Simultaneous presentation of Kimura disease and angiolymphoid hyperplasia with eosinophilia

Kimura disease and angiolymphoid hyperplasia with eosinophilia are rare disease entities that can manifest as subcutaneous nodules, plaques, or papules of the head and neck, and less commonly the orbit and ocular adnexa. The merits of each entity have been debated with regard to whether they are truly individual or are separate points on a single spectrum. Current opinion favors the former. This clinicopathologic report of a patient who presented with a right lower eyelid mass with pathologic features consistent with Kimura disease and a left conjunctival mass with features consistent with angiolymphoid hyperplasia with eosinophilia poses a challenge to the notion that these are distinct conditions.     

Angiolymphoid hyperplasia with eosinophilia of the eyelid with spontaneous regression

A 53-year-old Chinese man presented with a painless left upper eyelid lump of 2 weeks' duration. Histopathology was consistent with angiolymphoid hyperplasia with eosinophilia, and also revealed neutrophilic infiltrate secondary to ulceration. After incisional biopsy, the residual lesion, which was not treated, developed surface keratinization over the next 2 weeks and sloughed off, leaving intact epidermis and no scar. This case illustrates that observation awaiting spontaneous regression may be considered prior to surgical and other therapeutic interventions. The authors also briefly review the literature on angiolymphoid hyperplasia with eosinophilia and Kimura disease, with emphasis on ocular involvement.     

Orbital angiolymphoid hyperplasia with eosinophilia. Presentation as chalazion

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon, benign slow-growing lesion primarily found in the head and neck region, with onset between the third and fourth decades, and a female predominance in the nonOriental population. Kimura's disease, occurring primarily in young Oriental males, is a similar lesion with peripheral blood eosinophilia, regional lymphadenopathy, and occasional nephrotic syndrome. Orbital ALHE is rare, previously reported in only nine patients. Orbital ALHE is a solitary lesion, with an older age of onset, between the fourth and eighth decades, than ALHE elsewhere. There is a male predominance, in contradistinction to ALHE elsewhere in nonOrientals. Orbital ALHE demonstrates the same histology as other locations, with exuberant capillary proliferation and an inflammatory infiltrate of eosinophils, lymphocytes, plasma cells, and mast cells. The maturity of the lesion, not necessarily the duration, determines the presence of lymphoid follicles and germinal centers. A distinctive endothelial cell lines and even extends into the vascular lumens. Peripheral blood eosinophilia occasionally occurs, and a few cases have been reported in conjunction with asthma. Therapeutic regimens for ALHE include excision, carbon dioxide and argon laser, irradiation, steroids, electrodesiccation, and cytotoxic agents. Orbital lesions have been treated successfully with excision, or biopsy with debulking, or steroids.     

Conjunctival eosinophilia in atopic and non-atopic external eye symptoms.

We studied the occurrence in an ophthalmological office practice of conjunctival eosinophilia in 333 normal subjects (mean age 26 years) and in 152 atopic and 484 non-atopic patients (mean age 40 years) with various external eye symptoms. Eosinophils were present in 131/636 patients (21%) and in 22/333 asymptomatic normal subjects (7%). In those patients with conjunctival eosinophilia, a history of some kind of atopic condition was obtained only in 53/131 (40%). When the patients were characterized according to the presence of both eosinophilia and atopy, eosinophilia without signs of atopy was observed in 7-15% in different diagnostic groups. Patients with conjunctivitis or blepharoconjunctivitis had a fourfold risk for eosinophilia compared to normal subjects (odds ratios 4.52 and 4.09, confidence intervals 2.02, 10.12 and 2.40, 6.99), when atopy, sex, age, time of the examination and presence of bacteria were included as potential confounders in the regression model. We conclude that a considerable portion of patients with external eye symptoms, and especially with various forms of conjunctivitis, show eosinophilic inflammation without any evidence of atopic background. They can be labelled as having 'intrinsic' eosinophilic conjunctivitis analogously to the classification used in patients with intrinsic rhinitis and asthma. The etiology of this common nonatopic syndrome remains to be established.     

Methotrexate as an Alternative Treatment for Orbital Angiolymphoid Hyperplasia with Eosinophilia

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon, benign disorder that presents as solitary or multiple nodules in the dermis or subcutaneous tissue. Orbital ALHE has been reported on occasion. We report a case of orbital ALHE which was refractory to systemic steroids but effectively treated with low-dose subcutaneous methotrexate. To our knowledge, this is the first reported case of methotrexate as a successful treatment for refractory ALHE.     

Toxocara canis endophthalmitis with low ELISA titer

A five-year old boy had clinical findings consistent with ocular toxocariasis, blood eosinophilia, and repetitive negative (titer 1:2) ELISA. The eye was enucleated and larval fragments consistent with Toxocara were present within an eosinophilic granuloma in the vitreous cavity overlying a chorioretinal scar entrance site.     

Angiolymphoid hyperplasia with eosinophilia involving the lacrimal gland: case report.

A case is described of a 25-year-old male who developed angiolymphoid hyperplasia with eosinophilia involving the tissue over the left parotid gland and subsequently involving the right lacrimal gland with proptosis. Treatment was by excision.     

Kimura disease of the eyelid in an Indian man

Kimura disease is a rare idiopathic chronic inflammatory disease, characterized by subcutaneous nodular lesions in the head and neck area. Ophthalmic manifestation of Kimura disease involves orbital and eyelid lesions mostly in Asian patients, but it has been described in White patients and Black Caribbean patients. Kimura disease is usually associated with eosinophilia and occasionally with renal disease. Here, we report a case of Kimura disease of the eyelid in a 50-year-old Indian man with eosinophilia. The main differential diagnosis was angiolymphoid hyperplasia with eosinophilia. Histology is crucial to separate these two entities, and our case was shown to be Kimura disease by histology. To our knowledge, this is the first report of a person of Indian origin to develop Kimura disease involving the eyelid.     

Unusual presentation of ocular toxocariasis in friends.

Two cases of presumed ocular toxocariasis are reported in 19-year-old college students. One presented with an acute exudative intraocular inflammation, with marked peripheral eosinophilia, while the other had only minimal inflammatory changes of the disc and vitreous. Both had elevated Toxocara antibody titers by the enzyme-linked immunosorbent assay (ELISA) method. The simultaneous occurrence of ocular toxocariasis in friends and the early presentation and detection of their ocular lesions make these 2 cases interesting. The presence of positive laboratory data with only minimal clinical disease in one of the cases suggests that ocular toxocariasis may be more common than is usually appreciated.     

Churg-Strauss vasculitis presenting with severe visual loss due to bilateral sequential optic neuropathy.

A 44-year-old man with severe visual loss due to an acute bilateral sequential optic neuropathy is described, where the associated pulmonary disease and peripheral eosinophilia led to a diagnosis of Churg-Strauss syndrome (allergic angiitis). The mechanism of the optic neuropathy was most probably acute ischaemia of the anterior optic nerve due to direct involvement of the short posterior ciliary arteries by inflammatory disease of the vessel wall.     

Angiolymphoid hyperplasia with eosinophilia (Kimura's disease) of the orbit and ocular adnexa

We conducted a clinicopathologic study of eight cases of angiolymphoid hyperplasia with eosinophilia (Kimura's disease). The orbit was involved in five patients and the ocular adnexa (eyelid and inner canthus) in three. The median age of the six men and two women was 51 years (range, 38 to 72 years). The lesions were characterized by an abnormal proliferation of small vessels lined by plump endothelial cells, associated with a chronic inflammatory infiltrate with prominent tissue eosinophilia and scattered lymphoid follicles. Serial sectioning of two tumors disclosed that these small vessels apparently originated within the walls of arteries. Immunohistochemistry disclosed factor VIII-related antigen within the endothelial cells in two of the four lesions studied. Ultrastructurally, some of the capillaries showed prominent multilaminar basement membranes surrounding their walls. The apical surface of the markedly swollen endothelial cells disclosed broad villous processes protruding into the lumen. Although this vascular lesion can be mistaken for angiosarcoma, it is considered benign. There was no evidence of recurrences of any of the six of the seven lesions that were completely excised and followed up (follow-up periods ranged from five to 95 months). Complete surgical excision appears to be the best treatment.     

Role of TGF-β in tissue eosinophilia associated with vernal keratoconjunctivitis

To determine the role of TGF-β₁ in the tissue eosinophilia associated with vernal keratoconjunctivitis (VKC), we investigated the immunohistochemical expression of TGF-β₁ and TGF-β₁-related proteins in giant papillae obtained from VKC patients. We also investigated the effect of TGF-β₁ on production of eotaxin by cultured conjunctival and corneal fibroblasts using ELISA. Finally, the effects of glucocorticoids, cyclosporine, and tacrolimus on eotaxin production by corneal fibroblasts were assessed. Our investigations revealed that eosinophils expressing TGF-β₁ and TGF-β₁-related proteins (such as phosphorylated Smad2, integrin αvβ₆, α-smooth muscle actin, type I procollagen, and tenascin-C) were expressed in the giant papillae. TGF-β₁ and IL-4/IL-13 caused a synergistic increase of eotaxin production in cultured conjunctival and corneal fibroblasts. This effect of TGF-β₁ and IL-4/IL-13 was inhibited by glucocorticoids, but neither by cyclosporine nor by tacrolimus. In conclusion, TGF-β₁ has an important role in the tissue eosinophilia associated with VKC.     

Idiopathic Inflammatory Orbital Pseudotumor in Childhood: II. Results of Diagnostic Tests and Biopsies

Twenty-nine patients with pediatric orbital pseudotumor underwent a wide variety of diagnostic tests including biopsies. The following abnormalities were discovered: peripheral blood eosinophilia (9/29 patients); elevated ESR (17/17); elevated antinuclear antibody titres in the Tolosa-Hunt variant (2/2); hypercomplementemia (2/3); and mild CSF pleocytosis (2/6). Thyroid function tests were normal in nine patients so studied. B-mode ultrasonography performed on 12 patients displayed abnormalities in all cases (some combination of Tenonitis, myositis, perioptic inflammation, or mass effect). Computed tomography in seven patients provided higher resolution confirmation of these findings. Orbital bone changes and serious sinus disease were absent on routine radiographic studies. Biopsies performed on 16 patients disclosed mild lymphocytic inflammation in all cases, fibrosis and tissue eosinophilia in 9 biopsies (6 correlating with peripheral blood eosinophilia). Nine biopsies demonstrated a lipogranulomatous response to damaged fat cells. A true vasculitis or extensive lymphoid hyperplasia was not identified in any biopsy specimen.     

Lymphangiomatous hyperplasia with eosinophilia; Kimura's disease (author's transl)]

Kimura's disease is a benign affection of young people in which there are non-specific subcutaneous nodules, mainly found in the region of the head. They can be identified by their particular histological appearance which includes vascular and inflammatory components, and by local and sometimes blood eosinophilia. The etiology is not known but could be allergic in nature.     

Angiolymphoid hyperplasia with eosinophilia of the lacrimal gland: a case report

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare clinicopathologic entity that has been the subject of considerable confusion and debate. ALHE shares both clinical and histopathologic features with Kimura disease (KD). Because of this overlap, ALHE and KD have been used synonymously in the Western medical literature, as they were thought to represent variations of the same disease. Some pathologic reports have called for distinguishing ALHE and KD as two separate nosological entities, based on their clinical, laboratory, and especially histopathologic findings. ALHE commonly affects women in the third and fourth decades, with head and neck involvement. ALHE has been described rarely in the orbit, eyelids, and lacrimal gland. We report a case of ALHE involving the left lacrimal gland in a 40-year-old Mexican woman. Because ALHE can cause proptosis, lid swelling, ocular dysmotility, or a palpable mass, it should be considered in the differential diagnosis of orbital lesions occurring in adults.     

Conjunctival involvement in Churg-Strauss syndrome

PURPOSE: To describe an unusual case of Churg-Strauss syndrome (CSS) that presented with a conjunctival nodule and was successfully treated with oral corticosteroids. METHODS: Case report. RESULTS: A 30-year-old woman with a history of adult-onset asthma, seasonal allergies, and a lung mass presented with a nodular elevation of the conjunctiva. Excisional biopsy demonstrated necrotizing eosinophilic granulomas. Systemic evaluation revealed peripheral eosinophilia and elevated IgE, consistent with Churg-Strauss syndrome. The patient's symptoms and eosinophilia resolved after an increase in the dose of oral corticosteroids. CONCLUSIONS: This case describes a unique presentation of conjunctival involvement in CSS that differs from prior case reports by having a small, well-demarcated nodule and lacking signs of active inflammation.     

Bilateral Kimura''s disease of the eyelids.

A case of Kimura's disease affecting the eyelids bilaterally is reported in a 5-year-old boy of Afro-Caribbean extraction who has been followed for 12 years with repeat biopsies. He initially presented at 5 years of age with swelling of the left upper eyelid, left cervical lymphadenopathy, and eosinophilia. One year later he developed swelling of the right upper eyelid. There has been no change in the clinical appearance over the next 12 years. Repeated biopsies of the eyelids showed a diffuse inflammatory infiltrate with many eosinophils and lymphocytes. A lymph node biopsy showed reactive lymphoid hyperplasia. Immunohistochemistry using lymphoid markers showed a polyclonal pattern. Kimura's disease is a rare cause of eyelid swelling, particularly at such a young age and with bilateral involvement. This case demonstrates that bilateral orbital lymphoid lesions with cervical node involvement do not always imply lymphoma, but may have a benign pathogenesis. The unusually long follow up in this case confirms an excellent prognosis for Kimura's disease with conservative management. Accurate diagnosis in small orbital biopsies may spare the patient unnecessary radical surgery.     

Angiolymphoid hyperplasia with eosinophilia should not be confused with Kimura's disease

PURPOSE: To emphasize the differences between angiolymphoid hyperplasia with eosinophilia (ALHE) and Kimura's disease (KD), two entities often confused in the ophthalmic literature. METHODS: Case report of a subcutaneous mass in the periocular region of a 45-year old woman and a MEDLINE review of the ophthalmic and non-ophthalmic literature on ALHE and KD. RESULTS: The clinical and histopathological findings of the present case were consistent with ALHE, but not with KD. A survey of the current ophthalmic literature indicates that these two designations are still often used synonymously, despite that non-ophthalmic papers now separate ALHE from KD. CONCLUSION: The clinical and histopathological features of ALHE are most often distinctly different from KD and these entities should be clearly separated in the ophthalmic literature.     

Unilateral iritis by cysticercal larva in the anterior chamber

An 8-year-old girl complained of progressive pain, visual loss and photophobia in her right eye for 1 week. Treatment with local steroids for acute iritis had not been successful. Slitlamp examination revealed a marked cellular and fibrinous inflammatory reaction of the anterior chamber and a whitish, fibrin-like structure on the surface of the iris. There was mild serum eosinophilia and leucocytosis. The suspected organic material was removed surgically. By light and electron microscopy, parts of the wall of a helminthic parasite were discovered, anatomically consistent with the diagnosis of an immature stage of Taenia solium found in cysticercosis. Such cases of intra-ocular parasitosis are described very rarely but might still occur, although there is no history of suspicious typical exposition to parasites. It is important to know that eosinophilia may be insignificant or even absent and that complete surgical removal will be the only successful treatment.     

Extracellular deposition of eosinophil major basic protein in orbital histiocytosis X

Although eosinophils are prominent in orbital lesions of patients with histiocytosis X (Langerhans' cell histiocytosis), little is known of their pathogenic significance in the disease. To determine whether eosinophils degranulate and deposit toxic proteins in orbital histiocytosis X, the authors examined lesions by indirect immunofluorescence for localization of the core granule protein (major basic protein) outside of eosinophils. Four patients with histiocytosis X were studied: three with eosinophilic granuloma and one with Hand-Schüller-Christian disease. Tissue eosinophilia was prominent in all specimens; striking extracellular deposition of eosinophil major basic protein was noted in three patients, and focal deposition was present in the fourth patient. Orbital specimens obtained at autopsy from patients without orbital disease were studied as control specimens; no tissue eosinophilia or deposition of eosinophil major basic protein was observed. These findings indicate that eosinophils likely degranulate in lesions of orbital histiocytosis X and may participate in the pathogenesis of the disease.