The hyperimmunoglobulin E syndrome—A neutrophil chemotactic defect reversible by histamine H2 receptor blockade?

Mawhinney, Helen; Killen, M.; Fleming, W. A.; Roy, A. D.

doi:10.1016/0090-1229(80)90144-0

Cited by 45 publications

(16 citation statements)

References 23 publications

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“…On the other hand, long-lasting corticosteroid therapy can favor the secondary colonization of amicrobial pustules. We treated 5 of our 6 patients with a combination of cimetidine, a histamine 2 receptor antagonist, and ascorbic acid, which have been reported to improve chemotactic responsiveness [20,21,22]. We obtained a complete response without noticeable relapses in 1 patient and partial remission in the other 4 cases, both lasting until the time of writing, over up to 12 years of follow-up.…”

Section: Discussionmentioning

confidence: 90%

Amicrobial Pustulosis of the Folds

2008

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Background: Amicrobial pustulosis of the folds (APF) is a rare entity characterized by relapsing pustular lesions involving mainly the cutaneous folds. The disease typically occurs in the context of an autoimmune disorder and is classified within the spectrum of neutrophilic dermatoses. Objective: We studied 6 young females having APF associated with various autoimmune diseases or with the presence of serum autoantibodies and reviewed the literature, in order to give a clinical overview on this syndrome. Methods: Various routine and immunological laboratory tests, histopathological examination as well as direct and indirect immunofluorescence examinations were performed. In vitro neutrophil function was evaluated in 5 cases. We report our findings and compare our cases with those published in the literature. Results: Clinically, at least one major fold and at least one minor fold as well as the anogenital area were always involved. We documented an impaired neutrophil chemotaxis in 2 subjects, neutrophil dysfunction, thus failing to be a verifying criterion. Conclusion: APF is a neutrophilic dermatosis affecting young females, which usually shows a benign clinical behavior. Although systemic corticosteroids are the most widely used therapeutic agents, we suggest that the combination of cimetidine and ascorbic acid represents a safe alternative, which may induce long-lasting clinical remission.

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Section: Discussionmentioning

confidence: 90%

Amicrobial Pustulosis of the Folds

2008

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“…A brief review of the literature on Job's syndrome and its associated infective pulmonary manifestations is also presented. as another possible treatment to enhance neutrophil chemotactic ability (11). Pulmonary pathogens that have been commonly associated with Job's syndrome include Staphylococcus, Pseudomonas, Aspergillosis and Candida species (Table 1).…”

Section: Discussionmentioning

confidence: 99%

Recurrent Hemoptysis with Penicillium Marneffei and Stenotrophomonas Maltophilia in Job’s Syndrome

Bosco

Lam

et al. 2009

Canadian Respiratory Journal

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A 30-year-old man was admitted to hospital for the management of massive hemoptysis. He had a long history of childhood cutaneous infections and recurrent pneumonia complicated by empyema, and thoracic and pulmonary abscesses that required prolonged intravenous antibiotics and surgical decortications. His family history was unremarkable. A previous sweat test was negative. Further investigations revealed marked eosinophilia and grossly elevated serum immunoglobulin (Ig) E levels of 2510 IU/L (normal range 0 to 100 IU/L), but normal IgA, IgG and IgM levels, which led to the clinical diagnosis of Job's syndrome. Despite prophylactic oral antibiotics (predominantly ampicillin-based), he suffered from further bouts of severe pneumonia with massive hemoptysis and the subsequent development of a large right upper lobe cavitating abscess that required lobectomy. Interestingly, the abscess culture grew Stenotrophomonas maltophilia, which was successfully treated with sulperazone (cefoperazone and sodium sulbactam).During the current admission 10 years later, he again presented with dyspnea and life-threatening hemoptysis. Multiple scars were noted from previous skin abscesses of the face and limbs, as well as from bilateral thoracotomy incisions. Breath sounds were reduced bilaterally, especially over the right side. A complete blood panel revealed leukocytosis (white blood cell count 16.4×10 9 /L [normal range 4×10 9 /L to 11×10 9 /L]), predominantly neutrophils (85.1%) and eosinophils (9.5%). A chest radiograph showed consolidative changes and cavitations over the right lower zone. Computed tomography revealed multiple pneumatoceles (Figure 1), the largest being 5.5 cm in size, with fluid levels in the right lower lobe. Urgent angiography was performed and two culprit hypertrophic bronchial arteries supplying the right main and right lower lobe bronchi ( Figure 2A) were successfully occluded by microsphere embolization (500 µm to 700 µm) ( Figure 2B), with subsequent resolution of hemoptysis. In addition, percutaneous drainage of the dominant lung abscess was performed. Both sputum and abscess fluid cultures grew Penicillium marneffei, which produces a characteristic soluble red pigment at 30°C (Figure 3). The patient was treated with amphotericin B and the prophylactic antibiotic cefepime. Despite appropriate antifungal and antibiotic therapy, his contralateral lung also rapidly became infected and he finally succumbed to respiratory failure. DisCussionThe term Job's syndrome was coined in 1966 by Davis et al (1), who described a rare condition characterized by recurrent staphylococcal skin infections, sinusitis, otitis media and pulmonary infections. Other clinical manifestations described since then include candida endocarditis, keratitis and corneal perforation, craniofacial and dental abnormalities, and skeletal deformities (2). Pulmonary complications such as recurrent pneumonia, pneumatocele formation and lung abscesses caused by bacterial and fungal infections are also common (3-7) ( Table 1).The mode of inher...

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“…There is good evidence that HIE patients have antistaphylococcal and antiCandida IgE (7,25), and it is possible that an infection with these organisms could lead to the combination of mast-cell bound IgE and staphylococcal antigens that might then lead to the release of mediators such as histamine that would alter leukocyte functions. Hill and Quie have suggested that histamine might play such a role in HIE (5), and there is evidence that H2 antagonists improve neutrophil function in HIE in vitro (26) and in vivo (9). However, this mechanism cannot be the sole basis of recurrent infections in HIE as it will not explain the lack of similar recurrent infections in patients with anti-Aspergillus IgE in broncho-pulmonary aspergillosis or in the millions of people with parasites and antiparasite IgE or in patients with systemic mastocytosis.…”

Section: Discussionmentioning

confidence: 99%

“…We believe that a deficiency in polymorphonuclear leukocyte (PMN) function may be more important in the pathogenesis of the repeated infections. Studies in several laboratories (2,4,5) and in ours revealed that, although HIE patients are not neutropenic and their PMN ingest and kill bacteria normally, both their neutrophils and monocytes have a chemotactic defect in vitro (3)(4)(5)(6)(8)(9)(10)(11)(12). But as Buckley & Becker (6) pointed out, the chemotactic defect of HIE leukocytes is not constantly present in these patients. We felt that an abnormality in the immunoregulatory system could explain the peculiar findings of the HIE syndrome.…”

Section: Introductionmentioning

confidence: 99%