Extranodal Rosai-Dorfman Disease presenting as an epibulbar tumor

PURPOSE: To describe a case of an epibulbar tumor as a manifestation of Rosai-Dorfman Disease (RDD) and review the pertinent literature. METHODS: This was an interventional case report and literature review. A 19-year-old African-Brazilian man was referred for evaluation of a 12 x 11-mm subconjunctival mass at the temporal limbus OD. Intraocular pressure was normal, and funduscopy was unremarkable OU. Ultrasound biomicroscopy showed an ill-defined scleral homogeneous lesion at the temporal quadrant, without intraocular invasion. A superficial sclerokeratectomy was performed. RESULTS: Histopathologic evaluation revealed inflammatory aggregates composed of lymphocytes, plasma cells, and histiocytes. The histiocytes appeared pale, and some of them had intact lymphocytes and plasma cells within their cytoplasm. Russell bodies were also found. The histiocytes were positive for CD68 and S-100 and negative for lysozyme and CD1a. Special stains for microorganisms were all negative. Those findings were consistent with the diagnosis of extranodal RDD. Systemic workup failed to reveal lymphadenopathy or extranodal disease elsewhere. At the 14-month follow-up, there were no signs of recurrence. CONCLUSIONS: Although extranodal manifestations are common in RDD and the lack of lymph node involvement is rare, our study supports that whenever there is an epibulbar tumor as a manifestation of RDD, the absence of lymphadenopathy is characteristic. Only 2 of the 9 reported cases presented with lymphadenopathy. The presence of emperipolesis and S-100-positive histiocytes during histopathologic evaluation confirms the diagnosis even in the absence of lymphadenopathy.     

Extranodal Rosai-Dorfman disease presenting as an isolated epibulbar mass

Rosai-Dorfman disease is a rare idiopathic disorder characterized by painless lymphadenopathy with cervical involvement in more than 80% cases. We report a case of Rosai-Dorfman disease presenting as an isolated epibulbar mass in a healthy young adult male. Epibulbar involvement in Rosai-Dorfman disease is a rare presentation as can be seen from a review of all literature. The presentation, differential diagnosis and treatment are discussed.     

Sinus histiocytosis with massive lymphadenopathy: bilateral orbital involvement spanning 17 years

We report a case of sinus histiocytosis with massive lymphadenopathy (SHML) with a right inferior orbital mass. The mass was excised and, on histopathologic examination, was believed to be a "lipogranuloma." Thirteen years later, the patient was seen at our institution for what appeared to be a recurrent orbital mass. Orbital tumors were removed from the right superior and inferior orbit and from the left superior orbit. Histopathologic examination of these tumors at this institution and at the Armed Forces Institute of Pathology showed them to be SHML. The original biopsy tissue obtained in 1969 was reexamined and found also to be SHML. The clinical and histopathologic features of this disease are discussed.     

Xanthoma disseminatum with bilateral epibulbar involvement

PURPOSE: To describe the ocular involvement in xanthoma disseminatum. DESIGN: Case report and literature review. METHODS: Histopathologic study of excised conjunctival tumor and clinical follow-up. RESULTS: A 29-year-old woman with normal serum lipoproteins developed widespread xanthomas of the skin, throat, and episclera. There were no signs of recurrence 12 months after removal of the conjunctival xanthomas. CONCLUSIONS: Xanthoma disseminatum is a rare condition for which there is no medical treatment. The distinction between the normolipemic xanthomatoses awaits a better understanding of their pathogenesis. Surgical removal of episcleral xanthomas can be successful, but long-term follow-up of such patients has been limited.     

Radiowave surgery for epibulbar tumor and nontumor masses

The paper analyzes the results of removal of epibulbar tumor and nontumor masses, by using the radiowave surgical technique. The authors used two basic modes: fulguration and completely rectified and/ or completely filtered wave. Postoperative healing was aseptic and areactive. The duration of reparation depended on the working conditions, the volume of excised tissues, and the individual pattern of reparative processes. No intraoperative and postoperative complications were found. There were no cases of relapses and on-going growth during 9-12-month follow-ups. The authors have concluded that a radiowave knife may be used in surgery for epibulbar tumor and nontumor masses.     

Systemic sarcoidosis presenting initially with bilateral orbital and upper lid masses

A patient with systemic sarcoidosis presented initially with bilateral orbital and upper lid masses. Computed tomographic scan showed bilateral anterior orbital and upper lid masses, diffuse irregular thickening of the rectus muscles, circumferential cuffing of both globes, and irregular thickening of the distal portion of the right optic nerve sheath. A biopsy specimen of the right upper lid mass showed fibroadipose tissue with multiple noncaseating granulomas. Bilateral pulmonary hilar lymphadenopathy, a positive gallium scan, anergy by skin testing, and the presence of erythema nodosum confirmed the diagnosis of systemic sarcoidosis.     

Meningococcal septicemia presenting as bilateral endophthalmitis

We present a patient with bilateral endophthalmitis as the presenting sign of meningococcal septicemia. Systematic examination and vitreous tap conclusively identified the microbe, and appropriate treatment was administered, with good recovery of vision.     

Orofacial granulomatosis presenting as bilateral eyelid swelling

Orofacial granulomatosis (OFG) is an uncommon but increasingly recognized disease of unknown etiology. The typical presentation is chronic swelling of the perioral soft tissue, but eyelid edema can be the sole manifestation. Terminology of this disease can be confusing as it may also be referred to as granulomatous cheilitis and a monosymptomatic presentation of Melkersson–Rosenthal syndrome (MRS). Crohn’s disease and sarcoidosis should also be considered in the differential as the histopathology can be similar. Corticosteroids are the mainstay of treatment but can lack efficacy. Atypical presentations and the possibility of systemic disease involvement can further challenge the management. We describe an unusual case in which OFG manifests solely as chronic eyelid swelling. This 69-year old Asian female patient had a delayed diagnosis that responded well to intralesional corticosteroid injection with surgical skin reduction. In addition to describing this unusual presentation of OFG, we review the relevant literature and evaluate the current terminology used to describe this entity.     

Leucocytoclastic vasculitis presenting as bilateral marginal keratitis

Leucocytoclastic vasculitis is an immune-mediated, neutrophil-induced small vessel disease. Clinically, it presents with cutaneous palpable painless purpuric papules on the extremities. Ocular manifestation reported in the literature is rare and includes peripheral ulcerative keratitis, panuveitis and multifocal retinitis all preceded by cutaneous lesions. Herein a fatal case of leucocytoclastic vasculitis initially presenting with bilateral marginal keratitis without any cutaneous lesions is reported.     

Multiple myeloma presenting as bilateral orbital proptosis

A 58-year-old-man presented with painful rapidly progressive bilateral proptosis with restricted ocular movements of 15 days duration. There was history of significant weight loss in the recent past. Computed tomography scan of the head and orbit revealed bilateral multiple, well-defined, round, soft tissue masses, isointense with muscles in intraconal and extraconal space. Fine needle aspiration cytology and incision biopsy from the lesion, urine for Bence-Jones proteins and immunofixation clinched the diagnosis of multiple myeloma. Skeletal survey did not reveal any bony involvement.The diagnosis of multiple myeloma should be kept in mind in cases of bilateral proptosis. Bony involvement is not universal in cases of orbital myeloma. Early diagnosis can be established with extensive biochemical and histopathological investigations and timely treatment is life saving for these patients.     

Atypical Cogan's syndrome presenting as bilateral endogenous endophthalmitis

We report a case of atypical Cogan's syndrome presenting as bilateral endogenous endophthalmitis in a woman with ovarian cancer. A 62‐year‐old woman with ovarian cancer developed bilateral interstitial keratitis and panuveitis accompanied by bilateral sensorineural hearing loss and chondritis. Auricular cartilage biopsy ruled out relapsing polychondritis and the diagnosis of atypical Cogan's syndrome was set clinically.     

Rosai-Dorfman disease presenting as bilateral lacrimal gland enlargement

PURPOSE: To report a patient with bilateral lacrimal gland enlargement as the initial manifestation of Rosai--Dorfman disease. METHODS: Case report. RESULTS: A 14-year-old female presented with left lacrimal gland enlargement followed by right lacrimal gland enlargement 11 weeks later. Bilateral lacrimal gland biopsies were performed, and histopathologic examination revealed the diagnosis of Rosai--Dorfman disease. CONCLUSION: Patients with Rosai--Dorfman disease may present with bilateral lacrimal gland swelling in the absence of lymphadenopathy. Rosai--Dorfman disease should be considered in the differential diagnosis of bilateral lacrimal gland enlargement.     

Infectious mononucleosis presenting as bilateral acute dacryocystitis.

A case of infectious mononucleosis presenting as bilateral acute dacryocystitis in a 7-year-old girl is reported. Acute dacryocystitis is uncommon in this age group, and an underlying systemic illness should be suspected particularly when it is bilateral.     

Cystic conjunctival masses as the presenting sign of disseminated lymphoma

Systemic malignant lymphoma rarely presents as conjunctival masses. Biopsy of unusual cystic conjunctival masses from a 49-year-old woman showed malignant lymphoma. On subsequent examination, the patient was found to have disseminated systemic malignant lymphoma. Examination of possible predictive clinical features of the conjunctival lesions showed that these were of little value in diagnosing the systemic malignancy.     

A case of amniotic band syndrome with bilateral epibulbar choristoma.

An autopsy case of amniotic band syndrome with bilateral epibulbar choristoma is described. The left eye reveals a complex choristoma and the right eye a dermis-like choristoma. Both choristomatous lesions included lenticular tissue suggesting that rupture of the amnion, which is the initial event of amniotic band syndrome, might have occurred at about the fourth week of gestation. Since the other systemic manifestations of amniotic band syndrome are considered to be compression deformities of the fetus caused by oligohydramnios or amniotic band, the occurrence of epibulbar choristomas in both eyes in this case suggests that a compression mechanism may play a role in the pathogenesis of epibulbar choristoma.