Daniel Kearney

IgG4-related orbital disease: a meta-analysis and review

IgG4‐related orbital disease (IgG4‐ROD) is a recently described condition that may account for a significant proportion of idiopathic lymphoplasmacytic or sclerotic orbital lesions. This study is the first meta‐analysis of published cases and reveals several differences between IgG4‐related disease affecting the orbit and that affecting the pancreas. IgG4‐ROD affects a slightly younger group of patients, affects men and women approximately equally, is commonly associated with salivary gland lesions, is associated with a relatively higher serum IgG4 and may confer an increased risk of non‐Hodgkin Lymphoma. Its pathogenesis may involve an immune response to antigen exposure in the upper aerodigestive tract.

Immunoglobulin G4-related disease of the hard palate.

A 71-year-old woman presented with erythematous, nontender, bilateral hard palate nodules of 6-month duration. Biopsy showed collagenous sclerosis and a follicular lymphoplasmacytic infiltrate among the minor salivary glands. Immunoglobulin G (IgG) and IgG4 staining showed 280 IgG4(+) cells per high-power field and a ratio of IgG4(+) to IgG(+) cells of 0.8. The patient subsequently developed bilateral lacrimal gland and parotid gland enlargement associated with an increased serum IgG4 level of 3,031 mg/dL (≤ 135 mg/dL). Left lacrimal gland biopsy confirmed IgG4-related dacryoadenitis. The patient declined corticosteroid treatment for IgG4-related disease (IgG4-RD) and remained stable at 15 months after the first presentation. Spontaneous, partial resolution of the palatal lesion was observed during follow-up. IgG4-RD should be considered in the differential diagnosis of lymphoplasmacytic lesions of the hard palate.

Applying the consensus statement on the pathology of IgG4-related disease to lacrimal gland lesions.

Applying the consensus statement on the pathology of IgG4-related disease to lacrimal gland lesions

Sequential biopsies from immunoglobulin G4-related orbital disease demonstrate progressive fibrosis

intensely stained blue, precluding any fundal examination (Fig. 1b). Red reflex of the right eye was unremarkable (Fig. 1a). Ten days postoperatively, the blue staining of the vitreous cleared and clinical examination was unremarkable. Best corrected visual acuity was 6/24 in the left eye and the patient has remained aphakic. We have described a case of trypan blue staining of the vitreous during cataract surgery in an eye with a previous peripheral iridotomy. Trypan blue staining of the vitreous has previously been reported in eyes with a history of trauma and in an eye with pseudoexfoliation syndrome. In these cases, there had been probable or confirmed compromise of the zonular integrity. In our case, there was no preoperative or intraoperative evidence of zonular dehiscence, although we postulate that the peripheral iridotomy or very dense cataract may have damaged the zonular apparatus and anterior hyaloid face, thus providing access for trypan blue to stain the vitreous. Although there was no evidence of retinal toxicity in this case, it has been suggested that trypan blue may have toxic effects on the retina. Given this, we propose that in cases where there is suspected compromise of the anterior hyaloid-zonular complex, special caution should be taken during the administration of trypan blue, as well as limiting the volume and duration of trypan blue in the anterior chamber. Premixing trypan blue with viscoelastic material to stain the anterior capsule may be an alternative. In summary, our case highlights the possibility of vitreous staining of trypan blue in an eye with a peripheral iridotomy, but no clinical signs of zonular dehiscence. Ophthalmic surgeons should be aware of this potential complication.

Successful Giant Thymic Cyst Removal: Case Report and Review of the Literature

Giant thymic cysts are a rare clinical entity evolving from smaller benign thymic cysts over many years. Benign thymic cysts account for approximately 3% of all mediastinal masses. There is a paucity of literature regarding benign thymic cyst management, especially when dealing with giant cysts. This can lead to potential confusion amongst clinicians on how to best treat these patients. We report the successful diagnosis and treatment of a 76 year-old female with a giant, benign thymic cyst. This cyst was discovered incidentally and after consultation of the literature it was found management strategies regarding this condition are scarce. After careful consideration of surgical principles, patient preference and potential complications of a conservative approach, the successful surgical removal of a 1.8 kg cyst took place. The patient improved symptomatically with improved exercise tolerance and lung function tests. This case demonstrates the benefits of giant thymic cyst removal thus confirming diagnosis, reducing potential serious complications and improving patient quality of life.

Cutaneous manifestations of peripheral T-cell lymphoma, not otherwise specified: A case series highlighting the diagnostic challenges for this heterogeneous group

Peripheral T‐cell lymphoma, not otherwise specified (PTCL, NOS) is a rare, heterogeneous group of nodal and extranodal mature T‐cell lymphomas that do not correspond to any of the defined T‐cell entities, according to the World Health Organization classification. Most cases present with late stage nodal disease; however extranodal involvement is common. Skin and subcutaneous involvement is reported in approximately 20% of cases. Little attention has been given to the highly variable skin manifestations in the literature. It is our experience that lesions can present in ways other than previously described nodular or tumourous lesions that often ulcerate. We present a case series from a large tertiary institution of seven cases of PTCL, NOS with skin involvement, highlighting the variable presentations and diagnostic challenges for this heterogeneous group.

IgG4 Staining in Thyroid Eye Disease.

IgG4-related ophthalmic disease is increasingly widely recognized. Moreover, IgG4 staining can occur in other inflammatory diseases. The authors report a case of IgG4 staining of an enlarged, inflamed levator palpebrae superioris in a patient with a past history of thyroid eye disease. A 78-year-old woman with quiescent hyperthyroidism had clinical and radiological evidence of levator palpebrae superioris inflammation without superior rectus involvement. A biopsy was consistent with IgG4-related ophthalmic disease. There was a marked but incomplete response to an orbital injection of triamcinolone. The authors discuss the association between thyroid eye disease and IgG4 staining and the diagnostic issues that arise when IgG4-related ophthalmic disease criteria are fulfilled in patients with other orbital inflammatory conditions.

Griseofulvin associated with drug reaction with eosinophilia and systemic symptoms (DRESS)

We present a case of drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome associated with griseofulvin, which, to our knowledge, has not been previously reported.