Info

in large neurons.

Eyes

For removal and specimen preparation, see p. 85.

1. Landing BH, Ang SM, Villarreal-Engelhardt G, Donnell GN. Galactosemia: clinical and pathologic features, tissue staining patterns with labeled galactose- and galactosamine-binding lectins, and possible loci of nonenzymatic galactosylation. Persp Pediatr Pathol 1993;17:99-124.

2. Jevon GP, Dimmick JE. Histopathologic approach to metabolic liver disease: Part 2. Pediatr Dev Pathol 1998;1:261-269.

Ganglioneuroma (See "Tumor of the peripheral nerves.") Gangliosidosis

Synonyms and Related Terms: Activator protein deficiency (type AB); beta galactosidase deficiency; GM! gangliosidosis, infantile, type 1 (with visceral involvement); late infantile, type 2; adult, type 3; GM2 gangliosidosis with infantile, late infantile, and adult forms; hexosaminidase A deficiency (type B); hexosamine A and B deficiency (type 0); lysosomal disorder (1); Tay Sachs disease; Sandhoff's disease.

From: Handbook of Autopsy Practice, 3rd Ed. Edited by: J. Ludwig © Humana Press Inc., Totowa, NJ

Organs and Tissues

Procedures

Possible or Expected Findings

External examination

Fascia lata (see also "Liver and spleen")

Liver and spleen

Other organs

Brain and spinal cord

Placenta

Obtain routine body measurements and weight. Photograph all abnormalities.

Fascia lata should be collected using aseptic technique for tissue culture for biochemical studies (see Chapter 10) and electron microscopic examination (p. 132).

Record weights. See also below under "Brain and spinal cord." Obtain tissue for tissue culture for assay of enzyme deficiency. Enzyme assay can be performed on fresh or frozen liver tissue. If evidence of other organ involvement (heart, kidney) is present, follow procedures described below under "Brain and spinal cord." For removal and specimen preparation, see pp. 66 and 70, respectively. Request LFB/PAS and/or Sudan Black (on frozen tissue) stains (p. 172). Submit samples for electron microscopic study (p. 132). Enzyme assay can be performed on fresh or frozen brain tissue. If analysis of lipids is intended, place fresh tissue in liquid nitrogen and store at -90°C until lipids can be extracted and analyzed— for instance, by thin-layer chromatography. Weigh, snap-freeze a portion, and submit portion for histologic study.

Hydrops fetalis; coarse facies; macroglossia; depressed, broad nose; large ears; frontal bossing; gingival hypertrophy; squat hands and feet; flexor contractures; ascites; hernias. Cultured fibroblasts can be used for enzyme assay. "Empty" vacuoles in lymphocytes by EM.

Hepatosplenomegaly; accumulation of PAS and Sudan Black positive material (ganglioside) in histiocytes (1).

Cerebral atrophy; neurons distended by lipid (ganglioside); disintegration of neurons and reactive phagocytosis and astrocytosis; fibrillogranular inclusions in fibroblasts and endothelial cells (2-4).

Vacuolated syncytiotrophoblast.

References

1. Jevon GP, Dimmick JE. Histopathologic approach to metabolic liver disease: Part 2. Pediatr Dev Pathol 1998;1:261-269.

2. Lake B. Lysosomal and peroxisomal disorders. In: Greenfield's Neuropathology, vol. 1. Graham BI, Lantos PL, eds. Arnold, New York, 1997, pp. 658-668.

3. Rapola J, Lysosomal storage diseases in adults. Pathol Res Pract 1994; 190:759-766.

4. Suzuki K. Neuropathology of late onset gangliosidosis. A review. Dev Neurosci 1991;13:205-210.

Gangrene, Gas

Synonym: Clostridial infection.

NOTE: (1) Collect all tissues that appear to be infected. (2) Request aerobic and anaerobic bacterial cultures. (3) Request Gram stain (p. 172). Inflammation may be minimal or absent. (4) No special precautions are indicated. (5) Serologic studies are not indicated. (6) This is not a reportable disease.

Organs and Tissues

Procedures

Possible or Expected Findings

External examination

Skeletal muscles

Record appearance of wounds or of other possibly infected lesions. If foreign bodies are present, record their nature and location (roent-genograms may be helpful). Prepare smears of wounds and request Gram stain (p. 172). Prepare roentgenograms of suspected areas; palpate abnormal areas and record extent of crepitation; submit samples of grossly involved and of uninvolved skeletal muscle for bacterio-logic and histologic study (see above under "Note").

Edema surrounding wound; gas bubbles in a discharge; foul odor of the wound; loose blebs containing serosanguinous fluid.

Muscle necrosis (Clostridial myonecrosis) and accumulation of gas; little leukocytic infiltration.

Organs and Tissues Procedures Possible or Expected Findings

Other organs Procedures depend on expected findings or Pneumonia;* empyema;* cholecystitis;*

grossly identified abnormalities as listed in uterine infection (postabortal or postpartum). right-hand column. See also above under "Note" and under "Skeletal muscles."

Gastroenteritis, Eosinophilic

Organs and Tissues

Procedures

Possible or Expected Findings

Abdomen

Esophagus and gastrointestinal tract

Pancreas and bile ducts Other organs

Record volume of peritoneal exudate and prepare smears of sediment.

Record location of and photograph involved segments; state distance of these areas from anatomic landmarks. Leave esophagus attached to stomach. For in situ fixation of small bowel, see p. 54. Record thickness of wall, width of lumen, and length of involved segments. Submit samples of all grossly involved and of grossly uninvolved segments for histologic study. Request azure-eosin or Giemsa stain (p. 172).

Submit samples for histologic study. Request azure-eosin or Giemsa stain (p. 172). Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column.

Chronic peritonitis and ascites in cases with serosal involvement of the affected stomach or gut segments.

Eosinophilic esophagitis may occur (1). Presence of infiltrates most common in antrum of stomach with thickening of the pylorus. Ulcers may be found in antrum or duodenum. Various portions of small bowel also may be involved, with or without intestinal obstruction. Colonic involvement (2) is rare. Eosinophilic infiltrates may be found in all layers of the affected hollow viscera. There should be no evidence of parasite infestation.

Pancreatitis (3) and cholangitis (2) in rare instances.

Manifestations of malabsorption syndrome* and of protein-losing enteropathy.* There should be no evidence systemic eosinophilic disease.

References

1. Mahajan L, Wyllie R, Petras R, Steffen R, Kay M. Idiopathic eosinophilic esophagitis with stricture formation in a patient with longstanding eosinophilic gastroenteritis. Gastrointest Endosc 1997;46: 557-560.

2. Schoonbroodt D, Horsmans Y, Laka A, Geubel AP, Hoang P. Eosinophilic gastroenteritis presenting with colitis and cholangitis. Dig Dis Sci 1995; 40:308-314.

3. Maeshima A, Murakami H, Sadakata H, Saitoh T, Matsushima T, Tamura J, et al. Eosinophilic gastroenteritis presenting with acute pancreatitis. J Med 1997;28:265-272.

Gastroenteropathy, Hemorrhagic

(See "Enterocolitis, pseudomembranous" and "Shock.")

Gigantism, Hyperpituitary (See "Acromegaly.")

Glomerulonephritis

Synonyms and Related Terms: Acute postinfectious glomerulonephritis (nonstreptococcal postinfectious glomerulonephritis;* minimal change disease; mesangial proliferative glomerulonephritis;* focal and segmental glomerulosclerosis with hyalinosis (focal sclerosis); poststreptococcal glomerulonephritis; idiopathic nephrotic syndrome; IgA nephropathy (Berger's disease); membranous glomerulonephritis; membranopro-liferative glomerulonephritis; mesangial proliferative glomerulonephritis; rapidly progressive glomerulonephritis (associated with systemic infectious or immunologic multisystem diseases; drug idiosyncrasy; or as primary crescentic glomerulonephritis or superimposed on another primary glomerular disease).

Possible Associated Conditions: Acquired immunodeficiency syndrome (AIDS);* Alport's syndrome;* amyloidosis; anaphylactoid purpura; bee stings; chronic allograft rejection; dermatomyositis;* dermatitis herpetiformis; diabetes mellitus;* drug dependence;* Fabry's disease;* Goodpasture's syndrome;* Guillain-Barre syndrome;* Henoch-Schönlein purpura;* hemolytic uremic syndrome;* infective endocarditis;* leprosy;* malignancies; mixed connective tissue disease; myxedema; polyarteritis nodosa;* rheumatoid arthritis;* preeclamptic toxemia; renovascular hypertension; sarcoidosis;* serum sickness;* Sjö-gren's syndrome;* syphilis;* systemic lupus erythematosus;* systemic sclerosis;* thrombotic thrombocytopenic purpura;* thyroiditis;* vasculitis; viral hepatitis;* Wegener's granulomatosis;* and many other conditions.

Organs and Tissues

Procedures

Possible or Expected Findings

Blood

Urine Kidneys

Other organs

Eyes

Refrigerate sample for possible serologic study —for instance, of basement membrane antibodies. Submit sample for urinalysis. Record weights; photograph surfaces and cut sections. Submit sample for immunofluorescent study. For electron microscopic study, see p. 132. Request 3-pm paraffin sections stained with PAS, methenamine silver, and Masson's trichrome stains (p. 173). Procedures depend on expected underlying, associated, or complicating conditions. If leg ulcers, wounds, or other acute infections are present, or if possibly nephritogenic chronic infections are found, submit material for appropriate bacterial cultures—for instance, from pharynx or middle ears. For removal and specimen preparation, see p. 85.

Cylindruria; hematuria; proteinuria. For specific types of glomerulonephritis, see above under "Synonyms and Related Terms." For further information, appropriate nephropathological texts should be consulted.

See above under "Possible Associated Conditions." See also under "Failure, kidney" and, if applicable, under "Dialysis (for chronic renal failure)."

Hypertensive retinopathy; in Alport's syndrome,* cataracts and other abnormalities.

Glycogenosis (See "Disease, glycogen storage.") Gout

Related Term: Hyperuricemia.

Possible Associated Conditions: Alcoholism;* berylliosis;* chronic renal failure with long-term renal dialysis; diabetes insipidus;* Down's syndrome;* drug toxicity; glycogenosis

(III, V, and VII); hemolysis; hyperparathyroidism;* hypertension;* hypothyroidism;* lead poisoning;* obesity;* Paget's disease; polycystic renal disease; polycythemia vera;* previous chemotherapy or radiation therapy of myeloproliferative disease; psoriasis;* pyelonephritis;* Reiter's syndrome;* rheumatoid arthritis;* sarcoidosis;* status post renal transplantation; toxemia of pregnancy,* and others.

Organs and Tissues

Procedures

Possible or Expected Findings

External examination and subcutaneous tissues

Blood

Heart and blood vessels

Trachea and major bronchi Kidneys

Other organs

Bones, joints, bursae, and tendons

Photograph and record location of tophi. For fixation for histologic study, place tophi in alcohol (p. 129), formalin-alcohol (p. 130), or Carnoy's fixative (p. 130). For murexide test for the macroscopic diagnosis of urates, see p. 134. Prepare skeletal roentgenograms.

Submit sample for determination of uric acid concentration.

Submit samples of myocardium and of elastic and muscular arteries for histologic study. For fixation procedures, see above under "External examination and subcutaneous tissues." Submit samples for histologic study. Prepare roentgenogram of soft tissues; photograph surfaces and cut sections. For fixation procedures, see above under "External examination and subcutaneous tissues." In cases of secondary gout, procedures depend on suspected underlying disease, as listed above under "Possible Associated Conditions." For removal of synovial fluid and for identification of crystals in gout and pseudogout, see p. 96. For removal, prosthetic repair, and specimen preparation of bones and joints, see p. 95.

In tophaceous gout, tophi at helices of ears and on elbows, knees, hands, and feet.

Acute or chronic gouty arthritis with punched-out bone lesions. Hyperuricemia. For interpretation of postmortem findings, see p. 114. Sodium urate deposits (uncommon in heart but may be responsible for cardiac dysrhythmia*).

Nephrolithiasis;* urate nephropathy; uric acid nephropathy.

See above under "Possible Associated Conditions."

In rare instances, pseudogout* or pyarthrosis may occur. Monosodium urate in synovial neutrophils.

Organs and Tissues

Procedures

Possible or Expected Findings

Bones, joints, bursae, and tendons

(continued)

Eyes

Use brush to clean frontal saw section of spine, and photograph. Photograph joint surfaces and other synovial surfaces that show white deposits. Submit samples of all involved tissues for histologic study. For fixation procedures and murexide test, see above under "External examination and subcutaneous tissues." For proper sampling of joints, consult roentgenograms. For preparation of museum specimens, see p. 134. For removal and specimen preparation, see p. 85. For fixation procedures, see above.

Urate deposits in intervertebral disks and on synovial surfaces; gouty and tophaceous arthritis.

Urate deposits in scleras and corneas.

Granulocytopenia (See "Pancytopenia.")

Granuloma, All Types or Type Unspecified (See "Disease, chronic granulomatous," "Granuloma,...," "Granulomatosis,...," and "Pneumoconiosis."

See also under name of specific granulomatous disease, such as "Sarcoidosis" and "Tuberculosis.")

Granuloma, Eosinophilic

(See "Histiocytosis, Langerhans cell.")

Granuloma, Midline

Synonyms: Idiopathic midline granuloma; lethal midline granuloma; granuloma gangrenescens. (The last two names are obsolete.)

NOTE: Midline granulomas may belong to the angiocentric immunoproliferative lesions, which are related to lymphoma-toid granulomatosis* and malignant lymphoma. The name "idiopathic midline granuloma" should be reserved for the few cases without evidence of malignant lymphoma or Wegener's granulomatosis* (1). The diagnosis of idiopathic midline granuloma also can be ruled out if studies reveal fungal organisms or features of leishmaniasis;* leprosy,* rhinoscleroma, pseudotumor of the orbit or tuberculosis.* Complications of nasal cocaine abuse also may mimic midline granuloma (2).

Organs and Tissues

Procedures

Possible or Expected Findings

External examination

Nasal cavities and paranasal sinuses

Neck organs Other organs

Record extent of necrosis, and photograph facial lesions.

For exposure of nasal cavities and sinuses, see p. 71. Submit material for bacterial and fungal cultures. Prepare smears and histologic sections of affected tissues. Request Verhoeff-van Gieson, Gram, and Grocott's methenamine silver stains (p. 172).

Submit lymph nodes for microbiologic (p. 102) and histologic study.

Necrosis of skin of nose and eyelids.

Necrosis with perforation of nasal septum, hard and soft palate, paranasal sinuses, and orbital cavities. Noncaseating granulomas with intense inflammatory reaction. Review material to rule out conditions mentioned under "Note."

See above under "Nasal cavities and paranasal sinuses."

For manifestations of diseases that may produce features of midline granuloma, see above under "Note."

References

1. Barker TH, Hosni AA. Idiopathic midline destructive disease: does it exist? J Laryngol Otol 1998;112:307-309.

2. Sevinsky LD, Woscoff A, Jaimovich L, Terzian A. Nasal cocain abuse mimicking midline granuloma. J Am Acad Dermatol 1995;32:286-287.

Granulomatosis, Allergic, and Angiitis (Churg-Strauss Syndrome) Related Term: Pulmonary granulomatous vasculitis (1). Possible Associated Condition: Asthma.*

Organs and Tissues Procedures Possible or Expected Findings

External examination Record extent of skin lesions and prepare Purpura; cutaneous and subcutaneous photographs. nodules (see below under "Other organs").

Organs and Tissues

Procedures

Possible or Expected Findings

Lungs

Other organs and soft tissues

Eyes

Brain, spinal cord, and peripheral nerves

Perfuse lungs with formalin (p. 47) and sample for histologic study.

Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column. Techniques are similar to those described under "Polyarteritis nodosa."

Histologic sampling should include cutaneous and subcutaneous nodules.

For removal and specimen preparation, see p. 85. For removal and specimen preparation, see pp. 65, 67, and 79, respectively.

Eosinophilic pneumonitis (degenerating eosinophils with Charcot-Leyden crystals) and granulomas. Angiitis (mostly arteritis), typically with giant cells in tunica media (1). Findings resemble those in polyarteritis nodosa.* Heart (2), grastrointestinal tract (3), skin, muscles, and joints are commonly involved. However, renal disease is often (but not always) mild or absent. Necrotizing vasculitis of small arteries and veins is present, with extravascular granulomas and eosinophilic infiltration of vessels and perivascular tissues. Optic neuritis may be found (4). May be affected by the vasculitis (4).

References

1. Travis WD. Pathology of pulmonary granulomatous vasculitis. Sarcoid Vasculit Diff Lung Dis 1996;13:14-27.

2. Terasaki F, Hayashi T, Hirota Y, Okabe M, Suwa M, Deguchi H, et al. Evolution of dilated cardiomyopathy from acute eosinophilic pancarditis in Churg-Strauss syndrome. Heart Vessels 1997;12:43-48.

3. Matsuo K, Tomioka T, Tajima Y, Takayama K, Tamura H, Higami Y, et al. Allergic granulomatous angiitis (Churg-Strauss syndrome) with multiple intestinal fistulas. Am J Gastroenterol 1997;92:1937-1938.

4. Sehgal M, Swanson JW, DeRemmee RA, Colby TV. Neurologic manifestations of Churg-Straus syndrome. Mayo Clin Proc 1995;70:337-341.

Granulomatosis, Bronchocentric

Synonyms and Related Terms: Allergic bronchopulmonary aspergillosis (1); eosinophilic pneumonia (eosinophilic pulmonary syndrome*); extrinsic allergic alveolitis; idiopathic bronchocentric granulomatosis; microgranulomatous hypersensitivity reaction of lungs; mucoid impaction of bronchi.

Possible Associated Conditions: Asthma;* cystic fibrosis.*

Organs and Tissues

Procedures

Possible or Expected Findings

Lungs

Submit one lobe for bacterial and fungal cultures (p. 103). Prepare smears of fresh cut sections. For pulmonary arteriography and bronchography, see p. 50. Perfuse one lung through bronchi and also through pulmonary arteries (plugged bronchi may prevent proper perfusion; see also p. 47).

Aspergillus (usually Aspergillus fumigatus) in dilated bronchi (2), with or without inspissation of mucus or fungus ball; necrotizing granulomatous pneumonia or bronchitis (2) with bronchial chondritis; eosinophilic pneumonia; obstructive (cholesterol-type) pneumonia; atelectases; emphysema.* Secondary arteritis may be present.

References

1. Bosken C, Myers J, Greenberger P, Katzenstein A-L. Pathologic features of allergic bronchopulmonary aspergillosis. Am J Surg Pathol 1988;12: 216-222.

2. Yousem AS. The histological spectrum of chronic necrotizing forms of pulmonary aspergillosis. Hum Pathol 1997;28:650-656.

Granulomatosis, Lymphomatoid

Related Term: Angiocentric immunoproliferative lesion; angiocentric malignant lymphoma.

Possible Associated Conditions: AIDS* (1) and other immunodeficiency states such as Wiskott-Aldrich syndrome or post-transplant immunosuppression.

Organs and Tissues Procedures Possible or Expected Findings

External examination Record extent of skin lesions; photograph skin Lymphoreticular infiltrates, primarily in and skin lesions; prepare histologic sections of involved dermis but also in subcutis. See also under skin and of grossly uninvolved skin. "Lungs."

Organs and Tissues

Procedures

Possible or Expected Findings

External examination and skin (continued) Blood

Lungs

Liver Kidneys

Other organs and tissues

Brain and spinal cord

Prepare chest roentgenogram.

Submit sample for bacterial, fungal, and viral cultures (p. 102). Snap-freeze sample for possible biochemical and immunologic study. Record weights; submit samples of fresh tissue for B- and T-cell gene derangement studies, frozen-section immunostains, and other investigations (see refs. 3 and 4). Submit one lobe for microbiologic study (p. 103). Touch preparations of cut surfaces of lungs for cytologic study may be helpful.

For pulmonary arteriography, see p. 50. Perfuse one lung with formalin (p. 47). Photograph cut sections of lungs. Submit samples of all lobes and of hilar lymph nodes for histo-logic study. Request Verhoeff-van Gieson, Gram, and Gridley's fungal stains (p. 172). For preparation of specimens for electron microscopy, see p. 132.

Record weight and sample for histologic studies.

Follow procedures described under "Glomerulonephritis."

Samples for histologic study should include heart, pancreas, spleen, adrenal glands, urinary bladder, prostate, neck organs (with nasopharynx and tongue), salivary glands, lymph nodes, thymus, bone marrow, and all other tissues with grossly identifiable lesions. For removal and specimen preparation, see pp. 65 and 67, respectively.

Multiple nodules, with or without cavitation; cavitation; rarely pneumothorax (2).

PCR studies on paraffin sections via RNA in situ hybridization may confirm presence of Epstein-Barr virus-positive B-cell proliferations combined with dense T-cell accumulations (3-5). The condition closely resembles angiocentric T-/NK cell lymphoma (3).

Infiltration of lymphocytoid cells, plasma cells, and macrophages with necroses and granulomatous features, which are found primarily in the vicinity of blood vessels. Special stains may reveal evidence of infection.

Lymphoreticular and granulomatous infiltrates (see "Lung"). Lymphoreticular and granulomatous infiltrates (see "Lung"). Characteristic infiltrates may be present in all organs and tissues. Involvement of spleen, lymph nodes, and bone marrow is uncommon. In rare instances, the disease is confined to the abdomen.

In most instances, characteristic infiltrates are present.

References

1. Haque AK, Myers JL, Hudnall SD, Gelman BB, Lloyd RV, Payne D, et al. Pulmonary lymphomatoid granulomatosis in acquired immunodeficiency syndrome: lesions with Epstein-Barr virus infection. Mod Pathol 1998;11:347-356.

2. Morris MJ, Peacock MD, Lloyd WC III, Johnson JE. Recurrent bilateral spontaneous pneumothoraces associated with pulmonary angio-centric immunoproliferative lesion. South Med J 1995;88:771-775.

3. Jaffe ES, Wilson WH. Lymphomatoid granulomatosis: pathogenesis, pathology and clinical implications. Canc Surv 1997;30:233-248.

4. McNiff JM, Cooper D, Howe G, Crotty PL, Tallini G, Crouch J, et al. Lymphomatoid granulomatosis of the skin and lung. An angiocentric T-cell-rich B-cell lymphoproliferative disorder. Arch Dermatol 1996; 132:1464-1470.

5. Myers J, Kurtin P, Katzenstein A-L, Tazelaar H, Colby T, Strickler J, et al. Lymphomatoid granulomatosis. Evidence of immunopheno-typic diversity and relationship to Epstein-Barr virus infection. Am J Surg Pathol 1995;19:1300-1312.

Granulomatosis, Wegener's

Related Terms: Angiocentric granulomatosis; granulomatous angiitis; pulmonary angiitis and granulomatosis (1).

Organs and Tissues

Procedures

Possible or Expected Findings

External examination and skin; oral cavity; breasts

Prepare histologic sections of skin lesions and of grossly uninvolved skin.

Prepare histologic sections of accessible mucosal lesions in mouth.

Skin papules, vesicles, ulcers. Subcutaneous nodules (vasculitis and granulomas). Granulomatous infiltrates of breast (2). Gangrene of digits (2,3). Necrotizing and ulcerative stomatitis.

Organs and Tissues

Procedures

Possible or Expected Findings

Blood Lungs

Spleen Kidneys

Neck organs with larynx and trachea

Other organs and tissues

Paranasal sinuses; ear, nose

Brain and spinal cord Eyes and orbitae

Bones and joints

Submit samples for microbiologic (p. 102) and for immunologic study. Submit one lobe for microbiologic study (p. 103). For pulmonary arteriography, see p. 50. Perfuse one lung with formalin (p. 47). Request Verhoeff-van Gieson stain (p. 172). Record weight; submit samples for histologic study.

Follow procedures described under "Glomerulonephritis."

Remove neck organs together with oropharynx and soft palate. Photograph lesions. For histo-logic study, submit samples with gross lesions and samples of grossly uninvolved tissue. Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column.

For exposure of sinuses, see p. 71. Specimens should include surrounding bone; submit samples for histologic study (for decalcification procedures, see p. 97).

For exposure of middle ear, see p. 72.

For removal and specimen preparation, see pp. 65 and 67, respectively.

For removal and specimen preparation, see pp. 85 and 73, respectively.

Septicemia; circulating immunoglobulin complexes.

Angiocentric granulomatosis (4); necrotizing arteritis with infarctions; granulomatous bronchitis; pleuritis.

Necrotizing arteritis. Infarctions (5).

Focal necrotizing glomerulitis; necrotizing arteritis (1).

Necrotizing granulomatous inflammation and ulcers of soft palate, larynx, and trachea. Subglottic stenosis. Acute obstruction may be a cause of death (6). Necrotizing arteritis and granulomatous inflammation—for example in heart, gastrointestinal tract, and urogenital organs (1). Necrotizing and ulcerative sinusitis with perifocal osteomyelitis. Necrotizing lesions in nasal cavities.

Otitis media.

Angiocentric granulomatous lesions may be present (1).

Pseudotumor of the orbit; other ocular lesions (1), such as conjunctivitis, dacryocystitis, scleritis, and episcleritis, granulomatous sclerouveitis; ciliary vasculitis. Arthritis.*

References

1. Lie JT. Wegener's granulomatosis: histological documentation of common and uncommon manifestations in 216 patients. VASA 1997; 26:261-270.

2. Trueb RM, Pericin M, Kohler E, Barandun J, Burg G. Necrotizing granulomatosis of the breast. Br J Dermatol 1997;137:799-803.

3. Handa R, Wali JP. Wegener's granulomatosis with gangrene of toes. Scand J Rheumatol 1996;25:103-104.

Gunshot (See "Injury, firearm.")

4. Travis WD. Pathology of pulmonary granulomatous vasculitis. Sarcoidosis, Vascul Diff Lung Dis 1996;13:14-27.

5. Fishman D, Isenberg DA. Splenic involvement in rheumatic diseases. Semin Arthritis Rheum 1997;27:141-155.

6. Matt BH. Wegener's granulomatosis, acute laryngotracheal airway obstruction and death in a 17-year-old female: case report and review of the literature. Int J Pediatr Otolaryngol 1996;37:163-172.

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