A case of cutaneous Rosai-Dorfman disease with increased IgG4-positive plasma cells / 中华皮肤科杂志

A 28-year-old female patient presented with indurated erythema and nodules on the right lower limb for 2 years,with mild itching and pain.Skin examination showed a well-circumscribed irregular dark red patch measuring about 10 cm × 5 cm in size on the extensor aspect of the right thigh.On the patch,there were scattered or densely distributed mung bean-to soybean-sized quasi-circular violaceous nodules with a smooth surface,which were hard on palpation.Subcutaneous nodules with medium hardness were found on palpation,and hyperpigmentation was observed on the surface of some nodules.Local skin temperature was increased,with tenderness on palpation.Histopathologically,mononuclear cells showed nodular or sheet-like distribution in the middle and upper dermis,some of which had pale-staining cytoplasm.Moreover,plenty of plasma cells were observed.Immunohistochemistry revealed that histiocytes were stained strongly positive for S100.The number of IgG4-positive plasma cells increased obviously,and was more than 50 per high-power field (× 200).The proportion of IgG4-positive plasma cells in IgG-positive plasma cells was 45％.Finally,the patient was diagnosed with cutaneous Rosai-Dorfman disease with increased IgG4-positive plasma cells.

A Case of Immunoglobulin G4-Related Sclerosing Disease not Responded to Steroid in Nasal Cavity

IgG4-related sclerosing disease was first discovered when researching autoimmune pancreatitis. It is an autoimmune disease that causes fibrosis on the pancreas, bile duct, gall bladder, salivary glands, and lungs. It is rarely reported in the nasal cavity. The diagnosis of IgG4-related sclerosing disease is based on biopsy findings demonstrating the characteristic histopathologic findings and immunohistochemical staining. IgG4-related sclerosing disease shows good response to high-dose steroid therapy. However, some patients do not respond to this treatment. Azathioprine or rituximab can be helpful in such case. We report our experience with IgG4-related sclerosing disease treated with azathioprine for 2 years, along with a related literature review.

Immunoglobulin G4-Related Sclerosing Disease Manifesting as Bilateral Tonsillar Hypertrophy on MR Images: A Case Report

Immunoglobulin G4-related sclerosing disease (IgG4-SD) is currently recognized as a distinct systemic disease involving various organs. We reported the imaging findings of a case of pathologically confirmed IgG4-SD involving bilateral palatine tonsils. CT and MRI showed diffuse enlargement of both palatine tonsils with homogeneous contrast enhancement. Focal contour bulging was noted in the right palatine tonsil. Lesions appeared as isointense on T1-weighted and slightly hyperintense on T2-weighted MRI images, as compared with muscle. The T2-weighted MRI image showed a striated pattern in both tonsils. Despite its rare occurrence, IgG4-SD should be included in the differential diagnoses of patients with symptomatic bilateral tonsillar hypertrophy that is non-responsive to medication.

Three Cases of IgG4-Related Sclerosing Disease in Nasal Cavity

IgG4-related sclerosing disease (IgG4-RSD) is characterized by extensive IgG4-positive plasma cell and T-lymphocyte infiltration of various organs. However, the nasal cavity is an extremely rare site of involvement. The authors experienced three cases of igG4-RSD in the nasal cavity. A 54-year-old woman presented with a nasal cavity mass on endoscopic exam and computed tomography (CT) revealed not only a hypoenhancing mass in the right nasal cavity but also a pituitary gland macroadenoma. Endoscopic removal and endoscopic TSA were performed. A 20-year-old man diagnosed with chronic sinusitis, underwent endoscopic sinus surgery. A 26-year-old woman showed thickening of the nasal septum and cribriform plate, and an infiltrating soft tissue lesion in right the alveolar process on CT and an endoscopic biopsy was performed. Pathologic specimens of all cases showed IgG4-positive plasma cell infiltration, consistent with IgG4-RSD. Serum IgG4 levels were checked in two of the cases, and the results were within normal range. All cases were further treated with methylprednisolone and all showed clinical improvement.

IgG4-Related Sclerosing Disease Involving the Superior Vena Cava and the Atrial Septum of the Heart

A 55-year-old woman presented with frequent episodes of syncope due to sinus pauses. During ambulatory Holter monitoring, atrial fibrillation and first-degree atrioventricular nodal block were observed. Magnetic resonance imaging and CT scans showed a tumor-like mass from the superior vena cava to the right atrial septum. Open chest cardiac biopsy was performed. The tumor was composed of proliferating IgG4-positive plasma cells and lymphocytes with surrounding sclerosis. The patient was diagnosed with IgG4-related sclerosing disease. Because of frequent sinus pauses and syncope, a permanent pacemaker was implanted. The cardiac mass was inoperable, but it did not progress during the one-year follow-up.

A Case of IgG4-Related Sclerosing Disease Involving the Eyelid in an Idiopathic Sclerosing Myositis Patient

PURPOSE: To report a case of IgG4-related sclerosing disease involving the eyelid in an idiopathic sclerosing myositis patient. CASE SUMMARY: A 51-year-old woman presented with swelling, redness, and tenderness of the left lower eyelid of 1 month duration had taken an immunosuppressant for idiopathic sclerosing myositis. Eye movements showed limitation all directions but there was no exophthalmos. A palpable mass was noted in the left lower eyelid. The left extraocular muscles were hypertrophied but the lacrimal gland was normal on orbital magnetic resonance imaging. IgG4-related sclerosing disease was confirmed by immunostained biopsy from the left lower eyelid, showing infiltration of IgG4-positive lymphoplasmacytic cells. The patient was given oral steroids and an immunosuppressant and the symptoms did not recur for at least 7 months. CONCLUSIONS: IgG4-related sclerosing disease involving ocular adnexa usually consists of bilateral lacrimal gland involvement. Additionally, the orbital soft tissue involvement without dacryoadenitis is rare. The authors of the present study report a case of IgG4-related sclerosing disease involving the left lower eyelid in an idiopathic sclerosing myositis patient and should be considered in the differential diagnosis of eyelid masses.

An Immunoglobulin G4-Related Sclerosing Disease of the Small Bowel: CT and Small Bowel Series Findings

Immunoglobulin G4 (IgG4)-related sclerosing disease is rare and is known to involve various organs. We present a case of histologically proven IgG4-related sclerosing disease of the small bowel with imaging findings on computed tomography (CT) and small bowel series. CT showed irregular wall thickening, loss of mural stratification and aneurysmal dilatation of the distal ileum. Small bowel series showed aneurysmal dilatations, interloop adhesion with traction and abrupt angulation.

An IgG4-Related Inflammatory Pseudotumor of the Greater Omentum / 대한내과학회지

IgG4-related sclerosing disease is a novel clinical disease entity characterized by an elevated serum IgG4 concentration and tissue infiltration by IgG4-positive plasma cells. Although this disease is a novel entity, it is not rare and is present in a proportion of patients with a wide variety of diseases, including autoimmune pancreatitis, sclerosing cholangitis, retroperitoneal fibrosis, and inflammatory pseudotumor. Despite the effectiveness of steroid therapy, IgG4-related sclerosing disease is often misdiagnosed as a malignant tumor. Here, we report an inflammatory pseudotumor of the great omentum that was misdiagnosed as a malignant tumor and subject to surgical resection.

IgG4-Related Sclerosing Disease, an Emerging Entity: A Review of a Multi-System Disease

Immunoglobulin G4-related systemic disease (IgG4-RSD) is a recently defined emerging entity characterized by a diffuse or mass forming inflammatory reaction rich in IgG4-positive plasma cells associated with fibrosclerosis and obliterative phlebitis. IgG4-RSD usually affects middle aged and elderly patients, with a male predominance. It is associated with an elevated serum titer of IgG4, which acts as a marker for this recently characterized entity. The prototype is IgG4-related sclerosing pancreatitis or autoimmune pancreatitis (AIP). Other common sites of involvement are the hepatobiliary tract, salivary gland, orbit, and lymph node, however practically any organ can be involved, including upper aerodigestive tract, lung, aorta, mediastinum, retroperitoneum, soft tissue, skin, central nervous system, breast, kidney, and prostate. Fever or constitutional symptoms usually do not comprise part of the clinical picture. Laboratory findings detected include raised serum globulin, IgG and IgG4. An association with autoantibody detection (such as antinuclear antibodies and rheumatoid factor) is seen in some cases. Steroid therapy comprises the mainstay of treatment. Disease progression with involvement of multiple organ-sites may be encountered in a subset of cases and may follow a relapsing-remitting course. The principal histopathologic findings in several extranodal sites include lymphoplasmacytic infiltration, lymphoid follicle formation, sclerosis and obliterative phlebitis, along with atrophy and destruction of tissues. Immunohistochemical staining shows increased IgG4+ cells in the involved tissues (>50 per high-power field, with IgG4/IgG ratio >40%). IgG4-RSD may potentially be rarely associated with the development of lymphoma and carcinoma. However, the nature and pathogenesis of IgG4-RSD are yet to be fully elucidated and provide immense scope for further studies.

Unusual Involvement of IgG4-Related Sclerosing Disease in Lacrimal and Submandibular Glands and Extraocular Muscles

Chronic sclerosing sialadenitis, also known as Kuttner tumor, is a chronic inflammatory disease of the salivary glands that is reported in a few cases in medical literature. Recent reports suggest that certain aspects of sclerosing diseases, including chronic sclerosing sialadenitis or dacryoadenitis, should be classified under immunoglobulin G4 (IgG4)-related sclerosing disease based on immunohistochemical studies. This study reports an unusual case of IgG4-related sclerosing disease appearing simultaneously in the lacrimal glands, submandibular glands, and extraocular muscles. A 56-year-old male presented with complaints of bilateral eyelid swelling and proptosis that began two years ago. Computed tomography confirmed that bilateral submandibular enlargements also existed five years ago in the subject. Orbital computed tomography and magnetic resonance imaging revealed bilateral lacrimal gland enlargement and thickening of extraocular muscles. Typical findings of chronic sclerosing dacryoadenitis were revealed upon pathologic exam of the right lacrimal gland. Immunostaining revealed numerous IgG4-positive plasma cells. Through these clinical features, we make a diagnosis of IgG4-relataed sclerosing disease in the subject.

A Case of IgG4-Related Sclerosing Disease Involving the Optic Nerve

PURPOSE: To report a case of IgG4-related sclerosing dacryoadenitis masquerading for a long period as a. CASE SUMMARY: A 59-year-old man with visual acuity loss and proptosis in the left eye was referred to our hospital. Ten years prior, the patient was diagnosed with lymphoma and underwent chemotherapy at another hospital. However, the patient spontaneously stopped treatment and took steroids for the relief of periorbital swelling. Magnetic resonance imaging revealed diffuse infiltrative lesions involving both optic nerves and extraocular muscles. Incisional biopsies of the lacrimal gland's posterior side and the lateral rectus muscle's lateral side were performed. Staining for IgG4 via immunochemistry showed infiltration of IgG4-positive lymphoplasmacytic cells. Under the diagnosis of IgG4-related sclerosing disease, the patient underwent high-dose steroid pulse therapy.

A Case of IgG4-Related Sclerosing Disease Involving the Optic Nerve

PURPOSE: To report a case of IgG4-related sclerosing dacryoadenitis masquerading for a long period as a. CASE SUMMARY: A 59-year-old man with visual acuity loss and proptosis in the left eye was referred to our hospital. Ten years prior, the patient was diagnosed with lymphoma and underwent chemotherapy at another hospital. However, the patient spontaneously stopped treatment and took steroids for the relief of periorbital swelling. Magnetic resonance imaging revealed diffuse infiltrative lesions involving both optic nerves and extraocular muscles. Incisional biopsies of the lacrimal gland's posterior side and the lateral rectus muscle's lateral side were performed. Staining for IgG4 via immunochemistry showed infiltration of IgG4-positive lymphoplasmacytic cells. Under the diagnosis of IgG4-related sclerosing disease, the patient underwent high-dose steroid pulse therapy.

IgG4-Related Sclerosing Sialadenitis: Report of Three Cases

Chronic sclerosing sialadenitis, Mikulicz disease or Kuttner tumor has been recently recognized as a spectrum of IgG4-related sclerosing disease. IgG4-related disease is characterized by a high serum IgG4 level and tissue infiltration of IgG4-positive plasmacytes. We report three cases of chronic sclerosing sialadenitis with variably associated systemic involvement. All patients presented with a submandibular mass or swelling, and all the resected submandibular glands showed diffuse lymphocytic infiltration, lymphoid follicles, and septal fibrosis. Two of the specimens revealed numerous IgG-positive plasma cells, most of which were IgG4-positive on immunohistochemical staining. One of them was associated with dacryoadenitis and hypophysitis. The other patient had ureterorenal lesions. Immunohistochemical study was unavailable in remaining one case, but the histologic features along with elevated IgG level and associated pancreatitis supported the diagnosis. All patients received steroid therapy postoperatively and are doing well. Salivary gland involvement in IgG4-related fibrosclerosis should be recognized in systemic medical pathology.