Manifestations of immunoglobulin G4 related disease in otolaryngology: case reports and review of the literature.
نویسندگان
چکیده
BACKGROUND Immunoglobulin G4 related disease is an inflammatory condition characterised by the presence of fibrotic lesions infiltrated by immunoglobulin G4 positive plasma cells. It can arise from almost any region of the body and it is being increasingly recognised in the head and neck. Regardless of the site of involvement, the histopathological resemblance is remarkable. Dense lymphoplasmacytic infiltration, overabundance of immunoglobulin G4 bearing plasma cells and presence of storiform fibrosis are typical findings. CASE REPORTS This paper presents two cases of immunoglobulin G4 related disease in which there was involvement of the orbit, the infraorbital nerve and the infratemporal fossa. Diagnosis was established in both cases by biopsying radiologically abnormal tissue in the infratemporal fossa. CONCLUSION An awareness of this condition is required to establish the diagnosis and initiate appropriate therapy. Glucocorticoids are the mainstay of initial treatment. The effectiveness of B-lymphocyte depletion with rituximab has also been reported. Correct diagnosis may spare patients from unnecessarily radical surgery.
منابع مشابه
Immunoglobulin G4-related diseases in the head and neck: a systematic review
BACKGROUND Immunoglobulin G4 related disease (IgG4-RD) is a poorly understood chronic inflammatory disorder affecting the middle-aged and elderly that can present to the otolaryngologist. We aim to summarize the current literature regarding the manifestations and management of IgG4-RD in the head and neck. METHODS Pubmed and EMBASE were searched using the term relevant search algorithm utiliz...
متن کاملPrimary Cutaneous Mucormycosis of the Neck:A CaseReport with Review of Literature
Background: Cutaneous mucomycosis is an invasive fungal infection that usually develops in the immunodeficient patients. Although it also occurs in cases without underlying disease. So far in Iran and the world, primary cutaneous mucormycosis of the neck without predisposing factor has not been reported. Materials and Methods: In this case,we present a 51 years old patient with progressive nec...
متن کاملA Rare Case Report with Periorbital Edema
IgG4 related disease is fibro-inflammatory condition characterized by infiltration and increased levels of G4 immunoglobulin, multi-organ involvement and periorbital swelling. It is more common among men especially in middle to old ages. The diagnosis should be confirmed with histopathology. The diagnostic three important features of disease are lymphoplasmacytic infiltration, storiform fibrosi...
متن کاملIgG4-related disease presenting with an epidural inflammatory pseudotumor: a case report.
BACKGROUND Inflammatory pseudotumor is a rare clinical condition that can be related to immunoglobulin G4 disease. Only a few cases of spinal inflammatory pseudotumors have been reported in the literature and an association with immunoglobulin G4 disease was not conclusive in any of them. We describe what we believe to be the first biopsy-proven case of an epidural inflammatory pseudotumor rela...
متن کاملImmunoglobulin G4-related pleuritis – A case report
Immunoglobulin G4-related disease (IgG4-RD) is a chronic disease that presents with inflammation and fibrosis of involved tissue. It encompasses several disorders previously described using different terms, but all disorders are characterised by IgG4-positive plasma cells and lymphocytes infiltration of tissues. We report a rare case of a 58-year-old man with IgG4-related pleural disease withou...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- The Journal of laryngology and otology
دوره 128 Suppl 2 شماره
صفحات -
تاریخ انتشار 2014