From the Departments of Ophthalmology and Pediatrics, Vanderbilt University School of Medicine, Nashville, Tennessee.
The authors have no financial or proprietary interest in the materials presented herein.
Address correspondence to David Morrison, MD, Department of Ophthalmology and Visual Sciences, 8000 Medical Center East, Nashville, TN 37232-8808.
The management of uveitis in children can be challenging. Many systemic associations exist and symptoms may not present concurrently with the ocular inflammation. The following case describes a rare cause of pediatric uveitis associated with a benign dermatitis.
A healthy 5-year-old boy presented with a 2-week history of redness, photophobia, and right eye pain. Additional history was notable for a tick bite on the scalp 4 months previously, although no subsequent rash was noted. The patient’s family history was significant for leukemia in an aunt and tuberculosis exposure in several family members. The patient was initially seen by a community ophthalmologist and diagnosed as having acute, nongranulomatous anterior uveitis. Prednisolone drops were given four times daily with no apparent improvement in symptoms.
On initial examination at our institution, the patient’s uncorrected visual acuity was 20/25 in both eyes. Intraocular pressures were normal. Slit-lamp examination of the anterior segment was significant for 2+ cell and flare bilaterally. The dilated fundus examination demonstrated mild optic disc edema and creamy chorioretinal lesions bilaterally (Fig. 1). There was 1+ vitreous cell.
Figure 1. Color Fundus Photographs of the (A) Left Eye and (B) Right Eye Demonstrating Chorioretinal Lesions.
The initial working diagnosis was nongranulomatous panuveitis. Results of erythrocyte sedimentation rate, antinuclear antibody, rheumatoid factor, and rapid plasma reagin tests were negative. Complete blood cell count, metabolic panel, serum angiotensin converting enzyme, lysozyme, and calcium were within normal limits. Borrelia and Toxoplasma antibody titers were negative. A chest X-ray was normal. Topical prednisolone was increased to hourly and a short course of 1 mg/kg of oral prednisone was given. The choroidal lesions resolved but the anterior uveitis persisted, requiring intermittent topical prednisolone.
Approximately 4 months after initial presentation, the patient was noted to have skin lesions on his ankles and hands (Fig. 2), which were diagnosed by the pediatric dermatology service as granuloma annulare. This is an idiopathic disorder characterized by flesh-colored to erythematous papules arranged in a ring-like pattern.1
Figure 2. Granuloma Annulare Lesion of the Hand.
Due to the continued anterior uveitis and new skin lesions, the child was given methotrexate for long-term immunosuppression. The granuloma annulare resolved and his eyes have remained quiet during 2 years of follow-up.
Granuloma annulare is a benign, self-limited inflammatory dermatosis characterized clinically by dermal papules and plaques in an annular configuration. It is distinguished histologically by palisading granulomas with central connective tissue degeneration and mucin. The cause of granuloma annulare is currently unknown, although proposed pathogenic mechanisms, currently without sufficient supporting evidence, include cell-mediated immunity (type IV), immune complex vasculitis, and an abnormality of tissue monocytes.1 These lesions have also been suggested as a precursor to sarcoidosis.2
To date in the literature, there have been nine cases of granuloma annulare-associated uveitis reported. Eight patients with intermediate uveitis and granuloma annulare were reported as a retrospective case series in 2003.3 One other article in 2003 described a case of anterior uveitis and granuloma annulare in a 51-year-old woman.4 Our case is unique because this is the youngest patient ever reported and a panuveitis was present rather than intermediate uveitis. Additionally, choroidal lesions have not been previously reported, although retinal vasculitis is known to occur.
- Howard A, White CR. Granuloma annulare. In: Bolognia JL, Jorizzo JL, Rapini RP, eds. Dermatology,1st ed. Vol. 2. London: Mosby; 2003:1460–1463.
- Umbert P, Winkelmann RK. Granuloma annulare and sarcoidosis. Br J Dermatol. 1977;97:481–486. doi:10.1111/j.1365-2133.1977.tb14123.x [CrossRef]
- van Kooij B, van Dijk MC, de Boer J, Sigurdson V, Rothova A. Is granuloma annulare related to intermediate uveitis with retinal vasculitis?Br J Ophthalmol. 2003;87:763–766. doi:10.1136/bjo.87.6.763 [CrossRef]
- Oz O, Tursen U, Yildirim O, Kaya TI, Ikizoglu G. Uveitis associated with granuloma annulare. Eur J Ophthalmol. 2003;13:93–95.