Case Reports of Severe Paediatric Sickle Cell Retinopathy: Disease Manifestations, Progression and Treatments.

Severe sickle retinopathy is commonly known in adults but not in children, hence any related treatment for sickle retinopathy in children was not well described. We reported 2 paediatric sickle patients (aged 12 and 13) presented with severe sickle retinopathy and described details of their disease progression and treatments over 2-3 years, along with the challenges faced when managing this particular group of young age sickle cell patients. Our case reports also demonstrated the benefits of laser photocoagulation treatment to early sickle proliferative disease, and how complications from advanced severe retinopathy hindered effective treatments.

Foreign body giant cell reaction to silicone oil presenting as a salmon-patch conjunctival lesion.

Purpose: To describe a case of foreign body giant cell reaction to silicone oil that presented as a salmon-patch conjunctival lesion. Observations: An elderly female with prior retinal surgery and oil tamponade was referred for a salmon-patch lesion in the conjunctiva. Biopsy revealed multiple vacuolations and foreign body giant cells in the substantia propria, consistent with a foreign body reaction to silicone oil. Conclusion and importance: Silicone oil can elicit an inflammatory reaction in the conjunctiva that could mimic a neoplasm. Excessive leakage of oil into the subconjunctival space should be avoided to prevent this complication.

Conjunctival myxoma masquerading as conjunctival lymphoma: A case report.

INTRODUCTION AND IMPORTANCE: Ocular myxomas are very rare and can involve the orbit, eyelids, and conjunctiva. Conjunctival myxoma can be misdiagnosed as amelanotic nevus, conjunctival cyst, or ocular surface squamous neoplasia, among others. They can appear as an isolated lesion or can be associated with systemic manifestations as part of the Carney complex or Zollinger-Ellison syndrome. CASE PRESENTATION: We describe a 64-year-old healthy male who presented with a right eye painless peri-limbal salmon-colored patch lesion in the infero-temporal bulbar conjunctiva over a period of 2 years. There was no of ocular trauma or surgery and no effect on vision. The mass was not tender, raised, and mobile with fine intrinsic vascularity. Excisional biopsy with the presumed diagnosis of lymphoma revealed a typical sub-conjunctival myxoma. DISCUSSION: The recognition of ocular myxoma necessitates systemic evaluation to rule out possible associated cardiac myxoma in Carney complex, thus can prevent life-threatening events. The excised mass in our patient showed an area of pseudo-elastotic degeneration, which has further complicated the clinical appearance of the lesion, however, the color, and consistency of the mass were highly suspicious of lymphoma. The diagnosis of myxoma by histopathology was helpful especially in presence of atypical appearance such as in our case. CONCLUSION: The histopathological characteristics of conjunctival myxoma can aid in the diagnosis. The lesion in our case was associated with focal severe pseudo-elastotic degeneration and prominent salmon-patch appearing area thus was initially misdiagnosed clinically as a conjunctival lymphoma.

Salmon patch-associated vitreous hemorrhage in non-proliferative sickle cell retinopathy masquerading as infectious uveitis.

PURPOSE: To report three cases of non-proliferative sickle cell retinopathy (NPSR) with vitreous hemorrhage masquerading as infectious uveitis. OBSERVATIONS: Three patients were referred from ophthalmologists to our practices with clinical findings suggestive of infectious uveitis. The first patient was referred for new-onset floaters in both eyes, bilateral vitritis and dome-shaped lesions on B-scan ultrasound. He was initially treated for tuberculosis uveitis due to a positive purified protein derivative test. The second patient was referred with floaters and hazy vision in the setting of recent fever and headache and was also reported to have vitritis and unilateral yellow vitreoretinal lesions on fundoscopy. She was initially treated for toxoplasmosis and endogenous endophthalmitis. The third patient presented with flashes, floaters, and decreased vision four months after a ring-enhancing lesion was found on brain imaging, and was found to have unilateral vitritis with yellow vitreoretinal lesions. He was initially started on topical steroids and cycloplegics empirically for uveitis. All patients were ultimately diagnosed as having manifestations of NPSR, including vitreous hemorrhage, and dehemoglobinized salmon patch hemorrhages. CONCLUSIONS AND IMPORTANCE: NPSR can occasionally masquerade as infectious uveitis. Obtaining a detailed history with relevant ancillary testing, along with performing a careful physical exam to recognize important clues, can help the physician arrive at the correct diagnosis in these equivocal cases.

Dermoscopic Features of Nail Psoriasis: An Observational, Analytical Study.

INTRODUCTION: Nail involvement in psoriasis occurs in up to 30-50% of patients, and 5-10% may have isolated nail disease. Onychoscopy, a noninvasive tool, might obviate the need for nail biopsy, which is a diagnostic gold standard. OBJECTIVE: The aim of this study was to evaluate onychoscopic features of nail unit in patients with nail psoriasis. METHODS: Fifty-five patients with clinically diagnosed and histologically documented nail psoriasis were recruited. Onychoscopy was performed for each nail (excluding the 5th toenail). Clinically, 443/550 fingernails and 101/440 toenails were involved. The frequency distributions of various onychoscopic features was assessed and compared using the χ2 test (p value <0.05 was considered significant). RESULTS: With onychoscopy, additional 52 fingernails and 64 toenails showed psoriatic involvement. Pitting was the commonest finding in fingernails (60.5%) followed by subungual hyperkeratosis (SUH) (52.8%), onycholysis (40.8%), and dotted capillaries in hyponychium, proximal, and lateral nail folds (38.6 vs. 35.8 vs. 35.8%). In toenails, we observed SUH (85.1%), nail plate thickening (82.1%), onycholysis (77.2%), and dotted capillaries in hyponychium and nail folds (59.4 vs. 53.4 vs. 45.5%). Fuzzy lunula was a novel onychoscopic finding noted in 33.6% fingernails and 4.95% toenails (p < 0.00001). LIMITATIONS: Small sample size. CONCLUSIONS: Onychoscopy may aid in diagnosing nail lesions even before the clinical signs are apparent.

Multiple myeloma manifesting as an ocular salmon patch - a case report.

Multiple myeloma (MM) is the second most prevalent hematologic malignancy after non-Hodgkin lymphoma and is currently considered incurable. Clinical ophthalmic manifestations of MM are rare but at the same time diverse. Ocular surface manifestations of multiple myeloma are uncommon. Conjunctival 'salmon patch' is a typical ocular surface ophthalmological sign with a distinct set of differential diagnoses, including most often ocular adnexal lymphoma. This case report presents a 33-year-old female with a relapse of MM manifesting as a conjunctival 'salmon patch'. The patient initially responded well to medical management including high dose melphalan supported by a third autologous stem cell transplantation (ASCT) and did not require further surgical excision of the ocular lesion. It is suggested that MM should be included in the differential diagnosis of 'salmon patch' conjunctival lesions.

Classification, diagnosis, and management of conjunctival lymphoma.

Lymphoma is a malignant lymphoproliferative tumor that can involve the conjunctiva. Approximately 5-15% of all extranodal lymphomas are found in the ocular adnexal region, with approximately 25% of those involving the conjunctiva. Ninety-eight percent of conjunctival lymphomas arise from B-lymphocytes. The most common subtype of conjunctival lymphoma is extranodal marginal zone lymphoma (80%), followed by follicular lymphoma (8%), diffuse large B-cell lymphoma (3%) and mantle cell lymphoma (3%). Natural killer and T cells (NK/T) are rare causes of lymphoma. While most conjunctival lymphomas are localized to the ocular adnexa at the time of presentation, systemic examination and management are of key importance in the long-term care of the patient. This review outlines the classification, etiology, presentation, diagnosis, and management of conjunctival lymphoma. The novel use of high resolution optical coherence tomography, both as a diagnostic tool and as a means for ongoing evaluation during treatment, is illustrated. Treatment options discussed include external beam radiation, chemotherapy, immunotherapy, antibiotic therapy, and combination regimens. Future investigation of the etiology and pathogenesis of conjunctival lymphoma is expected to reveal opportunities for innovative and individualized therapeutic agents. Collaboration between multiple disciplines is key in the advancement of the field.

Nuchal Nevus Flammeus and Alopecia Areata: When Size Matters.

INTRODUCTION: Alopecia areata (AA) is a high-prevalence immuno-mediated hair loss disorder. Extra follicular affections, including nail and ocular abnormalities, are classically related to a worse prognosis of the disease, and previous studies have suggested that the presence of a persistent nuchal nevus flammeus (NNF) also indicates a greater severity and duration of the disease. The association between AA and persistent NNF was first described by Hatzis et al in 1988, who demonstrated that the relation was statistically evident and not due to a simple observer bias. OBJECTIVES: To determine and compare the presence/absence and size of the NNF in 80 individuals (40 patients diagnosed with AA and 40 controls). RESULTS: We found a statistically significant association not only between AA and the presence of NNF, but also with its size. Moreover, we found that the size of the NNF was also associated with the severity of AA. CONCLUSION: The size of the NNF in AA patients might be a useful marker of widespread and chronic disease.

Ocular Adnexal Lymphoma Presenting with Visual Loss.

CONTEXT: Elderly patients with visual loss often have age-related macular degeneration, diabetic retinopathy, glaucoma, and cataract as common causes of visual loss. Other less common etiologies should be considered, especially in those presenting with systemic associations. CASE REPORT: The patient discussed in our review is an 80-year-old female, with a history of diabetic retinopathy and macular degeneration who presented with a sudden deterioration of vision. While this was initially attributed to diabetic retinopathy, she was eventually noted to have a salmon patch lesion in her conjunctiva, diagnosed on biopsy to be a diffuse large B-cell lymphoma. CONCLUSION: Because of the significant rate of disseminated disease among patients with lymphomas in the orbit that carries a worse prognosis, early diagnosis is essential to promote better overall survival of these patients. We describe here a patient diagnosed with conjunctival lymphoma associated with pronounced visual loss and review the literature on this subject.