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Granuloma faciale is an eosinophilic dermatosis that typically presents as red-brown facial plaques of middle-aged white men, while eosinophilic angiocentric fibrosis is a histologically similar fibrotic condition affecting the respiratory mucosa. Both are very uncommon conditions and pose a therapeutic challenge. While a handful of reports have reported concurrent presentation, the authors present the first case of eyelid granuloma faciale with eosinophlilic angiocentric fibrosis.

Considering the immune system dysfunction in unifocal orbital eosinophilic granuloma; possible complications of surgery, intralesional steroid, and radiotherapy; immunomodulatory and cytoreductive effects of interferon-α2b; and its safety profile in treatment of ocular surface neoplasia, intralesional interferon-α2b was first commenced in treatment of 3 cases (8-, 25-, and 43-year-old men) with biopsy proven orbital eosinophilic granuloma (2007-2014). Three intralesional injections of interferon-α2b (1.5 million units/0.5 ml) were given every other day. Cases 1 and 3 showed a rapid response with no recurrence in 86 and 29 months follow up. Case 2, however, required a second course of injection 2 months after the first one due to a partial response to the first injections with no recurrence at last follow up (57 months). Transient flulike symptom was the only side effect that was observed in Cases 2 and 3.

A 43-year-old man reported right visual loss after biopsy of an intranasal mass, which improved after treatment at another hospital. On first examination at our hospital, his visual acuity was 1.2 OD, and MRI showed a right intraorbital mass involving the ethmoid sinus. His visual acuity decreased to 0.08 OD 12 days after orbital biopsy, with right globe tenting shown by MRI. An emergent lateral canthotomy and cantholysis were performed. The histopathological diagnosis was eosinophilic angiocentric fibrosis, and treatment with intravenous methylprednisolone (125 mg/day) and oral diaminophenyl sulfone (75 mg/day) was started immediately after surgery. One month later, the diaminophenyl sulfone was discontinued and cyclophosphamide (50 mg/day) was started. The methylprednisolone and cyclophosphamide doses were tapered over 9 months and 7 months, respectively. At the 1.5-year follow-up examination, his visual acuity was 1.0 OD. The lesions did not grow in size.

The presentation of RDD as an anterior subperiosteal orbital mass with bone involvement has, to the authors' knowledge, not been previously reported. We describe a case of Rosai-Dorfman disease (RDD) presenting as an anterior superolateral subperiosteal orbital mass with erosion of overlying bone mimicking eosinophilic granuloma. It was debulked using endoscopic-guided curettage and the patient was given both local and systemic corticosteroids. Careful histological analysis revealed the diagnosis of RDD and the patient remains asymptomatic and recurrence free at 16 months follow-up. Involvement of the pituitary gland, a recognized yet unusual finding in this condition was also noted. RDD should be considered in the differential diagnosis of a soft tissue mass of the superolateral orbit.

A 30-year-old woman complained of right-sided epiphora for 2 years. She also reported diplopia on certain gaze positions and felt a hard mass behind the right medial inferior orbital rim. Magnetic resonance imaging studies demonstrated a relatively well-delineated mass in the right inferomedial orbit with minimal ethmoid sinus involvement. Histopathological evaluation following a large incisional biopsy showed massive eosinophilic infiltration and fibrosis with the final diagnosis of eosinophilic angiocentric fibrosis. She was then discovered to have significant peripheral eosinophilia and elevated serum IgE levels and clinical findings of allergic rhinitis and sinusitis. She was treated with systemic fluorocortolon and desloratadin for 4 months. She remained stable without recurrence for 32 months. The patient with this exceptionally rare tumour of the orbit benefited from debulking surgery followed by systemic corticosteroids and antihistaminics.

We present clinical, radiological, and histopathological findings and treatment results of two infants with orbital eosinophilic granuloma. Both patients presented at 1 year of age with unilateral proptosis, periorbital edema, and a superotemporally located, ill-defined orbital mass on computed tomographic (CT) imaging, with bone erosion in one patient. After subtotal excision, histopathological examination revealed eosinophilic granuloma, and the proptosis resolved with no sign of orbital tumor recurrence or systemic involvement at 12 and 23 months follow-up. Eosinophilic granuloma is a rare childhood orbital tumor that may or may not present with osteolytic change.

The authors report a case of eosinophilic granuloma involving the roof of the orbit in a 3-year-old girl. The clinical presentation and the imaging features suggested a malignant tumor and the final diagnosis was obtained by biopsy with histopathologic examination. Despite its alarming radiologic appearance, there was spontaneous healing of the eosinophilic granuloma.

The authors report a case of eosinophilic granuloma involving the roof and the lateral wall of the left orbit in a 5-year-old boy. The clinical presentation and especially the imaging features (computed tomography and MRI) suggested a malignant tumor and the final diagnosis was obtained by fine needle aspiration biopsy with histopathologic examination. Despite its alarming radiologic appearance, there was spontaneous healing of the eosinophilic granuloma with restitution ad-integrum of the bone.

Purpose: To describe the findings and outcomes in eosinophilic granuloma (unifocal Langerhans-cell histiocytosis [LCH]) of the orbit and to explain the paradox of aggressive bone destruction responsive to minimal intervention., Methods: Retrospective, consecutive, interventional case series of patients treated from 1985 to 2001. Minimum inclusion criteria were demonstration of CD1a positivity or Birbeck granules, treatment by a single surgeon, systemic evaluation by a pediatric oncologist, and follow-up of 12 months. A pathogenetic construct was assembled from general LCH concepts and the specific orbital findings., Results: Seven patients met study criteria. All were male, 2 to 16 years of age. All had eyelid or forehead swelling and osteolytic defects, with symptoms of 2 to 6 weeks' duration. All underwent incisional biopsy, with frozen-section examination suggestive of LCH in 6 of 7 cases. The 2 earliest patients received low-dose irradiation after simple biopsy. The 5 most recent patients had subtotal curettage at the time of biopsy; 4 of 5 received simultaneous intralesional corticosteroid injection. In all cases, systemic evaluation showed no other focus of LCH, reossification was timely, and no local recurrence or additional focus was noted in follow-up of 1 to 17 years., Conclusions: Transient immune dysfunction may provoke the cytokine-mediated proliferation of pathologic Langerhans cells within the hematopoietic marrow of the anterolateral frontal bone. These cells cause osteolysis through elaboration of interleukin-1 and prostaglandin E2. Corticosteroids can inhibit the mediators. We recommend incisional biopsy, frozen-section provisional diagnosis, subtotal curettage, intralesional corticosteroid instillation, postoperative systemic evaluation, and long-term surveillance.

Purpose: To describe the systemic treatment of recurrent eosinophilic granuloma., Methods: Case report and review of Langerhans cell histiocytosis., Results: A 9-year-old boy had painful, right upper eyelid swelling. A computed tomography scan showed a right superolateral orbital mass with evidence of bony erosion. Frozen section evaluation was consistent with eosinophilic granuloma. Biopsy and curettage were performed. Systemic work-up was negative for extraorbital disease. Recurrence of the orbital mass 6 weeks after surgery was confirmed by magnetic resonance imaging (MRI). Four weeks after treatment with prednisone and vinblastine, MRI showed a marked decrease in the size of the orbital lesion. Additional treatment with 6-mercaptopurine and methotrexate was initiated, and MRI 6 months later showed complete resolution of the lesion., Conclusions: The treatment of recurrent eosinophilic granuloma is challenging. The goal is to effectively cure the lesion with minimal short-term and long-term side effects. Systemic steroids, vinca alkaloids, antimetabolites, and antifols are preferred because they have less toxicity and mutagenicity. Combination chemotherapy used in conjunction with treatment by a pediatric hematologist/oncologist appears to be an effective method of treating recurrent disease.

Orbital involvement of eosinophilic granuloma accounts for less than 1% of all orbital tumors. The most common presenting sign of eosinophilic granuloma is bilateral or unilateral proptosis; rarely, neural parenchyma involvement is observed. This article features a case report of a 16-year-old male patient who presented to the authors after minor trauma that simulated a fracture near the orbital apex. The authors conclude that the diagnosis of eosinophilic granuloma should be considered if there is unusual location of apparent "fracture," a mass in the region of the fracture, lysis of bone, and recurrence of inflammation after the initial injury has subsided. Timely intervention with appropriate excision, histologic confirmation, and reconstruction with proper follow-up are the cornerstones of therapy for this rare disorder.

A rare tumour in a 5-year-old boy is presented and discussed. In time and location the story had two parts (Fig. 1): 1) a conjunctival granuloma at the nasal limbus of the right eye was surgically removed. 2) a few months later a huge lesion presented in the posterior segment of the same eye. Was it an ocular tumour with extension to the orbit or an orbital process with impression or invasion of the eye? Repeated surgical biopsies have indicated nodular scleritis of the posterior eye segment as the definitive diagnosis.

We present the cytologic findings of 18 fine-needle aspirates performed on orbital and periorbital masses. An accurate specific diagnosis was confirmed by histology in ten cases. In eight cases the diagnostic evaluation and/or therapeutic regimen was significantly altered by the results of the fine-needle biopsy. In three cases the cytologic diagnoses obviated the need for open biopsy. Two specimens were insufficient for diagnosis. No false positive diagnosis of malignancy occurred. Electron microscopy was useful in three cases to confirm the correct cytologic diagnosis. Immunocytochemistry helped to define a lymphoid proliferation as monoclonal. The cytologic findings from an unusual case of sebaceous carcinoma are presented.

Two children (aged 18 and 23 months at the initial examinations) were each ultimately found at surgery to have a solitary eosinophilic granuloma of the lateral orbital wall. Both patients had a symptomatic period of six weeks during which time other diagnoses were considered: bacterial preseptal cellulitis and mumps dacryoadenitis in the first case and traumatic recurrent orbital hematoma in the second. Diagnostic difficulties stemmed from confusing features in their histories, as well as the location of the lesion and the deceptively minimal swelling relative to the actual size of the lesion. Follow-up ten and 18 months after curettage of the two lesions showed no recurrence or evidence of systemic involvement. Although most reported cases describe the orbital frontal bone as the site of origin in the orbit, our cases demonstrated that unifocal eosinophilic granuloma may occur in the lateral wall of the orbit.

A 61-year-old Japanese man was treated for eosinophilic granuloma of the soft tissue in the orbit. The clinical characteristics were typical for eosinophilic granuloma of the soft tissue: 8% eosinophils, very soft tumor masses in both orbits, multifocal lesions including the salivary glands, good response to steroid hormone, and hypergammaglobulinemia. The ocular signs were exophthalmos and lid swelling. Light microscopy showed many lymphoid follicles with many eosinophils, also in other parts of the body. The previously reported cases of orbital lesion are reviewed. Patients with orbital eosinophilic granuloma of the soft tissue are apt to be 30-50 years of age, older than patients without ocular lesions.