Conflicts of interest D.M.H. has received research funding from AstraZeneca, consulting income and honoraria from Genomic Health, research funding and honoraria from Centocor Ortho Biotec, and honoraria from Genentech. P.K. received remuneration for an advisory board for Genentech. J.V. was a member of an AstraZeneca advisory board. M.W.A. has received consultancy fees and research funding from AstraZeneca. J.L. received honoraria from AstraZeneca. B.M. was employed by AstraZeneca and owns stock in the 3 company. J.X. and K.B. are employees of AstraZeneca and own stock in the company.

No abstract

A 3-year-old boy was examined for a large nodular fasciitis in the upper eyelid. The lesion was confined to the preseptal plane of the upper eyelid, provoking mechanical ptosis. Excision by an eyelid crease approach was sufficient to restore normal eyelid position.

Purpose To describe an extremely uncommon outbreak of eye lesions in a specific area of the Brazilian Amazonia. Methods Prospective noncomparative case series. Fifty-nine patients who developed eye lesions after swimming in the Araguaia river of Tocantins state in Brazil were examined. A team of ophthalmologists equipped with a slit-lamp, gonioscopic lenses, and indirect ophthalmoscopy performed full eye examination. Analysis of the flora and fauna of the river water was undertaken by a group of experts. Results and Conclusions Eighty-three eyes were affected. The most common lesions were corneal opacities seen in 34 eyes and conjunctival nodules diagnosed in 12 eyes. Severe visual acuity loss was detected in seven children with unilateral anterior chamber lesions. Spicules of the sponge species Drulia uruguayensis and Drulia ctenosclera were found inside three blind eyes that have been enucleated for diagnostic purposes. All eye lesions could be attributed to an outbreak of foreign bodies from fresh water sponges. Organic enrichment of the water resulting from the absence of sanitation probably was the key factor, which initiated a cycle of ecological imbalance that provoked human disease.

A 28-year-old woman presented with progressive proptosis of the left eye. She had a history of familial thrombocytopenia and Hashimoto thyroiditis. CT of the orbits demonstrated a bilateral diffuse intraconal and extraconal infiltration. Biopsy from the left intraconal area revealed the typical histopathology of xanthogranuloma with a mixture of foamy histiocytes, Touton giant cells, and eosinophils. Systemic examination revealed a mediastinal and retroperitoneal infiltration with a focal lesion in the left kidney. A biopsy of the retroperitoneal area showed histopathology identical to that of the orbital lesion. A review of the literature indicated that the association between non-Langerhans histocytoses and immunologic dysfunctions is not uncommon. We hypothesize that Erdheim-Chester disease may be linked to an abnormal interaction between T-lymphocytes and macrophages similarly to the macrophage activation syndromes.