Ann Thorac Surg 1995;59:759-761
© 1995 The Society of Thoracic Surgeons
Case Reports
Fungal Pulmonary Abscess in an Adult Secondary to Hyperimmunoglobulin E (Job's) Syndrome
R. Alan Hall, MD,
Kevin E. Salhany, MD,
Elise Lebel, MD,
Joseph E. Bavaria, MD,
Larry R. Kaiser, MD
Department of Surgery, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania
Accepted for publication July 14, 1994.
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Abstract
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Job's syndrome is characterized by recurring bacterial infections of the skin and sinopulmonary tract. Laboratory evaluation reveals consistent elevation of circulating immunoglobulin E levels. The syndrome has been reported as a rare cause of bacterial pulmonary abscess and pneumatocele formation in childhood; here we present a case of cavitating fungal abscess in an adult with Job's syndrome.
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Introduction
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A 30-year-old man with Job's syndrome, diagnosed at age 6 years, was referred to the Thoracic Surgical Service for evaluation of an enlarging left upper lobe cavitary lesion. The patient had been diagnosed with bacterial pneumonia approximately 2 months before admission and had received both intravenous and oral antibiotics. Sputum specimens grew Pseudomonas aeruginosa and Staphylococcus aureus. In spite of antibiotic therapy, the patient's predominant symptom of nonproductive cough persisted and serial chest radiographs suggested a gradually enlarging left upper lobe abscess. A thoracotomy with resection was recommended.
The patient's medical history was significant for low birth weight with probable intrauterine growth retardation. His birth was otherwise uncomplicated and his family history noncontributory. Since birth, the patient's life has been dominated by recurrent infections of the skin, soft tissue, nasopharynx, and pulmonary tract requiring more than 50 hospital admissions. At 6 months of age he presented with ``cold'' staphylococcal abscesses of the skin and a perirectal abscess requiring incision and drainage. In addition to countless skin infections, the patient has been treated for recurrent episodes of bronchitis, pneumonia, middle ear infections, conjunctivitis, pharyngitis, sinusitis, and candidal infections of the mouth and groin. To help control the occurrence of skin infections, he has taken dicloxacillin since childhood. The patient was diagnosed with hyperimmunoglobulin E syndrome at the age of 6 years with immunoglobulin E (IgE) levels measured between 900 to 4,000 IU/mL (normal, 0 to 100 IU/mL). In spite of his recurring bacterial infections and frequent hospitalizations, the patient enjoyed a highly functional life as a gainfully employed college graduate.
On admission, the patient was afebrile with a nonproductive cough. His trunk and proximal extremities provided evidence of multiple previous skin infections. There was no adenopathy appreciated on physical examination and pulmonary auscultation was significant only for decreased breath sounds in the left upper chest. His preoperative spirometry revealed moderate impairment with a forced expiratory volume in 1 second (FEV1) of 2.54 L (61% predicted) and a forced vital capacity of 3.81 L (77% predicted). Chemistry studies all were within normal limits. Complete blood count yielded a white blood cell count of 10.6/mL with 76% granulocytes. The remaining differential was normal. Although levels of immunoglobulins M and A were normal, the immunoglobulin G level was slightly low, at 643 IU/mL (normal, 650 to 1,900 IU/mL). The IgE level at the time of admission was elevated at 900 IU/mL. Levels of CD3, CD4, and CD8 also were within the normal range.
Chest radiographs (Fig 1
) demonstrated a left upper lobe cavitary lesion with a thickened surrounding wall. On the second hospital day, the patient underwent thoracotomy and left upper lobectomy. A 13 x 9 x 7-cm multiloculated, cavitary lesion containing 200 mL of brown fluid was present within the specimen. A loosely adherent nodule was present within the cavity; the nodule proved to be an aspergilloma consisting of septate fungal hyphae with dichotomous 45-degree angle branching (Fig 2). Infarcted pulmonary parenchyma infiltrated by Aspergillus hyphae was identified lining the cavity wall (Fig 3). Focal extension of Aspergillus into adjacent bronchioles and pulmonary parenchyma was observed, but no vascular invasion was identified.
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Fig 3. . Infarcted pulmonary parenchyma infiltrated by Aspergillus (arrows). (Hematoxylin and eosin; original magnification, x50.)
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The postoperative course was notable for a persistent air leak, which closed spontaneously. Treatment with amphotericin B was instituted for invasive Aspergillus, although progressive renal insufficiency necessitated its discontinuation. The patient was placed on a 6-week course of itraconazole and has done well since discharge. There is no active disease on his follow-up chest radiograph, and his renal function studies have returned to normal.
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Comment
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From the Book of Job 2:7, ``So went Satan forth from the presence of the Lord, and smote Job with some boils from the sole of his feet unto his crown.'' From this reference was coined the term Job's syndrome by Davis and colleagues in 1966 [1]. Originally described in red-haired whites, the syndrome has now been reported in blacks, asians, and whites of all complexions. Characteristic of the disease are ``cold'' abscesses, so named because they lack the surrounding inflammation of typical purulent infections. However, despite their benign appearance, they are infectious in nature and yield S aureus upon drainage. The detection of marked elevations in the level of circulating IgE has since been recognized as the central immunologic defect and the defining characteristic of the disease [2]. In addition to elevations in IgE levels, defects in neutrophil chemotaxis have been reported by several authors [3–7]. A review of the National Institute of Health experience of 13 patients with Job's syndrome suggested that recurrent bronchitis is a common pulmonary complication of the syndrome. Pneumonia also was diagnosed, although less frequently, with S aureus and Haemophilus being the predominant pathogens. Empyemas developed in many of these patients, with chest tube insertion a common finding in the medical histories of these patients [8]. Our patient had chest tube placement at 6 months for a pneumothorax of unknown cause. Interestingly, a recent case of a child with Job's syndrome in whom a pneumothorax developed with underlying pneumatocele has been reported [9].
Typically, levels of IgE are consistently elevated over time, with levels of other immunoglobulins within the normal range. As yet, no explanation for the persistent elevation of immunoglobulin level exists, although lack of T-cell suppressor function allowing for increased IgE production has been proposed. It is known that cellular production of IgE in this syndrome is not governed by normal cellular regulatory signals [10]. Although increased IgE level has been blamed for impaired phagocyte chemotaxis, the National Institutes of Health review found that defects in chemotaxis are an inconsistent finding in their study population. Our patient was found to have a normal chemotactic response on two separate occasions. Interestingly, evidence does exist to suggest that mononuclear cells from patients with hyperimmunoglobulin E syndrome may produce an inhibitor of neutrophil chemotaxis distinct from IgE. A second and separate inhibitor may be released after mononuclear exposure to S aureus [11]. Finally, although no mode of inheritance has been established, a genetic pattern has been identified between family members [12] and in an infant with an affected mother [13].
In summary, we have presented a 30-year-old with hyperimmunoglobulin E syndrome in whom pulmonary cavitation developed secondary to Aspergillus fumigatus. Histologic study revealed the invasive nature of this fungus with extension to the visceral pleura. Although sinopulmonary infections are common, the development of a cavitary fungal abscess of the lung parenchyma is quite rare. Given the recurrent nature of the disease process, minimal resection with tissue preservation is advised. In addition to bacterial pneumonia, pneumatocele formation, and empyema, the development of pulmonary fungal infection must be included in the differential diagnosis in this immunocompromised population with pulmonary complications.
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Footnotes
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Address reprint requests to Dr Kaiser, Hospital of the University of Pennsylvania, 34th and Spruce Sts, 4 Silverstein, Philadelphia, PA 19104.
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References
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- Davis SD, Schaller J, Wedgwood RJ. Job's syndrome: recurrent ``cold'' staphylococcal abscesses. Lancet 1966;1:1013–5.[Medline]
- Buckley RH, Wray BB, Belmaker EZ. Extreme hyperimmunoglobulinemia E and undue susceptibility to infection. Pediatrics 1972;49:59–70.[Abstract/Free Full Text]
- Clark RA, Root RK, Kimball HR, Kirkpatrick CH. Defective neutrophil chemotaxis and cellular immunity in a child with recurrent infections. Ann Intern Med 1973;78:515–9.
- Gallin JI. Disorders of phagocyte chemotaxis. Ann Intern Med 1980;92:520–38.
- Gallin JI. Abnormal phagocyte chemotaxis: pathophysiology, clinical manifestations and management of patients. Rev Inf Dis 1981;3:1196–220.[Medline]
- Hill HR, Quie PG. Raised serum IgE levels and defective neutrophil chemotaxis in three children with eczema and recurrent bacterial infections. Lancet 1974;1:183–7.[Medline]
- Hill HR, Quie PG, Pabst HF, et al. Defect in neutrophil granulocyte chemotaxis in Job's syndrome of recurrent ``cold'' staphylococcal abscesses. Lancet 1974;2:617–9.[Medline]
- Donabedian H, Gallin JI. The hyperimmunoglobulin E recurrent-infection (Job's) syndrome: a review of the NIH experience and the literature. Medicine 1983;62:195–208.[Medline]
- Shamberger RC, Wohl ME, Perez-Atayde A, Hendren WH. Pneumatocele complicating hyperimmunoglobulin E syndrome (Job's syndrome). Ann Thorac Surg 1992;54:1206–8.[Abstract]
- Vercelli D, Jabara HH, Cunningham-Rundles C, et al. Regulation of immunoglobulin (Ig)E synthesis in the hyper-IgE syndrome. J Clin Invest 1990;85:1666–71.
- Donabedian H, Gallin JI. Mononuclear cells from the patients with the hyperimmunoglobulin E-recurrent infection syndrome produce an inhibitor of leukocyte chemotaxis. J Clin Invest 1982;69:1155–63.
- Nielsen H, Valerius NH, Schaffalitzky OB. Selective defect of phagocyte responsiveness to N-f-Met-Leu-Phe in a familial syndrome of recurrent cold abscesses. J Infect Dis 1986;153:1184–6.[Medline]
- Lindenbaum C, Chatwani A, Dyer R. Hyperimmunoglobulinemia E and pregnancy: a case report. Am J Obstet Gynecol 1987;157:1273–4.[Medline]
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Abstract
Introduction
