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A chronic infectious disease characterized by multiple draining sinuses and caused by the anaerobic gram-positive microorganism Actinomyces israelii, often present as a commensal on the gums, tonsils, and teeth.
Incidence and Pathology
The disease is seen most often in adult males. In the cervicofacial form, the most common portal of entry is decayed teeth; pulmonary disease results from aspiration of oral secretions; abdominal disease, from a break in the mucosa of a diverticulum or the appendix.
The characteristic lesion is an indurated area of multiple, small, communicating abscesses surrounded by granulation tissue. Disease spreads to contiguous tissue and, rarely, hematogenously. Other anaerobic bacteria are usually also present.
Symptoms and Signs
There are 4 forms. (1) The abdominal form affects the intestines (usually the cecum and appendix) and the peritoneum. Pain, fever, vomiting, diarrhea or constipation, and emaciation are characteristically present. An abdominal mass with signs of partial intestinal obstruction appears, and draining sinuses and fistulas may develop in the abdominal wall. (2) The cervicofacial form (lumpy jaw) usually begins as a small, flat, hard swelling, with or without pain, under the oral mucosa or the skin on the neck, or as a subperiosteal swelling of the jaw. Subsequently, areas of softening appear and develop into sinuses and fistulas with a discharge that contains the characteristic sulfur granules (rounded or spherical, usually yellowish, granules up to 1 mm in diameter). The cheek, tongue, pharynx, salivary glands, cranial bones, meninges, or brain may be affected, usually by direct extension. (3) In the thoracic form, lung involvement resembles TB. Extensive invasion may occur before chest pain, fever, and productive cough appear. Perforation of the chest wall, with chronic draining sinuses, may result. (4) In the generalized form, hematogenous spread occurs to the skin, vertebral bodies, brain, liver, kidney, ureter, and (in women) the pelvic organs. More recently, this disease has been a local complication of the contraceptive intrauterine device (IUD).
Diagnosis
This is based on clinical symptoms, x-ray findings, and demonstration of A. israelii in sputum, pus, or biopsy specimen. In pus or tissue, the microorganism appears as tangled masses of branched and unbranched wavy filaments or as the distinctive sulfur granules. These consist of a central mass of tangled filaments, pus cells, and debris, with a midzone of interlacing filaments surrounded by an outer zone of radiating, club-shaped, hyaline and refractive filaments that take the eosin stain in tissue but are positive on Gram stain.
Lung lesions must be distinguished from those of TB and neoplasms. Most abdominal lesions occur in the ileocecal region and are difficult to diagnose, except at laparotomy or when draining sinuses appear in the abdominal wall. Aspiration liver biopsy should be avoided because of the danger of inducing a persistent sinus. A tender, palpable mass suggests appendiceal abscess or regional enteritis. Nodules in any location may simulate malignant growths.
Prognosis and Treatment
The disease is slowly progressive. Prognosis relates directly to early diagnosis; it is most favorable in the cervicofacial form and progressively worse in the thoracic, abdominal, and generalized forms. Prognosis is worst in CNS disease: >50% of patients have neurologic sequelae and >25% die.
Most cases respond to medical treatment, but owing to the extensive induration and relatively avascular fibrosis, response is slow and treatment must be continued for at least 8 wk and occasionally for >1 yr. Extensive and repeated surgical procedures may be required. Aspiration is indicated for small abscesses and drainage for large ones. Penicillin G, at least 12 million u./day IV, should be given initially; penicillin V 1 gm orally qid may be substituted after about 2 wk. Tetracycline 500 mg orally q 6 h may be given instead of penicillin. Treatment must be continued for several weeks after apparent clinical cure.
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