ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyDrug Reaction DataReferencesInformation for PatientsView all Images (34)
Sarcoidosis - External and Internal Eye
See also in: Overview,Hair and Scalp,Oral Mucosal Lesion
Other Resources UpToDate PubMed

Sarcoidosis - External and Internal Eye

See also in: Overview,Hair and Scalp,Oral Mucosal Lesion
Contributors: Belinda Tan MD, PhD, Brandon D. Ayres MD, Christopher Rapuano MD, Harvey A. Brown MD, Sunir J. Garg MD, Lauren Patty Daskivich MD, MSHS
Other Resources UpToDate PubMed

Synopsis

Sarcoidosis is a granulomatous multi-system disease of uncertain etiology. Approximately one third of patients with sarcoidosis complain of mild fever (which can last for months), fatigue, and weight loss. Constitutional symptoms appear to happen more frequently in Asian and black individuals. Respiratory symptoms include a dry cough, dyspnea, chest pain, and tightness of the chest. Lymphadenopathy has a predilection for the axillary, cervical, epitrochlear, and inguinal locations. Splenomegaly is sometimes detectable. Salivary and lacrimal glands can be enlarged, and involvement of the heart, gastrointestinal tract, and upper respiratory tract may occur.

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.

Codes

ICD10CM:
D86.3 – Sarcoidosis of skin

SNOMEDCT:
31541009 – Sarcoidosis

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

Skin lesion differential diagnoses include the following:

Papules:
Nodules:
Plaques:
Eyelid granulomatous processes include chalazion, dermal TB, fungal infections, juvenile xanthogranuloma, and foreign bodies.

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:

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

Subscription Required

References

Subscription Required

Last Updated:10/25/2017
Copyright © 2021 VisualDx®. All rights reserved.
Sarcoidosis - External and Internal Eye
See also in: Overview,Hair and Scalp,Oral Mucosal Lesion
Sarcoidosis (Common Skin Lesions) : Face, Neck, Arms
Clinical image of Sarcoidosis
A close-up of a cluster of smooth reddish-brown papules arranged in an annulus.
Copyright © 2021 VisualDx®. All rights reserved.