemphigus. Although it is a disease of all ages, it typically occurs between the ages of 30 and 60. Pemphigus affects males and females equally. There is no known ethnic predilection. Its pathogenesis stems from an autoimmune mechanism in which circulating antibodies target keratinocyte cell surfaces. Cell-to-cell adhesion is disrupted and antibody complexes activate the complement cascade, creating local tissue damage. The cause of this autoimmune antibody formation is unknown, but it may be related to genetic factors or other autoimmune disorders.

Clinically, intermittent vesicles or bullae are produced that erupt, creating ulcerative lesions followed by complete resolution and remission. Nikolsky's sign is performed by the clinician rubbing unaffected areas of skin to precipitate skin sloughing. This test can be used to differentiate pemphigus from bullous pemphigoid and other blistering skin disorders. Diagnosis is made by direct immunofluorescence (DIF) microscopy of biopsies taken from affected areas or indirect immunofluorescence (IDIF) staining of a patient' s serum. Histopathological specimens demonstrate intradermal blistering in which basal layer cells are separated from one another. Immunoblotting, immunoprecipitation, and ELISA are newer tests performed to detect PD.

Treatments are targeted to reduce autoantibody production and decrease local inflammatory response. Azathioprine combined with prednisone is the most commonly used regimen, although other immunomodulating drugs have been described with some success. Antibiotics may be necessary if secondary bacterial infections are suspected in the pharynx.

Bullous Pemphigoid. Bullous pemphigoid (BP) is an autoimmune disorder consisting of cutaneous vesiculobullous lesions that appear in areas of a previous rash. Oral mucosal lesions are rare sequelae. In contrast to pemphigus, BP rarely affects persons under age 60. No racial or gender predilection has been identified. Mucosal membranes are involved in approximately 50% to 60% of cases and the oropharynx is the most common location of mucosal involvement. Diagnostic testing is similar to that for pemphigus: tissue biopsies of lesions are used for DIF staining or serum is screened for autoimmune antibodies with IDIF staining. Immunoprecipitation, immunoblotting, and ELISA are also available. Histologically, a subepidermal blister is seen. The basement membrane is thickened but remains attached to underlying connective tissues. This is the distinguishing characteristic between bullous pemphigoid and pemphigus. Treatment consists of corticosteroids when necessary; however, outbreaks are usually self-limiting and often resolve spontaneously in the absence of intervention.

Other "Blistering" Diseases. Cicatricial pemphigoid (CP), Stevens-Johnson syndrome (SJS), and epidermolysis bullosa (EB) are rare causes of pharyngitis that deserve mentioning. CP is a rare, chronic blistering disease that involves the oral mucosa in nearly all patients; lesions may extend to the oropharynx in a significant number of persons. Blisters, ulcers, erosions, and scarring are demonstrated clinically. Diagnosis is made by tissue biopsy and immunostaining microscopy. Steroids and other immunosup-pressants are used for supportive treatment.

SJS is an immune-complex-mediated hypersensitivity thought to arise from drug exposure, infection, or malignancy. Drugs associated with SJS include penicillin, sulfas, phenytoin, carbamazepine, and barbiturates. Infectious etiologies include viruses, bacteria, fungi, and protozoa. Erythematous vesicular skin lesions progress to bulla. Mucosal lesions in the oral cavity and pharynx are also common. Biopsy is used for histological identification. Treatment involves identifying, and when possible, eliminating the offending etiology. Skin and mucosal lesions generally resolve but SJS is fatal in 10% to 15% of patients. Please see Chapter 22 for a detailed discussion of SJS.

EB is a rare disorder characterized by skin and mucosal blister formation in response to mechanical trauma. The majority of cases are genetically inherited, although spontaneous cases are possible. Several forms of EB have been described, including EB simplex, EB junctional, and EB dystrophic, in order of decreasing incidence. The pathophysiology of this disease results from IgG autoantibodies targeting anchoring fibrils of collagen in the base membrane of the skin and mucosal surfaces. Shearing forces result in the rupture of bulla, leaving raw, painful submucosal exposure resulting in scar formation. Diagnosis is made by tissue biopsy. Preventative strategies are best; otherwise, treatment is supportive.

Wegener's Granulomatosis. Wegener's granulomatosis (WG) is an autoimmune disease characterized by granulomatous vasculitis in the respiratory tract and the kidneys. The vasculitis most commonly affects small arteries and veins, where it forms granulomas. Patients typically present to an otolaryngologist with sinus complaints such as drainage, pressure, and congestion. Mucosal ulceration may occur on the nasal septum and warrants a biopsy if WG is suspected. Primary involvement of the tonsils or pharynx is rare, but these areas may be inflamed secondary to post nasal drainage. The epidemiology, pathogenesis, diagnosis, treatment, and prognosis are discussed in more detail in Chapter 8.

Sarcoidosis. Sarcoidosis is an autoimmune disorder of unknown etiology. It affects multiple organ systems and is characterized by an accumulation of T-lymphocytes and mononuclear phagocytes causing noncaseating epithelioid granulomas. Its appearance in the oropharynx mimics that of infectious adenotonsillitis. Sarcoid frequently involves the epiglottis when head and neck manifestations are present. Tonsillar involvement is rare but has been described in case reports and results in tonsillar hypertrophy and erythema. The epidemiology, pathogenesis, diagnosis, treatment, and prognosis are discussed in more detail in Chapter 6.

Crohn's Disease. Crohn's disease is an inflammatory disease of the GI tract, of unknown etiology. It is characterized by mucosal ulceration that extends through all layers of the digestive tract wall and is not limited to any one area of the GI system from mouth to anus. Approximately 10% of patients with Crohn's disease have pharyngeal involvement. Most commonly, ulcerative lesions are seen on the pharyngeal walls. The epidemiology, pathogenesis, diagnosis, treatment, and prognosis are discussed in more detail in Chapter 20.

Behcet's Disease. Behcet's disease (BD) is a rare disorder of unknown etiology that affects mucocutaneous tissues, the eyes, and the genitourinary system. The classic triad of oral aphthous ulcers, uveitis, and genital ulcers is pathognomonic for BD. It may progress to involve the GI, pulmonary, renal, and central nervous systems, as well. Symptoms include malaise, fever, anorexia, and weight loss. Sore throat, dysphagia, and odynophagia are often present at acute presentation. BD is commonly misdiagnosed as pharyngitis or tonsillitis at initial presentation, resulting in a delay in appropriate treatment. Please refer to Chapter 3 for discussion of the epidemiology, pathogenesis, diagnosis, treatment, and prognosis of BD.

Extraesophageal Reflux Disease. The presence of refluxed gastric contents represents a noxious stimulant to the pharyngeal mucosa and is a frequent cause of pharyngitis. Approximately 4% to 10% of patients seen by an otolaryngologist will have extraesophageal reflux disease (EERD) (8). Symptoms include globus sensation, cough, hoarseness, dysphagia, throat clearing, and persistent sore throat. The pathophysiology of EERD may be a result of direct acid-peptic exposure to the pharynx, mediated by vagal reflux, stimulating constant throat clearing and cough, or alternatively related to cricopharyngeal dysfunction (9).

On physical exam, the mucosa is often erythematous and edematous. Posterior pharyngeal wall cobblestoning and lingual tonsillar hypertrophy are often present in pediatric patients. Edema and erythema of the posterior glottis are the most common physical exam findings and may extend to the arytenoids, aryepiglottic folds, or postcricoid area. Pseudosulcus of the true vocal cords is highly indicative of EERD. Twenty-four-hour pH dual probe esophageal monitoring is the gold standard test in the diagnosis of EERD; however, the diagnosis is often made clinically with treatment started empirically.

Treatment consists of lifestyle and diet modification, weight loss, and pharmacother-apy. Proton pump inhibitors (PPIs) are the drug of choice. Mild cases may be treated with once-a-day medications taken one hour prior to the largest meal of the day, but b.i.d. therapy is often required. Patients treated empirically with good results may consider undergoing a trial of treatment cessation to confirm the continued presence of EERD, and pharmacother-apy should be restarted if symptoms return. H2 blockers (cimetidine, famotidine, and ranitidine) are less effective but may provide benefit in some patients. Patients whose symptoms persist after adequate therapeutic trials of PPIs, with ongoing EERD proven by a pH probe study, should be referred for consideration of a fundoplication procedure. The prognosis for symptom improvement is excellent, although globus sensation symptoms may persist for months after treatment implementation. Laryngopharyngeal reflux is a distinct entity from EERD and is more likely to respond to twice-a-day PPI therapy.

Radiation. Radiation therapy, with or without chemotherapy, has become the primary treatment for oropharyngeal cancer. Primary treatment of head and neck neoplasms with radiation most commonly ranges from 60 to 70 Gy. Radiation exposure greater than 5 Gy may induce some degree of acute and/or chronic pharyngitis in nearly all patients (10). Furthermore, it affects the function of the major and minor salivary glands, reducing salivary outflow and increasing salivary viscosity.

Dysphagia and odynophagia are common symptoms, with swelling and airway obstruction occurring less frequently. Dehydration, malnutrition, and weight loss are common in severely affected patients without a secondary means of alimentation. Treatment is symptomatic. Appropriate hydration, in addition to the use of salivary substitutes and sucralfate, is recommended acutely. Salivary flow may be increased chronically with cholinergic drugs such as pilocarpine. Mouthwashes containing combinations of carafate, aluminum hydroxide, diphenhydramine, and nystatin can be useful for symptomatic relief. Superinfections with bacteria or fungi such as candida are common and should be managed accordingly. Oral or systemic glucocorticords may provide short-term relief of symptoms in severe manifestations of radiation pharyngitis.

The prognosis for symptom resolution is guarded at best, although improvement of symptoms up to one year following treatment is common. Predicting those who will experience severe symptoms from those with milder manifestations is not possible. The term "radiation recall" has been used to describe the return of symptoms in post-radiation-therapy patients who are undergoing chemotherapy. Its etiology is unknown and its treatment is similar to that of acute radiation therapy.

Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis. The symptom complex of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) is a rare but well-described cause of recurrent pharyngitis and adenotonsillitis. Intermittent high-grade fevers up to 40°C are common; these last for three to five days and recur every 26 to 30 days. It affects young children in their first decade of life, without ethnic predilection. Its etiology is not yet understood and the diagnosis is made clinically. Most patients will have a rise in IgD titers at the time of presentation. Treatment is symptomatic. Glucocorticoids are used to control intermittent fevers, aphthous ulcers, and lymphadenopathy at the onset of presentation. Tonsillectomy has been therapeutic in a small number of patients. Cimetidine, acetaminophen, ibuprofen, antibiotics, aspirin, acyclovir, and colchicines have all been used with variable success in a small number of patients (11). The disease is self-limiting and the prognosis is excellent.

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