Eosinophilic angiocentric fibrosis in a patient with nasal obstruction.

Abstract

Eosinophilic angiocentric fibrosis (EAF) is a rare clinicopathologic entity that usually affects the upper respiratory tract. We describe the case of a 45-year-old man with nasal obstruction caused by EAF. His lesion featured the distinct radiologic and histopathologic characteristics of EAF, which include an onionskin and whorled fibrosis that surrounds the blood vessels and an inflammatory infiltrate that is predominated by eosinophils. The etiology of EAF remains unknown. Given our increasing knowledge of this rare entity, reports of EAF may appear more frequently in the future.

Introduction

Eosinophilic angiocentric fibrosis (EAF) is a rare clinicopathologic entity. Only 13 cases have been previously reported in the literature, (1-8) the first three by Roberts and McCann in 1985. (1) This condition usually affects the upper respiratory tract. It is characterized by an early phase of eosinophilic vasculitis and a late phase of fibrosis with a whorled and onionskin appearance--all in a single biopsy specimen. In this article, we describe a new case of EAF.

Case report

A 45-year-old man, originally from India, came to our clinic in April 2002 for evaluation of nasal obstruction. Two years earlier, he had undergone surgical biopsy at another institution, and he was diagnosed with EAF. No definitive surgery was performed.

At our clinic, the patient reported progressive difficulty in breathing, and this had contributed to the onset of occasional panic attacks. He was also concerned about the external appearance of his nose, which had gradually increased in size and protuberance. The patient denied any other medical problems or any problem with allergies. He smoked a cigar twice a week and drank a beer every other day.

Physical examination revealed that a large bilateral nasal mass had involved the septum and soft tissues. The lower two-thirds of the nose was widened, and fibrosis was seen around the upper and lower lateral cartilages.

Computed tomography (CT) revealed the presence of a solid, soft-tissue mass in the right middle meatus (figure 1). The mass had eroded the bilateral nasal bones and the anterior aspect of the nasal septum. It extended posteriorly into the nares, but it did not involve the nasopharynx or sinuses.

[FIGURE 1 OMITTED]

The lesion was excised, and histopathology revealed that the specimen contained relatively homogenous, tan-gray, soft-tissue fragments. Microscopically, a whorled onionskin fibrosis surrounded the small blood vessels (figure 2). Specifically, a very hyalinized dense fibrous background contained relatively evenly spaced blood vessels, many of which were surrounded by a whorled matrix. The mixed inflammatory infiltrate (some lymphocytes, histiocytes, and plasma cells) in these areas was predominated by eosinophils. The fibrosis was extensive, and there was collagen deposition with bland spindle cells, which were likely fibroblasts. No granuloma or necrosis was present.

[FIGURE 2 OMITTED]

Discussion

Upon excision of the nasal soft tissue in this case, numerous sections exhibited similar histologic features. Variably cellular foci were primarily made up of eosinophils. Other cell types were also present, including lymphocytes and plasma cells. There was also extensive fibrosis in a whorled matrix surrounding the blood vessels. These findings are similar to those contained in other case reports that described evidence of an evolution in a single biopsy specimen; this evolution was manifested by the presence of early inflammation with lymphocytes, plasma cells, and eosinophils in some areas of the specimen and late lesions of dense fibrotic thickening in other areas. (1-8) In these reports, the fibrous tissue always appeared in a unique perivascular, onionskin, whorled pattern. Our specimen also demonstrated a pattern intermediate between the early and late stages of the evolution. According to some case reports, (3,5) an appearance resembling leukocytoclastic vasculitis can be seen, but this was not demonstrated in our case. Our specimen also did not contain any granulomas, intravascular thrombosis, necrotizing vasculitis, or lymphoid aggregates. (5) The lack of such findings ruled out Wegener's granulomatosis, Churg-Strauss syndrome, infective granulomatosis, sarcoidosis, Kimura's disease, and eosinophilic granuloma. In addition, granuloma faciale and inflammatory pseudotumor could be excluded from the differential diagnosis because these diseases are not characterized by intense fibrosis and onionskin angiocentric whorling.

The etiology of EAF remains unknown. Because of the predominance of eosinophils, some authors have proposed that it might be caused by an allergy. (1,2) Our patient did not have any history of allergy. EAF has been shown to be refractory to steroids, antibiotics, and other topical agents, another fact that would argue against the allergy hypothesis. Indeed, our patient had been treated with a trial of intranasal steroids at another institution, but he had experienced no relief. Fageeh et al reported that EAF appears to respond to surgical excision of the involved segment only. (2)

Nasal obstruction is a common symptom, and thickening of the fibrous mucosa of the lateral nasal wall and septum is a common finding. Our patient's nasal obstruction was significant, and it led to occasional panic attacks. During the time between the initial diagnosis at another institution and the surgical excision at our clinic, the mass had expanded to involve the septum and upper lateral cartilages. A large septal graft containing both bony and cartilaginous septum was required to reconstruct the defect. The remaining portions of the upper lateral cartilages were also resuspended to spreader grafts.

CT often demonstrates a solid soft-tissue mass, (3) as occurred in our case. The progressive inflammatory process can result in fibrosis and partial airway obstruction. Most reported cases of EAF have occurred in the nasal cavity; two others were reported in the subglottic area. (1,2) The age of our patient (45 yr) was consistent with the overall average age of onset of this disease (47 yr). (7) However, it should be noted that our patient was male, whereas 10 of the 13 previously reported cases occurred in women. (8) Some authors have reported that when EAF occurs in men, other entities such as granuloma faciale or Wegener's granulomatosis are more likely to coexist with the nasal lesion. (5,6) Our patient clearly did not demonstrate manifestations of any other disease process.

The unique characteristics of EAF have been rarely reported in the literature. Given our increasing knowledge of this entity, we may find that EAF will be reported more frequently in the future. Of the 13 lesions that were previously reported, 11 (85%) occurred in the nose and 2 occurred in the subglottic region. Given the fact that the initial symptom of EAF is airway obstruction, otolaryngologists should be familiar with this disease process.

References

(1.) Roberts PF, McCann BG. Eosinophilic angiocentric fibrosis of the upper respiratory tract: A mucosal variant of granuloma faciale? A report of three cases. Histopathology 1985;9:1217-25.

(2.) Fageeh NA, Mai KT, Odell PF. Eosinophilic angiocentric fibrosis of the subglottic region of the larynx and upper trachea. J Otolaryngol 1996;25:276-8.

(3.) Altemani AM, Pilch BZ, Sakano E, Altemani JM. Eosinopbilic angiocentric fibrosis of the nasal cavity. Mod Pathol 1997;10: 391-3.

(4.) Matai V, Baer S, Barnes S, Boxer M. Eosinophilic angiocentric fibrosis. J Laryngol Otol 2000;114:563-4.

(5.) Burns BV, Roberts PF, Dc Carpentier J, Zarod AP. Eosinophilic angiocentric fibrosis affecting the nasal cavity. A mucosal variant of the skin lesion granuloma faciale. J Laryngol Otol 2001;115: 223-6.

(6.) Loane J, Jaramillo M, Young HA, Kerr KM. Eosinophilic angiocentric fibrosis and Wegener's granulomatosis: A case report and literature review. J Clin Pathol 2001;54:640-1.

(7.) Thompson LD, Heffner DK. Sinonasal tract eosinophilic angiocentric fibrosis: A report of three cases. Am J Clin Pathol 2001; 115:243-8.

(8.) Pereira EM, Millas I, Reis-Filho JS, et al. Eosinophilic angiocentric fibrosis of the sinonasal tract: Report on the clinicopathologic features of a case and review of the literature. Head Neck 2002; 24:307-11.

Davis B. Nguyen, MD

James C. Alex, MD

Benjamin Calhoun, MD

>From the Division of Facial Plastic and Reconstructive Surgery, Section of Otolaryngology (Dr. Nguyen and Dr. Alex), and the Department of Pathology (Dr. Calhoun), Yale University School of Medicine, New Haven, Conn.

Reprint requests: Davis B. Nguyen, MD, Section of Otolaryngology, Yale University School of Medicine, 333 Cedar St., New Haven, CT 06520. Phone: [phone]; fax: [phone]; e-mail: [e-mail]