Whipple's disease scleral nodules: a novel presentation in 2 consecutive patients

Abstract

Background: Whipple's disease (WD) is a rare, chronic, infection caused by gram-positive filamentous aerobic actinobacterium Tropheryma whipplei occurs classically in the gastrointestinal tract and shows histopathologically foamy macrophages with typical numerous PAS-positive, non-acid fast particles. Ocular WD in the form of uveitis may occur in the absence of systemic disease but has not been reported to present with scleral manifestation. We describe for the first time to the best of our knowledge 2 cases of scleral nodules with typical histopathological morphology of WD and without systemic involvement.

Case presentation: The first was a 53-year old diabetic male farmer who presented with 2 nontender right eye scleral nodules for 3 months, had a negative systemic workup, and surgical excision showed Periodic acid Schiff (PAS)-positive eosinophilic structures inside macrophages. Grocott's methenamine silver (GMS) stain and acid-fast bacilli (AFB) stain of the tissue itself were negative. The second case was a 60-year old male who presented with an asymptomatic superior scleral nodule for 4 months, which showed similar appearance and negative GMS and AFB stains.

Conclusion: WD should be included in the differential diagnosis of scleral nodules even in the absence of systemic symptoms. Surgical excision without systemic treatment resulted in successful outcome without recurrence.

Keywords: Scleral nodule; Scleritis; Tropheryma whipplei; Whipple’s disease.

Fig. 1

a and b The clinical appearance of the 2 round scleral nodules located superiorly and inferiorly surrounded by feeder vessels in case 1 (Phenylephrine drops were not used). c Ultrasound Biomicroscopy of case 1 showing homogenous hypo-reflective subconjunctival lesion overlying an area of scleral thinning. d Intra-operative photo demonstrating purulent material within the nodule

Fig. 2

a Gram-positive filamentous organisms within the purulent exudate (Original magnification × 1000-oil, Gram stain). b Histopathological photo of the tissue excised in case 1 clearly demonstrating the large amounts of Diastase-resistant intracytoplasmic organisms within foamy macrophages (Original magnification × 1000-oil, Periodic acid Schiff with diastase)

Fig. 3

a The clinical photo of the superior round subconjunctival nodule in case 2. Note the quiet surrounding conjunctiva. b Ultrasound bio-microscopy similarly showing homogenous subconjunctival lesion. c Similar numerous tiny intracytoplasmic organisms within macrophages and adjacent chronic inflammatory cells in the scleral tissue of case 2 (Original magnification × 400, Periodic acid Schiff)