Disclaimer: The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature. Necrotizing Intranasal Lesions The differential diagnosis of necrotizing intranasal lesions is extensive. Worldwide, trauma and infection are by far the most common causes of these lesions. Excluding trauma, the differential diagnosis of necrotizing nasal lesions can be subdivided into four categories: infectious, vasculitic and autoimmune, idiopathic, and neoplastic. With a few exceptions, common to all of these categories is the presence of granulomatous inflammation. INFECTIOUS: A wide variety of infectious diseases can lead to necrotizing lesions of the nose. Most of these are caused by mycobacteria, fungi, or spirochetes. Although tuberculosis of the upper aerodigestive tract is now relatively uncommon in the United States, with the incidence of pulmonary tuberculosis on the rise, TB should be kept in mind when evaluating a patient with a necrotizing nasal lesion. Tuberculosis of the nose usually arises secondarily from pulmonary disease, but it may occur as a primary lesion. Patients generally present with nasal obstruction, mucopurulent rhinorrhea, and epistaxis. Classically, the lesions occur on the septum and are erythematous and ulcerated. Septal perforations are not uncommon. The diagnosis is confirmed by staining for acid-fast bacilli and through culture, and biopsy may reveal the presence of caseating granulomas. Leprosy, caused by Mycobacterium leprae, is endemic in Texas and Louisiana. Nasal involvement in the lepromatous form of leprosy is universal. Symptoms include obstruction, bleeding, crusting, sensory loss of the nose and face, and hyposmia. Early lesions are characterized by nodules and plaques with pale, yellowish thickening of the mucosa. The lesions most frequently involve the septum and inferior turbinates. Septal perforation with saddle nose deformity and atrophic rhinitis are late sequelae of the infection. The histologic appearance of leprosy is unique, characterized by the presence of large, foamy macrophages in the background of a chronic inflammatory cell infiltrate. Acid-fast staining shows abundant acid-fast bacilli within these macrophages. Once the diagnosis is established, standard treatment consists of combination therapy with dapsone and rifampin. However, these drugs are somewhat costly and toxic, and recent reports have shown that minocycline is very effective in curing this disease. Several fungal infections can be implicated in causing necrotizing, granulomatous lesions of the nose. Nasal lesions in histoplasmosis, coccidiodomycosis, blastomycosis, and cryptococcosis are almost invariably the result of systemic spread from a primary pulmonary infection. Skin and complement fixation testing can be helpful in making the diagnosis, but identifying the organism through appropriate stains or by growing it in culture is essential. Because the disease is usually disseminated by the time lesions appear in the nose, diagnosis and prompt treatment are crucial since untreated disease is frequently fatal. Amphotericin B is the treatment of choice. Rhinosporidiosis is a fungal infection that is quite prevalent in India. By far the most common site of infection is the nasal mucosa, targeting the nasal septum as well as the inferior turbinates in the majority of cases. Red, friable, polypoid masses are characteristically seen. Only multiple surgical excisions have been effective in controlling this disease. Histologically, multiple sporangia are seen, which contain thousands of rhinosporidial spores. Invasive fungal infections of the nose such as mucormycosis and aspergillosis can be devastating. Mucormycosis is a fulminant opportunistic infection that, if not treated with aggressive surgical debridement and amphotericin, can be rapidly fatal. The infection usually arises in the nose and ethmoid sinuses, and the patient often presents with headache, fever, obtundation, and a serosanguinous nasal discharge. Pale or necrotic mucosa may be seen on physical examination. Extension into the orbit through invasion and subsequent thrombosis of blood vessels occurs rapidly, leading to panophthalmoplegia and cavernous sinus thrombosis. The diagnosis is made on biopsy, demonstrating the presence of branching, nonseptate hyphae. Another infectious cause of ulcerative nasal lesions is syphilis. Nasal disease secondary to syphilis can occur at any age, including in the neonate. Primary, secondary, and tertiary syphilis can all cause intranasal disease. Congenital syphilis has severe nasal manifestations characterized by marked rhinitis, with nasal obstruction and a bloody, mucopurulent discharge. The ulcerative lesions can lead to chondritis, osteitis, and a saddle nose deformity. The diagnosis of nasal syphilis can be confirmed through identification of the organism under darkfield examination and through serologic testing (RPR, FTA-ABS, VDRL). Treatment is with penicillin. Of the bacterial causes of necrotizing nasal lesions, rhinoscleroma is of particular interest in this part of the country. It is endemic to Mexico, and Central and South America and is caused by Klebsiella rhinoscleromatis. Patients usually present with obstruction, rhinorrhea, epistaxis, and nasal deformity. Examination shows erythematous granular or nodular lesions covered with crusts. Untreated, the infection leads to ulceration, fibrosis, and scarring. Because of its tumor-like appearance and local extension, malignancy is often suspected. The diagnosis is made by biopsy. The classic histopathologic picture consists of the presence of large vacuolated histiocytes called Mikulicz's cells, and an infiltrate of plasma cells and lymphoctes. Russell bodies, which are degenerating plasma cells, may also be seen. On Warthin-Starry staining, gram negative bacilli can be seen within the Mikulicz's cells. Treatment is with ampicillin or other antibiotics that are effective against gram negative organisms. VASCULITIC/AUTOIMMUNE: Wegener's granulomatosis is a distinct clinical entity that is classically defined by the presence of a necrotizing granulomatous vasculitis involving both the upper and lower respiratory tracts, and a focal necrotizing glomerulonephritis. A systemic small vessel vasculitis involving many other organ systems may also be present. It represents a clinical continuum, ranging from patients who have disease limited to the upper airway to those who have multi-organ involvement. However, the nasal cavity is involved in some way in more than 90% of cases. Even when the disease appears to be confined to the nose, the patients frequently have systemic signs and symptoms that are out of proportion to the degree of nasal disease. Typical nasal findings include nasal obstruction and a serosanguinous nasal discharge, with diffuse destruction of the mucosa and the presence of foul-smelling crusts. The turbinates and nasal septum are preferentially affected. Progression of the necrotizing process leads to complete destruction of the septal cartilage, nasal collapse, and a saddle nose deformity. The diagnosis can be made through histologic examination and the presence of anticytoplasmic antibodies (ACPA) in the serum. These antibodies are unique to patients with active Wegener's. Unfortunately, these antibodies cannot be detected in the limited forms of the disease. Histologically, granulomatous inflammation (palisading histiocytes, multinucleated giant cells) and focal necrosis are seen. Well-formed granulomas, such as those seen in tuberculosis and sarcoidosis, are never present. The vasculitis is present and usually striking. The mainstay of therapy is cyclophosphamide, usually combined with a limited course of corticosteroids. Relapsing polychondritis is felt to be an autoimmune disease that is characterized by recurrent inflammation with destruction of cartilaginous and other connective tissue structures. The cartilage of the ears, nose, and airway are the most frequently involved. To make the diagnosis, three diagnostic criteria must be fulfilled: clinical evidence of recurrent inflammation of three or more cartilaginous structures; involvement of the eye; and histologic evidence of focal inflammatory destruction of cartilage. Involvement of the nose can lead to a saddle nose deformity, but it is the involvement of the airway cartilage that can be fatal. Treatment usually consists of corticosteroid therapy, but other immunosuppressive agents such as cyclosporin have also been used with some benefit. Churg-Strauss syndrome, or allergic granulomatosis and angiitis, is a fascinating clinical syndrome that is characterized by the presence of severe asthma, fever, and peripheral eosinophilia, along with signs and symptoms of vasculitis in other organ systems. What is not well-appreciated is that this syndrome has significant head and neck manifestations. In one study, 69% of the patients had nasal involvement. The nasal findings included nasal obstruction with rhinorrhea, extensive intranasal crusting, polyps, and septal perforations. The nasal mucosa appears granular and friable, similar to what is seen in Wegener's granulomatosis. The pathologic findings consist of an extensive eosinophilic inflammatory infiltrate with extravascular granulomas. Vasculitis is also seen. As in Wegener's, corticosteroids and cyclophosphamide are the mainstay of therapy. IDIOPATHIC: Sarcoidosis is a multisystemic granulomatous disease of unknown etiology that most commonly strikes in young adulthood and is more frequently seen in the black population. Every organ system can be involved in the granulomatous process. The diagnosis is made based on clinical and radiographic findings such as hilar adenopathy, as well as histologic evidence of widespread, noncaseating granulomas. Elevated serum angiotensin-converting enzyme levels, hypercalcemia, and impaired, delayed hypersensitivity responses also contribute to establishing the diagnosis. Five percent of patients with sarcoidosis have involvement of the upper respiratory tract. When this occurs, the nasal mucosa is always involved, while the mucosa of the larynx and pharynx is involved much less frequently. Early nasal lesions appear as small yellow mucosal nodules, and the surrounding mucosa is edematous and hyperemic. These nodules coalesce, leading to mucosal erosions and crusting. Septal perforation can occur, but it is usually as a result of trauma, such as from a septoplasty to relieve obstruction in unrecognized nasal sarcoidosis. Lupus pernio, an extremely disfiguring, violaceous, ulcerating skin lesion that involves the nose, cheeks, and ears, is closely associated with the presence of nasal sarcoidosis. Treatment is with corticosteroids. Idiopathic midline destructive disease is the ultimate diagnosis of exclusion for necrotizing intranasal lesions. It is a very rare disease that is defined as the progressive and relentless inflammatory destruction of the sinonasal tract and palate, with erosion of contiguous structures. The lesion is localized to the midfacial region, and the patients have no systemic signs or symptoms or evidence of extramidfacial involvement. It is seen most frequently in females and can occur at any age. Histologically, only nonspecific acute and chronic inflammation with necrosis is seen. Only high-dose local irradiation has been shown to control this disease NEOPLASTIC: A variety of malignant neoplasms can cause ulcerative lesions of the nasal cavity, including squamous cell carcinoma, adenocarcinoma, adenoid cystic carcinoma, and lymphomas. Intranasal lymphomas are of particular interest because of recent advances in their classification. Within the past few years, many studies have shown that polymorphic reticulosis (formerly called lethal midline granuloma) is, in fact, a peripheral T-cell lymphoma. In addition, the majority of other malignant lymphomas of the nasal cavity have been found to express the phenotypic markers of T-lymphocytes as well. This is in contrast to other head and neck extranodal non-Hodgkin's lymphomas, such as those that involve Waldeyer's ring, which are primarily of B-cell origin. These nasal T-cell lymphomas present similarly, with nasal obstruction, epistaxis, and serosanguinous discharge. Necrosis and destruction of the nasal cartilages and bone is seen, occasionally with local infiltration into the adjacent facial areas. All of these lesions, including polymorphic reticulosis, have the capacity to spread to regional lymph nodes, the lungs, and other distant sites. Histologically, polymorphic reticulosis is classically characterized by the presence of atypical lymphocytes mixed with inflammatory cells such as plasma cells, eosinophils, and histiocytes. These cells may have an angiocentric and angioinvasive growth pattern. The histiocytes do not form true granulomas. It has been postulated that the adjacent inflammatory cells serve to keep the disease localized, leading to a better prognosis. There are two schools of thought regarding treatment: those who advocate radiotherapy alone, and those who advocate combination radiotherapy and chemotherapy. Finally, cocaine abuse can cause extensive midline nasal destruction, ranging from simple septal perforations to alar necrosis, saddle deformities, and massive, progressive osteocartilaginous necrosis of the sinonasal tract and palate. It may mimic idiopathic midline destructive disease. These lesions should be treated conservatively with debridement of necrotic tissues, antibiotics, saline irrigations, and abstinence from cocaine. Radiotherapy should only be considered if all other measures fail. Reconstruction of the resultant nasal deformities should be undertaken only after the disease has stabilized and abstinence from cocaine can be documented. In conclusion, the differential diagnosis of necrotizing intranasal lesions is extensive, and accurate diagnosis is essential for determining the appropriate treatment. A thorough history and physical examination are mandatory, specifically looking for signs and symptoms of systemic disease. It is important to elicit information regarding trauma or septal surgery, recent travel, medications, and if possible, about cocaine abuse. At the very least, laboratory analysis should include a CBC with differential, a sedimentation rate, and VDRL and FTA-ABS tests. Also, ANA, rheumatoid factor, angiotensin-converting enzyme, and anticytoplasmic antibody levels may be helpful. Depending on the clinical picture, skin testing and/or complement fixation titers for tuberculosis, histoplasmosis, coccidiodomycosis, and blastomycosis should be obtained. Biopsies should also be performed, and special stains and cultures for acid-fast bacilli, fungi, spirochetes, and rhinoscleroma should be performed. Immunohistochemical studies may also be indicated. With appropriate therapy, a cure, as well as prevention of significant cosmetic deformities, can often be achieved. Case Presentation A 54-year-old white male who underwent a septorhinoplasty in 1972 for a traumatic nasal deformity. A satisfactory result was obtained and he experienced no complications from the surgery. However, in 1975, he began to use street-grade cocaine intranasally. His cocaine abuse increased in the mid 1980s, and he developed persistent, clear rhinorrhea. In 1990, he developed nasal obstruction with extensive nasal crusting. A large nasal septal perforation was diagnosed, and he was started on saline irrigations and advised to discontinue cocaine use. He underwent successful drug rehabilitation and has been completely abstinent from cocaine for one and one-half years. He presented to clinic for reevaluation and repair of the nasal septal defect. His past medical history is otherwise unremarkable. Specifically, there is no history of diabetes mellitus, autoimmune or collagen vascular diseases, or use of other topical intranasal vasoconstrictors. 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