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Long-term Nasal Plaque With Nasal Obstruction—Diagnosis

Long-term Nasal Plaque With Nasal Obstruction—Diagnosis Diagnosis: Granuloma faciale (GF) associated with eosinophilic angiocentric fibrosis (EAF). Diagnosis: Granuloma faciale (GF) associated with eosinophilic angiocentric fibrosis (EAF). Microscopic and laboratory findings and clinical course Histopathologic analysis of the mucosal biopsy specimen revealed whorled collagenous tissue associated with a background inflammatory infiltrate consisting of lymphocytes, plasma cells, and eosinophils. The fibrosis displayed an onionskin pattern (Figure 2). View LargeDownload Figure 2. Microscopic and laboratory findings and clinical course The skin biopsy specimen revealed a dense inflammatory infiltrate in the dermis, consisting of numerous eosinophils, neutrophils, lymphocytes, and histiocytes, extending into the subcutaneous fat but separated from the epidermis by a conspicuous Grenz zone. Focal vascular changes compatible with vasculitis with fibrinoid necrosis and karyorrhexis were found. We observed perivascular dermal fibrosis with a storiform pattern (Figure 3). In this case, different patterns were found in the mucosa (onionskin pattern) and in the skin (storiform pattern). View LargeDownload Figure 3. Microscopic and laboratory findings and clinical course All laboratory test findings were negative or within normal limits. A chest radiograph was unremarkable. A computed tomographic scan demonstrated a marked loss of the nasal airway secondary to diffuse thickening of the nasal mucosa associated with cartilage and bone loss. Bone gammography showed no pathologic catchment area. A bone-marrow biopsy finding was normal. Microscopic and laboratory findings and clinical course Treatment with intralesional injections of betamethasone was started. After 3 sessions at 1-month intervals, the patient's response was remarkable, and the skin lesion was completely flattened (Figure 4). View LargeDownload Figure 4. Diagnosis: Granuloma faciale (GF) associated with eosinophilic angiocentric fibrosis (EAF). Discussion Granuloma faciale is an uncommon benign cutaneous disorder that is considered to be a chronic form of leukocytoclastic vasculitis of unknown origin and poorly under-stood pathogenesis.1 Granuloma faciale lesions are particularly frequent on the nose, preauricular area, cheeks, and forehead and tend to be chronic. Histopathologically, the condition is characterized by a dense polymorphic cellular infiltrate in the upper dermis, which is separated from the epidermis and its appendages by a clear Grenz zone. The infiltrate is much more cellular early in its evolution rather than later, when lesions are characterized by fibrosis and are often arranged concentrically around vessels.2 Discussion Eosinophilic angiocentric fibrosis is a rare idiopathic condition that affects the upper respiratory mucosa. It is classified as a chronic fibrosing vasculitis.3 Allergy, atopy, and trauma have been suggested as predisposing factors.4 The disease progresses slowly. The diagnosis of EAF is based mainly on histologic findings. The early features include vasculitis without fibrinoid necrosis with a predominance of eosinophils. The lesion that develops later involves perivascular fibrosis that displays collagen fiber whorling in a characteristic onionskin pattern. Discussion Granuloma faciale and EAF resemble each other histologically. Six cases of concurrent presentation have been documented in the literature.1-9 It remains controversial whether GF and EAF represent 2 separate conditions or a cutaneous and/or mucosal variant of the same process. Discussion The treatment of both conditions is reported to be notoriously difficult. Medical approaches for GF have included treatment with colchicine, psoralen plus UV-A treatment, topical and intralesional corticosteroids, and antimalarial and antilepromatous agents, with variable responses. Physical therapies such as excision, cryotherapy, radiotherapy, electrodessication, and laser have also met with variable long-term success.2 Discussion In our patient, the clinical association of GF and EGF and the similar histopathologic findings suggest that the 2 are variants of the same process. We would like to emphasize the excellent response to treatment with intralesional corticosteroids. Discussion Return to Quiz Case. References 1. Chinelli PA, Kawashita MY, Sotto MN, Nico M. Granuloma faciale associated with sinonasal tract eosinophilic angiocentric fibrosis. Acta Derm Venereol. 2004;84(6):486-48715844648PubMedGoogle Scholar 2. Holme SA, Laidler P, Holt PJ. Concurrent granuloma faciale and eosinophilic angiocentric fibrosis. Br J Dermatol. 2005;153(4):851-85316181479PubMedGoogle ScholarCrossref 3. 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(11):1217-12254085985PubMedGoogle ScholarCrossref 4. Tabaee A, Zadeh MH, Proytcheva M, LaBruna A. Eosinophilic angiocentric fibrosis. J Laryngol Otol. 2003;117(5):410-41312803796PubMedGoogle ScholarCrossref 5. Nigar E, Dhillon R, Carr E, Matin RN. Eosinophilic angiocentric fibrosis and extrafacial granuloma faciale. Histopathology. 2007;51(5):729-73117927606PubMedGoogle ScholarCrossref 6. Yung A, Wachsmuth R, Ramnath R, Merchant W, Myatt AE, Sheehan-Dare R. Eosinophilic angiocentric fibrosis--a rare mucosal variant of granuloma faciale which may present to the dermatologist. Br J Dermatol. 2005;152(3):574-57615787836PubMedGoogle ScholarCrossref 7. Narayan J, Douglas-Jones AG. Eosinophilic angiocentric fibrosis and granuloma faciale: analysis of cellular infiltrate and review of literature. Ann Otol Rhinol Laryngol. 2005;114(1, pt 1):35-4215697160PubMedGoogle Scholar 8. Burns BV, Roberts PF, De 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(3):223-22611244533PubMedGoogle ScholarCrossref 9. Holmes DK, Panje WR. Intranasal granuloma faciale. Am J Otolaryngol. 1983;4(3):184-1866881462PubMedGoogle ScholarCrossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Long-term Nasal Plaque With Nasal Obstruction—Diagnosis

Archives of Dermatology , Volume 148 (6) – Jun 1, 2012

Long-term Nasal Plaque With Nasal Obstruction—Diagnosis

Abstract

Diagnosis: Granuloma faciale (GF) associated with eosinophilic angiocentric fibrosis (EAF). Diagnosis: Granuloma faciale (GF) associated with eosinophilic angiocentric fibrosis (EAF). Microscopic and laboratory findings and clinical course Histopathologic analysis of the mucosal biopsy specimen revealed whorled collagenous tissue associated with a background inflammatory infiltrate consisting of lymphocytes, plasma cells, and eosinophils. The fibrosis displayed an onionskin pattern (Figure...
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Publisher
American Medical Association
Copyright
Copyright © 2012 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.148.6.756-b
Publisher site
See Article on Publisher Site

Abstract

Diagnosis: Granuloma faciale (GF) associated with eosinophilic angiocentric fibrosis (EAF). Diagnosis: Granuloma faciale (GF) associated with eosinophilic angiocentric fibrosis (EAF). Microscopic and laboratory findings and clinical course Histopathologic analysis of the mucosal biopsy specimen revealed whorled collagenous tissue associated with a background inflammatory infiltrate consisting of lymphocytes, plasma cells, and eosinophils. The fibrosis displayed an onionskin pattern (Figure 2). View LargeDownload Figure 2. Microscopic and laboratory findings and clinical course The skin biopsy specimen revealed a dense inflammatory infiltrate in the dermis, consisting of numerous eosinophils, neutrophils, lymphocytes, and histiocytes, extending into the subcutaneous fat but separated from the epidermis by a conspicuous Grenz zone. Focal vascular changes compatible with vasculitis with fibrinoid necrosis and karyorrhexis were found. We observed perivascular dermal fibrosis with a storiform pattern (Figure 3). In this case, different patterns were found in the mucosa (onionskin pattern) and in the skin (storiform pattern). View LargeDownload Figure 3. Microscopic and laboratory findings and clinical course All laboratory test findings were negative or within normal limits. A chest radiograph was unremarkable. A computed tomographic scan demonstrated a marked loss of the nasal airway secondary to diffuse thickening of the nasal mucosa associated with cartilage and bone loss. Bone gammography showed no pathologic catchment area. A bone-marrow biopsy finding was normal. Microscopic and laboratory findings and clinical course Treatment with intralesional injections of betamethasone was started. After 3 sessions at 1-month intervals, the patient's response was remarkable, and the skin lesion was completely flattened (Figure 4). View LargeDownload Figure 4. Diagnosis: Granuloma faciale (GF) associated with eosinophilic angiocentric fibrosis (EAF). Discussion Granuloma faciale is an uncommon benign cutaneous disorder that is considered to be a chronic form of leukocytoclastic vasculitis of unknown origin and poorly under-stood pathogenesis.1 Granuloma faciale lesions are particularly frequent on the nose, preauricular area, cheeks, and forehead and tend to be chronic. Histopathologically, the condition is characterized by a dense polymorphic cellular infiltrate in the upper dermis, which is separated from the epidermis and its appendages by a clear Grenz zone. The infiltrate is much more cellular early in its evolution rather than later, when lesions are characterized by fibrosis and are often arranged concentrically around vessels.2 Discussion Eosinophilic angiocentric fibrosis is a rare idiopathic condition that affects the upper respiratory mucosa. It is classified as a chronic fibrosing vasculitis.3 Allergy, atopy, and trauma have been suggested as predisposing factors.4 The disease progresses slowly. The diagnosis of EAF is based mainly on histologic findings. The early features include vasculitis without fibrinoid necrosis with a predominance of eosinophils. The lesion that develops later involves perivascular fibrosis that displays collagen fiber whorling in a characteristic onionskin pattern. Discussion Granuloma faciale and EAF resemble each other histologically. Six cases of concurrent presentation have been documented in the literature.1-9 It remains controversial whether GF and EAF represent 2 separate conditions or a cutaneous and/or mucosal variant of the same process. Discussion The treatment of both conditions is reported to be notoriously difficult. Medical approaches for GF have included treatment with colchicine, psoralen plus UV-A treatment, topical and intralesional corticosteroids, and antimalarial and antilepromatous agents, with variable responses. Physical therapies such as excision, cryotherapy, radiotherapy, electrodessication, and laser have also met with variable long-term success.2 Discussion In our patient, the clinical association of GF and EGF and the similar histopathologic findings suggest that the 2 are variants of the same process. We would like to emphasize the excellent response to treatment with intralesional corticosteroids. Discussion Return to Quiz Case. References 1. Chinelli PA, Kawashita MY, Sotto MN, Nico M. Granuloma faciale associated with sinonasal tract eosinophilic angiocentric fibrosis. Acta Derm Venereol. 2004;84(6):486-48715844648PubMedGoogle Scholar 2. Holme SA, Laidler P, Holt PJ. Concurrent granuloma faciale and eosinophilic angiocentric fibrosis. Br J Dermatol. 2005;153(4):851-85316181479PubMedGoogle ScholarCrossref 3. 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(11):1217-12254085985PubMedGoogle ScholarCrossref 4. Tabaee A, Zadeh MH, Proytcheva M, LaBruna A. Eosinophilic angiocentric fibrosis. J Laryngol Otol. 2003;117(5):410-41312803796PubMedGoogle ScholarCrossref 5. Nigar E, Dhillon R, Carr E, Matin RN. Eosinophilic angiocentric fibrosis and extrafacial granuloma faciale. Histopathology. 2007;51(5):729-73117927606PubMedGoogle ScholarCrossref 6. Yung A, Wachsmuth R, Ramnath R, Merchant W, Myatt AE, Sheehan-Dare R. Eosinophilic angiocentric fibrosis--a rare mucosal variant of granuloma faciale which may present to the dermatologist. Br J Dermatol. 2005;152(3):574-57615787836PubMedGoogle ScholarCrossref 7. Narayan J, Douglas-Jones AG. Eosinophilic angiocentric fibrosis and granuloma faciale: analysis of cellular infiltrate and review of literature. Ann Otol Rhinol Laryngol. 2005;114(1, pt 1):35-4215697160PubMedGoogle Scholar 8. Burns BV, Roberts PF, De 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(3):223-22611244533PubMedGoogle ScholarCrossref 9. Holmes DK, Panje WR. Intranasal granuloma faciale. Am J Otolaryngol. 1983;4(3):184-1866881462PubMedGoogle ScholarCrossref

Journal

Archives of DermatologyAmerican Medical Association

Published: Jun 1, 2012

Keywords: fibrosis,nasal obstruction,nose,granuloma faciale

References