Granuloma annulare (GA) is a dermatologic condition of indeterminate etiology classically characterized by ringed erythematous plaques on the hands and feet corresponding to histopathological granulomatous inflammation.1
Reports of GA-associated uveitis include anterior uveitis (n = 4, one out of four with associated temporal arteritis), intermediate uveitis (n = 10, seven of 10 with associated retinal vasculitis), and panuveitis with chorioretinal lesions (n = 1, in a child).2–5 Although initial response to topical, systemic, and / or periocular steroids was often favorable, several patients had recurrent disease on attempted steroid taper, and those with intermediate uveitis tended to have persistent inflammation. Visual prognosis was good across all reports.
In this manuscript, we describe a case of ocular GA presenting as asymptomatic choroidal lesions in an adult. Institutional review board approval was not required for this single case report.
A 71-year-old white male developed a new floater in the right eye (OD). Visual acuity (VA) was 20/20 OD and 20/25 in the left eye (OS), with normal intraocular pressures and unremarkable examination OS. Examination OD revealed posterior vitreous detachment, trace vitreous white cell, and two minimally elevated, hypopigmented choroidal lesions (Figures 1A–1C). Optical coherence tomography (OCT) (Heidelberg Engineering, Heidelberg, Germany) (Figure 2) revealed deep, hyporeflective choroidal lesions, which were hyperfluorescent on late fluorescein angiography (FA) and hypocyanescent on indocyanine green (ICG) (Figure 3).
Color fundus photos at the time of presentation (A, B, C), 19 months later (D, E, F), 27 months later (G, H, I), and 6 weeks after posterior sub-Tenon's triamcinolone acetonide (J, K, L). Hypopigmented choroidal lesions can be seen superior to the disc and along the superotemporal arcade. Over time, the size of the lesions slowly increased, most easily seen with examination of the lesion along the superotemporal arcade (B, E, H). Following steroid treatment, the lesions appeared atrophic (J, K, L) and exhibited no further growth.
Spectral-domain optical coherence tomography of the hypopigmented lesions in the superotemporal macula (A) and superior to the disc (B) shows deep, hyporeflective choroidal lesions obliterating the normal vascular pattern that resolved 6 weeks after posterior sub-Tenon's triamcinolone acetonide (C, D).
Early (A) fluorescein angiography (FA) does not reveal evidence of the hypopigmented choroidal lesions, but very faint hyperfluorescence can be seen in the areas of the lesions on late (B) FA images. The lesions are readily visible as hypocyanescent areas on both early (C) and late (D) indocyanine green imaging.
Past medical history disclosed diabetes, hypertension, hyperlipidemia, benign multinodular thyroid goiter, hilar adenopathy with calcified granuloma secondary to histoplasmosis, and clinically diagnosed GA. Complete blood count, creatinine, electrolytes, calcium, erythrocyte sedimentation rate, C-reactive protein, antinuclear antibody, angiotensin-converting enzyme, muramidase, tuberculosis, syphilis, and Lyme testing were unremarkable. CT chest and abdomen showed known multinodular thyroid goiter and calcified lung granulomas. Colonoscopy and prostate cancer screening were up to date.
Examination remained stable for 1.5 years. At 19 months, the lesion borders grew (Figures 1D–1F). Given the lack of symptoms OD and new choroidal neovascularization (CNV) requiring anti-vascular endothelial growth factor OS, the choroidal lesions were observed for another 8 months. Slow growth continued (Figures 1G–1I). Serum protein electrophoresis and positron-emission tomography CT scan showed no malignancy.
With slow growth and posterior lesion location in the better-seeing eye, conservative management was preferred over choroidal biopsy. Given history of GA and unrevealing systemic workup, we suspected the lesions may be an ocular manifestation of this disease. Cutaneous biopsy confirmed the dermatologic diagnosis (Figures 4 and 5), and 20 mg posterior sub-Tenon's triamcinolone acetonide was given OD.
Color photos of the patient's left forearm (A) and right hand (B) show characteristic ring-shaped erythematous plaques, consistent with cutaneous granuloma annulare. The areas circled in black were biopsied.
Left forearm skin lesion biopsy shows in low power (A; hematoxylin-eosin stain; original magnification × 20) granulomatous inflammatory infiltrate involving the upper- and mid-dermis. Higher-power view (B; hematoxylin-eosin stain; original magnification × 200) reveals palisading of histiocytes and multinucleated giant cells around area of necrobiosis characteristic of granuloma annulare.
Six weeks later, the lesions appeared atrophic (Figures 1J–1L) with resolution on OCT. The patient remained asymptomatic with 20/20 VA OD. Examination remained stable 3 months later without further treatment.
To our knowledge, this is only the second case of GA associated with choroidal lesions. Although the etiology of GA is unknown, this patient had several conditions that may have put him at higher risk, including diabetes, hyperlipidemia, and multinodular thyroid goiter. Previous ocular associations with GA have been noted, including periocular disease, uveitis, and one child with panuveitis and concomitant chorioretinal lesions.2–7 This case, however, is unique in that the patient had only trace chronic vitreous cell and remained asymptomatic with slow growth of the choroidal lesions. The lesions in our patient appeared larger and more confluent than the multifocal, small, round chorioretinal lesions found in the 5-year-old child and were unilateral rather than bilateral.2 Unlike most of the patients in the intermediate uveitis series, our patient had no evidence of retinal vasculitis.5
Corticosteroids have shown some success in treating cutaneous and ocular GA, and this patient was no exception. His localized cutaneous lesions were managed periodically with topical corticosteroids, and the choroidal lesions became atrophic with resolution on OCT following a single dose of posterior sub-Tenon's triamcinolone acetonide.
The primary alternatives considered in the differential diagnosis were sarcoidosis and malignancy, especially lymphoma. Laboratory workup, imaging, prior investigation by a pulmonologist, and lack of other systemic findings during the course of 3 years made these alternative diagnoses unlikely. However, both lymphoma and sarcoidosis could present with yellow choroidal lesions that improve with steroid treatment, and annular cutaneous lesions mimicking GA can also be seen in sarcoidosis.8,9 Thus, cutaneous biopsy for histopathologic confirmation was particularly important in this case. Of course, biopsy of the choroidal lesions themselves or possibly of the vitreous cells could have further narrowed the differential diagnosis; but, given CNV in the fellow eye and the asymptomatic nature of the lesions, we felt that it would be inappropriate to subject the patient to this risk. Of note, although inflammatory eye disease could result in secondary CNV, CNV has not been previously reported in association with GA, and the patient's fellow eye had no signs of inflammatory disease. Therefore, CNV in this case was felt to be a result of age-related macular degeneration.
The patient has done well, without new systemic symptoms, visual disturbances, or discomfort related to these lesions 3 years after presentation and 4 months after treatment with sub-Tenon's corticosteroids. Although rare, ocular involvement of GA can affect the choroid, mimicking sarcoidosis or lymphoma, and a thorough workup is necessary to rule out such conditions. In the setting of appropriate systemic evaluation, GA should be considered in the differential diagnosis of hypopigmented choroidal lesions.
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