Sarcoidosis - External and Internal Eye
About a quarter to one half of patients with systemic disease develop eye involvement. Patients with ocular sarcoid may complain of double, decreased, or blurry vision. There are 2 ocular syndromes: Heerfordt syndrome, with uveitis, parotid enlargement, and papilledema; and Löfgren syndrome, with erythema nodosum, bilateral hilar adenopathy, and arthralgias.
Uveitis of the eye can present with photophobia, redness of the eye, and cloudy vision. Complications from uveitis include cataracts, glaucoma, posterior synechiae, corneal band keratopathy, and iris nodules. Retinal neovascularization and optic nerve damage are also possible with sarcoidosis. Granulomas of the eyelids, lacrimal glands, conjunctiva, iris, retina, and choroid can occur. Rarely, there can be granulomatous inflammation of the extraocular muscles leading to diplopia, which may be the presenting sign of sarcoidosis. May occur in tattoos.
Some drugs and exposures have been associated with the development of sarcoidosis and sarcoid-like granulomatosis. Patients undergoing antiviral therapy for chronic hepatitis C – both monotherapy with interferon (IFN)-alpha and combination therapy with IFN-alpha and ribavirin – have developed new-onset sarcoidosis or experienced re-activation of pre-existing sarcoidosis during or shortly after treatment. The disease typically manifests as pulmonary and/or cutaneous sarcoidosis and follows a benign course, resolving spontaneously or within months after antiviral treatment is completed. More complicated multisystem cases, eg, involving the CNS, have been reported. The use of systemic corticosteroids to treat sarcoidosis in such patients should be considered with caution due to their adverse effects on viral loads. In addition, there have been an increasing number of reports of new-onset sarcoidosis manifesting in patients who are receiving anti-tumor necrosis factor (TNF)-alpha therapy (etanercept, infliximab, adalimumab), eg, for a rheumatologic diagnosis. This is paradoxical, because TNF-alpha inhibitors have been used to treat sarcoidosis with some success. Disease typically resolves with discontinuation of the drug and steroid therapy. It has been reported that exposure to moderate to high levels of silica increases the risk for sarcoidosis. This association is more common in males due to occupational exposure.
D86.3 – Sarcoidosis of skin
31541009 – Sarcoidosis
- Tuberculosis (TB)
- Lichen planus
- Tuberous sclerosis (adenoma sebaceum)
- Lupus erythematosus
- Secondary syphilis
- Acne rosacea
- Granuloma annulare
- Necrobiosis lipoidica
- Leishmaniasis (Old World and New World)
- Combined variable immunodeficiency can have cutaneous and systemic sarcoid-like lesions.
Orbital granulomatous differential diagnoses are lipogranulomas, granulomatosis with polyangiitis, TB, syphilis, fungal infections, parasites (eg, microfilariae, nematode larvae), and panophthalmitis.
The differential diagnosis of granulomatous uveitis includes the following:
- Infectious bacterial agents, such as TB, cat-scratch disease, syphilis, brucellosis, leprosy, and leptospirosis.
- Protozoan infections, such as amebiasis, toxoplasmosis, and trypanosomiasis.
- Fungal infections, such as actinomycosis, aspergillosis, blastomycosis, candidiasis, coccidioidomycosis, cryptococcosis, histoplasmosis, mucormycosis, nocardiosis, and sporotrichosis.
- Helminth infestations, such as Ascaris, cysticercosis, taeniasis, Diptera larvae, and nematode infestations (onchocerciasis and Toxocara, Ancylostoma, and Necator species).
- Viral uveitis, such as herpes simplex virus (HSV), herpes zoster, vaccinia, and cytomegalovirus (CMV).