ABSTRACT
@#Introduction: Eosinophilic angiocentric fibrosis (EAF) is a rare lesion that predominantly affects the upper respiratory tract. Its etiology is unknown and it has been recently associated with the IgG4related disease (IgG4-RD) spectrum. To the author’s knowledge, this is the sixth case report of the relationship between EAF and IgG4-RD. Case Report: We report the case of a 37-year-old woman with nasal deformity and facial pain. The lesion was surgically excised. Histological examination revealed features of EAF with an IgG4/IgG plasma cell ratio >73% and 31 IgG4 stained cells per high power field. No clinical or radiological recurrence was detected during follow-up. Serum IgG4 quantification one year after surgery was within normal limits. Discussion: The relationship between both entities may have therapeutic impact because IgG4-RD of the head and neck has a high remission rate with corticosteroids and immunosuppressive therapy. Additional reports of this infrequent disease are necessary to elucidate appropriate treatment and prognosis.
ABSTRACT
Resumen La fibrosis angiocéntrica eosinofílica es una lesión tumoral infrecuente de la órbita ocular y del tracto respiratorio superior. Presentamos el caso de un hombre de 44 arios, con antecedentes de severa congestión y obstrucción nasal, y síntomas de sinusitis. Una TAC de senos paranasales mostró una masa focal de tejidos blandos de alta densidad que crecía desde la cara anterior del septum nasal. Se resecó parte del tabique. Los cortes histológicos mostraron una fibrosis concéntrica densa con patrón angiocéntrico y células inflamatorias mixtas. Se demuestra la presencia de fibrosis angiocéntrica eosinofílica. El dosaje sérico de IgG: 1.421 mg/dl (valor normal: 540-1.822) y el de IgG4: 168,70 mg/dl (valor normal: 6,1-121). Los preparados histológicos fueron inmunomarcados para IgG4 mostrando 50 plasmocitos IgG4(+)/HPF.
Abstract Eosinophilic angiocentric fibrosis is an uncommon tumefactive lesion of the ocular orbit and upper respiratory tract. We present a 44-year old man with a history of severe nasal congestion and nasal obstruction. The patient also reported symptoms of sinusitis. Submucous thickening tissue had been locally resected and the involved anterior nasal septum cartilage partially removed. Histopathological examination of the biopsy indicated eosinophilic angiocentric fibrosis. A CT scan of the sinuses showed a high-density focal mass of soft tissue arising from the anterior aspect of the nasal septum. Examination under low power view showed dense concentric fibrosis and mixed inflammatory cells. The fibrosis was in an angiocentric pattern, resembling an onionskin. High power view showed a mixture of lymphocytes, plasma cells, numerous eosinophils, and proliferating fibroblasts. A test for serum IgG performed ten years after the patient's initial presentation was 1421 mg/dl (normal range 540-1822 mg/dl), and the serum concentration of IgG4 was 168.70 mg/dl (normal range 6.1121 mg/dl). A review of the original haematoxylin and eosin-stained slides revealed that this case was stained immunohistochemically with an IgG4 stain showing 50 IgG4-positive plasma cells/HPF.
Subject(s)
Humans , Male , Adult , Fibrosis , Immunoglobulin G , Respiratory System , Wounds and Injuries , NeoplasmsABSTRACT
Eosinophilic Angiocentric Fibrosis (EAF) is an uncommon benign condition involving the sinonasal tract and rarely the larynx and orbit. Its etiology still remains unclear, although some studies have hypothesized it as a form of IgG4-related systemic disease. Histologically EAF is characterised by the presence of mixed inflammatory cells with eosinophil predominance in the early stage, followed by concentric layers of sclerosis around the small caliber vessels in the late stage. The rarity of this condition, tumor like presentation and oblivious etiology, makes it an interesting entity. We hereby report one such rare case of EAF, since the awareness of this entity is essential to make an accurate diagnosis.