Fungal gingival infection in hyper-IgE (Job's) syndrome patient.
Journal name: Journal of Indian Society of Periodontology
Original article title: Fungal infection of gingiva in a patient with hyperimmunoglobulin-E (Job's) syndrome
The Journal of Indian Society of Periodontology (JISP) publishes original scientific articles on periodontology (the study of supporting structures of teeth) and oral implantology. It is a bimonthly open-access journal with special issues for specific occasions.
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Original source:
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D. Deepa, K. V. Arun Kumar, Chander Shekhar Joshi, Sanjeev Kumar, Anita Pandey
Journal of Indian Society of Periodontology:
(A bimonthly open-access journal)
Full text available for: Fungal infection of gingiva in a patient with hyperimmunoglobulin-E (Job's) syndrome
Year: 2012 | Doi: 10.4103/0972-124X.99272
Copyright (license): CC BY-NC-SA
Summary of article contents:
Introduction
Hyperimmunoglobulin E syndrome (HIES), also known as Job's syndrome, is a rare primary immunodeficiency disorder characterized by a combination of symptoms, including eczema, recurrent infections of the skin and lungs, elevated serum IgE levels, and various connective tissue and skeletal abnormalities. Patients with HIES also share distinctive facial features and have a range of oral manifestations such as retained primary dentition, high-arched palates, oral mucosa variations, and recurrent oral candidiasis. The syndrome's recognition has evolved with advances in genetic understanding, particularly regarding mutations in the STAT3 gene, which have been identified as key contributors to the disease.
The Role of Oral Manifestations in HIES
Oral manifestations of HIES can significantly impact the patient's overall health and quality of life. As exemplified in a case report of an 18-year-old female patient, common oral issues include gingival swelling, bleeding, and significant abnormalities such as retained primary teeth and congenitally missing permanent teeth. Additionally, the development of oral candidiasis, evidenced by the growth of Candida albicans in the patient's gingival biopsy, showcases the immunocompromised state associated with HIES. Periodontal treatments, such as scaling and root planing combined with diode laser-assisted gingivoplasty, have been applied to address these oral challenges, emphasizing the importance of dental professionals in the diagnosis and management of this complicated syndrome.
Conclusion
Awareness of the diverse features of HIES, particularly its oral manifestations, can facilitate early diagnosis and improved patient care. Given that the disease is tied to immune system dysregulation, appropriate antibiotic and antifungal treatments can mitigate infectious complications. The ongoing study of HIES, especially in relation to STAT3 mutations, promises to deepen our understanding of various associated symptoms, including eczema, IgE regulation, and infection susceptibility, ultimately enhancing therapeutic strategies for affected patients.
FAQ section (important questions/answers):
What is Hyperimmunoglobulin E syndrome (HIES) or Job's syndrome?
HIES, or Job's syndrome, is a rare primary immunodeficiency characterized by eczema, recurrent infections, elevated serum IgE levels, and various connective tissue and skeletal abnormalities.
What are the common oral manifestations of HIES?
Common oral manifestations include retained primary teeth, a high-arched palate, gingival enlargement, variations of oral mucosa, gingivitis, and recurrent oral candidiasis.
How is the diagnosis of HIES established?
The diagnosis is based on clinical manifestations, laboratory features, and serological tests showing elevated IgE levels. Genetic testing for STAT3 mutations can confirm the diagnosis.
What treatment options are available for HIES patients?
Treatment includes anti-fungal antibiotics, scaling, root planing, gingival contouring, and nutritional support. Ongoing management of infections is also essential.
What role does STAT3 play in HIES?
Mutations in the STAT3 gene are responsible for the majority of autosomal dominant HIES cases, affecting immune regulation and leading to increased susceptibility to infections.
Glossary definitions and references:
Scientific and Ayurvedic Glossary list for “Fungal gingival infection in hyper-IgE (Job's) syndrome patient.”. This list explains important keywords that occur in this article and links it to the glossary for a better understanding of that concept in the context of Ayurveda and other topics.
1) Disease:
Hyperimmunoglobulin E syndrome (HIES), also known as Job's syndrome, is a rare primary immunodeficiency disease characterized by a range of clinical features. These include eczema, recurrent infections, elevated IgE levels, dental abnormalities, and connective tissue issues, which impact the patient's overall health and quality of life.
2) Mutation:
Recent research has identified mutations in the STAT3 gene as a significant cause of autosomal dominant HIES. These mutations affect protein interactions related to immune responses. Understanding these genetic factors is essential for characterizing the disease at the molecular level and linking it to various clinical manifestations.
3) Antibiotic (Antibacterial):
In managing HIES, antibiotics are crucial for treating recurrent infections associated with the condition. The case highlighted in the text involved the administration of antibiotics to address respiratory infections, emphasizing the necessity of these medications in controlling complications arising from the immunodeficiency.
4) Bleeding:
The presentation of gingival bleeding in the report signifies a related oral manifestation of HIES. Severe gingival enlargement and inflammation can lead to bleeding, reflecting the importance of oral health in patients with immunodeficiency and the need for dental interventions to manage such complications.
5) Swelling:
Gingival swelling was a prominent symptom in the presented case. This swelling can indicate underlying infections or chronic inflammation, common in individuals with HIES. Monitoring and addressing swelling through appropriate dental treatments are vital for maintaining the oral health of affected patients.
6) Fever:
The occurrence of intermittent fever in the patient suggests recurrent infections, a common issue for those with HIES due to their immunological deficiencies. Recognizing fever as a symptom aids in understanding the ongoing infection risks faced by patients and emphasizes the need for vigilant medical management.
7) Account:
In the context of HIES, 'account' relates to documenting and understanding the varied clinical manifestations tied to the syndrome. Case reports and clinical accounts contribute to a broader awareness of the syndrome, aiding in diagnosis and improving patient management through shared knowledge among healthcare providers.
8) Hygiene (Hygienic):
Maintaining excellent oral hygiene is critical for individuals with HIES, given their vulnerability to oral infections such as candidiasis. Effective hygiene practices can prevent conditions such as gingivitis and other periodontal issues, thereby improving the overall health and comfort of patients suffering from this syndrome.
9) Family:
The mention of family in the case report indicates the hereditary nature of HIES. Understanding familial occurrences of the disorder is crucial for genetic counseling and raising awareness about potential immunodeficiencies among relatives of affected individuals, thus aiding in early diagnosis and treatment.
10) India:
The patient's presentation in India highlights the global occurrence of HIES, regardless of geographic location. This context emphasizes the need for awareness among healthcare practitioners everywhere about the distinctive features of HIES, enhancing early recognition and management in diverse healthcare settings.
11) Visit:
The patient's visit to the dental department showcases the importance of multidisciplinary care in HIES management. Dental professionals can play a key role in identifying manifestations of the syndrome early, thus facilitating timely interventions and a comprehensive approach to the patient’s overall health.
12) Birth:
Recognition of symptoms since birth underlines the congenital nature of HIES. Symptoms can manifest at various stages, but early identification is vital for managing complications and providing adequate treatment to improve quality of life for affected individuals from a young age.
13) Study (Studying):
The study of HIES and its manifestations is essential for expanding the understanding of this rare syndrome. Investigating clinical features, immunological markers, and genetic factors allows healthcare professionals to develop better diagnostic criteria, treatment strategies, and patient management protocols for those affected.
14) Male (Mālē):
HIES affects both males and females equally, as highlighted in the literature. Understanding the gender distribution of the disease assists in recognizing that all individuals, regardless of sex, can exhibit the syndrome's symptoms, ensuring equitable awareness and access to medical care.
15) Viru (Vīṟu):
Though 'viru' is not explicitly detailed in the provided text, it likely relates to infections, particularly viral infections common in immunodeficient patients like those with HIES. Recognizing the susceptibility to various pathogens emphasizes the need for vigilant monitoring and preventative measures for affected individuals.