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

A 53-year old Saudi diabetic male farmer presented to our ophthalmology clinic with 2 subconjunctival scleral nodules in his right eye for 3 months. The nodules were stable in size, and the patient did not complain of any ocular symptoms until his recent presentation. His past medical history was significant for diabetic foot, for which he underwent above knee amputation and was receiving antidiabetic medications. He denied history of trauma, weight loss, joint pain, diarrhea, abdominal pain and any neurological symptoms. Visual acuity was 20/25 in both eyes, and the extraocular motility was full without nystagmus or signs of oculomasticatory myorhythmia. There was two nontender scleral nodules located superiorly and inferiorly in the right eye (Fig. 1a and b). The nodules were surrounded by dilated vessels while the remaining conjunctiva was quiet. The anterior chamber was deep and quiet with mild iris neovascularization in the right eye and dot blot hemorrhages and microaneurysms in all quadrants on posterior pole exam. Examination of the left eye was unremarkable except for severe non-proliferative diabetic retinopathy. Ultrasound bio-microscopy (UBM) showed homogenous subconjunctival lesions (Fig. 1c). Systemic workup including tuberculin skin test (TST), chest X-ray was, rheumatoid factor, antineutrophil cytoplasmic antibodies, and angiotensin converting enzyme was negative. The patient was booked for excisional biopsy with conjunctival advancement by an experienced ophthalmologist. Intraoperatively the nodule was filled with pus (Fig. 1d) with underlying scleral melting, but no areas of scleral perforation were noted to necessitate the use of scleral patch or amniotic membrane transplant. A sample obtained from the nodule was sent for microbiological assessment which revealed numerous gram-positive filamentous organisms (Fig. 2a). The histopathology of the excised scleral tissue showed fibrosis and lymphoplasmacytic cells infiltration with foamy macrophages (Fig. 2b). Periodic acid Schiff (PAS) stain with/and without diastase demonstrated numerous tiny eosinophilic structures inside the macrophages that represent Trophyrema whipplei. Grocott’s methenamine silver stain (GMS), Gram and acid-fast bacilli (AFB) stains were negative. Histopathology slides were reviewed by three different pathologists. After discussion with the internal medicine and infectious disease specialists and since the patient did not develop any systemic features, a decision not to start systemic antibiotics was taken. However, post-operative topical Ofloxacin 0.3% drops QID and Prednisolone acetate 1.0% drops QID with tapering dose over 1 month were used in the operated eye as standard topical medications following such a procedure. There was no recurrence over 6 months of follow up.

A 60-year old Saudi male presented with a superior scleral nodular swelling for 4 months with no change in size over time. Past medical history was unremarkable except for asthma. He denied history of trauma, weight loss, joint pain, diarrhea, abdominal pain and any neurological symptoms and his drug history was unremarkable. On physical examination, visual acuity measured 20/30 and 20/25 in in the right and left eyes, respectively. There was a nontender round subconjunctival nodule located superiorly measuring 13 × 7 mm in size (Fig. 3a). Dilated fundus examination was within normal limits in both eyes. Examination of the other eye was unremarkable. Extraocular motility was full without nystagmus or signs of oculomasticatory myorhythmia. UBM showed a hyporeflective homogenous subconjunctival nodule (Fig. 3b). Systemic workup including TST, chest X-ray was, rheumatoid factor, antineutrophil cytoplasmic antibodies, and angiotensin converting enzyme was negative. The patient was evaluated by an internist, who stated the patient did not have any systemic condition except asthma.

The patient was booked for incisional biopsy by his treating ophthalmologist. Histopathology results showed fibrotic scleral tissue with chronic inflammatory cells infiltration including lymphocytes, plasma cells, and focal foamy macrophages showing similar numerous tiny PAS- positive structures (Fig. 3c). GMS, Gram and AFB stains were also negative. Histopathology slides were reviewed by three different pathologists. The diagnosis was made after the patient’s discharge, and on follow up visits, the patient showed satisfactory results with the use of topical antibiotics and Prednisolone acetate drops with tapering dose in the operated eye similar to the first case. He was also monitored for any systemic features or recurrence. Eventually, there was no recurrence over 4 months of follow up. Systemic antibiotics were not started since the patient did not develop any systemic or other ocular signs, and the scleral nodules did not recur.