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

• Published in: BMC ophthalmology Vol. 20; no. 1; p. 413
• Main Authors: Alsarhani, Waleed K, Alkhalifah, Muhannad I, Alkatan, Hind M, Alsolami, Afaf L, Maktabi, Azza M Y, Alsuhaibani, Adel H
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 16-10-2020; BioMed Central; BMC
• Subjects: Acids; Air bases; Antibiotics; Asthma; Bacilli; Biopsy; Case Report; Case reports; Chronic infection; Diabetes; Diabetes mellitus; Diabetic retinopathy; Diagnosis, Differential; Differential diagnosis; Enzymes; Gastrointestinal tract; Health aspects; Histopathology; Humans; Infections; Leukocytes (eosinophilic); Macrophages; Male; Methenamine; Middle Aged; Motility; Nodules; Ophthalmology; Pain; Patients; Polymerase chain reaction; Sarcoidosis; Scleral nodule; Scleritis; Stains & staining; Systemic diseases; Tropheryma; Tropheryma whipplei; Tuberculosis; Tumors; Uveitis; Uveitis - diagnosis; Whipple Disease - diagnosis; Whipple Disease - drug therapy; Whipple's disease; Scleritis; Whipple’s disease; Tropheryma whipplei; Scleral nodule
• ISSN: 1471-2415; 1471-2415
• DOI: 10.1186/s12886-020-01695-4

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.
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.
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.