masquerade syndromes are an important consideration in patients with refractory or atypical presentations of ocular inflammatory disease . here we describe an unusual case of a patient referred for evaluation of uveitis and scleritis who was found to have intraocular and bilateral extraocular involvement of necrobiotic xanthogranuloma along with coexisting chronic lymphocytic leukemia ( cll ) . a 53-year - old man was referred to our uveitis clinic for evaluation of iritis and scleritis in the left eye . the patient initially presented to his ophthalmologist 10 months earlier complaining of left eye pain and was treated for exacerbation of his existing dry eye syndrome . three months later , he returned to his ophthalmologist with intermittent bilateral eye pain ( left more than the right ) and was found to have nongranulomatous anterior uveitis in the left eye with associated fine keratic precipitates as well as diffuse anterior scleritis . laboratory workup and imaging was negative at that time and included a complete blood count , antinuclear antibody count , erythrocyte sedimentation rate , rheumatoid factor , anti - ssa , anti - ssb , lupus anticoagulant , antineutrophil cytoplasmic antibodies panel , and a chest x - ray . after oral prednisone was tapered off , his symptoms returned , and he was then referred to our clinic for further evaluation . at presentation , visual acuity was 20/20 ou with intraocular pressures of 17 mmhg od and 15 mmhg os . slit - lamp examination revealed mild injection of the left eye with stellate keratic precipitates and 25 cells per high - powered field . bilateral yellow conjunctival lesions were seen temporally in the bulbar conjunctiva ( figure 1 ) . conjunctival biopsy was scheduled . at a subsequent follow - up visit shortly after initial examination , the patient presented with new onset of decreased vision in the left eye and was found to have subretinal fluid and leakage at the choroidal level ( figure 2 ) . a second laboratory workup was ordered , including complete blood count , fluorescent treponemal antibody - absorption test , lyme disease titers , erythrocyte sedimentation rate , c - reactive protein , angiotensin converting enzyme level , hepatitis panel , and liver function tests . results were unremarkable except for a significantly elevated white cell count of 43.5 with 85% lymphocytes , an erythrocyte sedimentation rate of 42 , and a c - reactive protein of 27.5 . the immunophenotype and morphological features of his peripheral blood smear were suggestive of atypical cll . bilateral conjunctival biopsies were performed and revealed necrobiotic xanthogranuloma ( figure 3 ) with an adjacent cll component . necrobiotic xanthogranuloma is a rare progressive histiocytic disorder classically characterized by firm yellow plaques and nodules , most often found on the periorbital skin . there is a close association with paraproteinemia , and many patients have an igg- monoclonal gammopathy . hematologic malignancy in patients with necrobiotic xanthogranuloma in the form of multiple myeloma , leukemia , and lymphoma has been reported.1,2 the pathogenesis of necrobiotic xanthogranuloma is not well understood , but borrelia spirochetal infection has been proposed because organisms have been identified within the lesions.3 others have hypothesized that there is enhanced monocyte phagocytic activity leading to lipid accumulation and a subsequent giant cell inflammatory response in the skin.4 the relationship between the hematologic abnormalities and skin lesions is not currently understood . in one review of 48 cases of necrobiotic xanthogranuloma , ophthalmic complaints were reported in 50% of patients.5 ocular manifestations of necrobiotic xanthogranuloma include decreased vision , burning , pain , proptosis , ptosis , diplopia , uveitis , episcleritis , and scleritis.2,6,7 this case is unique because it is unusual for patients to develop ophthalmic manifestations of necrobiotic xanthogranuloma without the classic periorbital skin findings . treatment of necrobiotic xanthogranuloma is primarily targeted at the skin lesions and various methods have been tried with inconsistent results largely due to our limited understanding of the disease process . currently , the mainstays of treatment are systemic alkylating agents such as chlorambucil , cyclophosphamide , or melphalan given alone or in combination with systemic corticosteroids . interestingly , systemic chemotherapy for concomitant hematologic malignancies does seem to have a beneficial effect on the skin lesions in a few reported cases . this case highlights the need to include masquerade syndromes , like necrobiotic xanthogranuloma and cll , in the differential diagnosis of  idiopathic  uveitis or scleritis , especially when there are conjunctival or periocular skin lesions and an abnormal complete blood count .
this report describes a unique case of coexisting necrobiotic xanthogranuloma and chronic lymphocytic leukemia in a patient presenting with scleritis and uveitis . a 53-year - old caucasian man diagnosed with anterior uveitis and scleritis for the prior year 
 was referred to our uveitis clinic for further evaluation . 
 prior uveitis / scleritis workup performed by the referring ophthalmologist was negative . 
 examination demonstrated unilateral uveitis and posterior scleritis along with bilateral conjunctival lesions . 
 incisional biopsy of the conjunctiva was carried out . 
 the excised tissue was sent fresh for histopathologic studies . 
 slit - lamp examination revealed bilateral yellow - white bulbar conjunctival lesions , 1 + conjunctival injection ou , stellate keratic precipitates os with 25 cells per high - powered field . 
 funduscopic examination was significant for subretinal fluid os . 
 conjunctival biopsy revealed necrobiotic xanthogranuloma and chronic lymphocytic leukemia . 
 systemic workup demonstrated paraproteinemia consistent with necrobiotic xanthogranuloma and a complete blood count consistent with leukemia . 
 this case demonstrates an atypical presentation of necrobiotic xanthogranuloma and concomitant chronic lymphocytic leukemia presenting in the form of uveitis , scleritis , and conjunctival infiltration . 
 masquerade syndromes , such as necrobiotic xanthogranuloma and leukemia , must be kept in mind when treating patients with uveitis or scleritis with negative workups .