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Pathology Quiz Case 1: Diagnosis

Pathology Quiz Case 1: Diagnosis Diagnosis: Granuloma faciale (GF) Granuloma faciale is a rare, benign, chronic dermatologic condition that is traditionally characterized by a single nodule or a few cutaneous nodules over the face.1It was first described in 1950 as a distinct entity because of its eosinophilic infiltrate.2It is most commonly found in whites, with a male predilection and a median age at diagnosis of 45 years. While the most common site of manifestation is the face, extrafacial GF has rarely been reported.3The pathogenesis is unknown, although it has been postulated that GF is a form of vasculitis that is mediated by a localized Arthus-like hypersensitivity reaction.4More recently, clonal T-cell production of interleukin 5 has been cited as an explanation for the eosinophilic inflammation seen in GF.5Growth of GF plaques is slow, insidious, and usually asymptomatic. Lesions occasionally cause mild pruritus or pain, while larger lesions may cause a localized mass effect. Grossly, the lesions appear as red to reddish brown, well-circumscribed, elevated plaques that vary from a few millimeters to several centimeters in diameter. Spontaneous involution of GF lesions is rare. Granuloma faciale does not have any malignant potential, although there is 1 reported case of coincident basal cell carcinoma and GF.6 The clinical differential diagnosis is extensive, encompassing autoimmune and inflammatory disorders such as discoid lupus erythematosus, sarcoidosis, cutaneous amyloidosis, and rosacea. Erythema elevatum diutinum is a type of chronic leukocytoclastic vasculitis that is characterized by red, brown, or yellow plaques or nodules and has a similar histologic appearance except that it has a higher proportion of neutrophils to eosinophils and a lack of a well-defined grenz zone.2Lesions are classically distributed over the dorsum of the joints of the hands and elbows. Although GF does not explicitly resemble either basal cell carcinoma or squamous cell carcinoma, GF lesions may be confused with a malignancy owing to an occasionally similar gross appearance. This underscores the necessity for a biopsy. Definitive diagnosis requires biopsy of the lesion. Microscopy demonstrates a normal-appearing epidermis and a grenz zone of uninvolved dermis overlying a mixed leukocytic infiltrate consisting of numerous eosinophils and neutrophils concentrated around dilated blood vessels. Lymphocytes, histiocytes, and, occasionally, mast cells may also be noted. Extravasated erythrocytes and hemosiderin deposits are often seen, which may contribute to the gross reddish brown appearance. Immunoflorescence reveals IgG deposits along the basement membrane of blood vessels.7Fibrosis may or may not be seen, depending on the duration of the lesion. The fibrosis generally develops in concentric circles around small vessels. Granuloma faciale is often resistant to medical therapy. A 585-nm pulsed-dye laser has been shown to produce resolution of the lesion without scarring.8Topical and intralesional corticosteroid therapies, dermabrasion, argon and carbon dioxide lasers, and cryotherapy have all been used with varying degrees of success. Dapsone has shown efficacy in treating GF, via its anti-inflammatory properties. Although the exact mechanism has yet to be fully elucidated, it is thought that dapsone interferes with the oxidative damage caused by an activated myeloperoxidase in neutrophils.4,9,10Unfortunately, many of these treatments result in significant scarring. Surgical excision may produce acceptable short-term results, but there is a substantial risk for recurrence depending on the size and location of the lesion.10Variables such as size and location of the lesion, as well as patient expectations regarding cosmesis and cure, should be considered before surgery is undertaken. After thorough explanation of procedural risks and benefits, our patient elected to undergo staged excision of the lesion under local anesthesia. Return to Quiz Case 2. References 1. Pedace FJPerry HO Granuloma faciale: a clinical and histopathologic review. Arch Dermatol 1966;94 (4) 387- 395PubMedGoogle ScholarCrossref 2. Lever WFLeeper RW Eosinophilic granuloma of the skin: report of cases representing the two different diseases described as eosinophilic granuloma. Arch Derm Syphilol 1950;62 (1) 85- 96PubMedGoogle ScholarCrossref 3. Sears JKGitter DGStone MS Extrafacial granuloma faciale. Arch Dermatol 1991;127 (5) 742- 743PubMedGoogle ScholarCrossref 4. Guill MAAton JK Facial granuloma responsive to dapsone therapy. Arch Dermatol 1982;118 (5) 332- 335PubMedGoogle ScholarCrossref 5. Gauger ARonet CSchnopp C et al. High local interleukin 5 production in granuloma faciale (eosinophilicum): role of clonally expanded skin-specific CD4+ cells. Br J Dermatol 2005;153 (2) 454- 457PubMedGoogle ScholarCrossref 6. Kamalpour LGammon BLarsen FCashman RCockerell C A finding of granuloma faciale associated with basal cell carcinoma. J Cutan Pathol 2009;36 (10) 1103- 1104PubMedGoogle ScholarCrossref 7. Nieboer CKalsbeek GL Immunofluorescence studies in granuloma eosinophilicum faciale. J Cutan Pathol 1978;5 (2) 68- 75PubMedGoogle ScholarCrossref 8. Sewell LElston D Extrafacial granuloma faciale successfully treated with 595-nm pulse dye laser. J Am Acad Dermatol 2008;58 (2) AB141Google ScholarCrossref 9. Zacarian SA Cryosurgery effective for granuloma faciale. J Dermatol Surg Oncol 1985;11 (1) 11- 13PubMedGoogle ScholarCrossref 10. Maillard HGrognard CToledano CJan VMachet LVaillant L Granuloma faciale: efficacy of cryosurgery in 2 cases. Ann Dermatol Venereol 2000;127 (1) 77- 79PubMedGoogle Scholar http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Otolaryngology - Head & Neck Surgery American Medical Association

Pathology Quiz Case 1: Diagnosis

Archives of Otolaryngology - Head & Neck Surgery , Volume 137 (4) – Apr 18, 2011

Pathology Quiz Case 1: Diagnosis

Abstract

Diagnosis: Granuloma faciale (GF) Granuloma faciale is a rare, benign, chronic dermatologic condition that is traditionally characterized by a single nodule or a few cutaneous nodules over the face.1It was first described in 1950 as a distinct entity because of its eosinophilic infiltrate.2It is most commonly found in whites, with a male predilection and a median age at diagnosis of 45 years. While the most common site of manifestation is the face, extrafacial GF has rarely been reported.3The...
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Publisher
American Medical Association
Copyright
Copyright © 2011 American Medical Association. All Rights Reserved.
ISSN
0886-4470
eISSN
1538-361X
DOI
10.1001/archoto.2011.48-b
Publisher site
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Abstract

Diagnosis: Granuloma faciale (GF) Granuloma faciale is a rare, benign, chronic dermatologic condition that is traditionally characterized by a single nodule or a few cutaneous nodules over the face.1It was first described in 1950 as a distinct entity because of its eosinophilic infiltrate.2It is most commonly found in whites, with a male predilection and a median age at diagnosis of 45 years. While the most common site of manifestation is the face, extrafacial GF has rarely been reported.3The pathogenesis is unknown, although it has been postulated that GF is a form of vasculitis that is mediated by a localized Arthus-like hypersensitivity reaction.4More recently, clonal T-cell production of interleukin 5 has been cited as an explanation for the eosinophilic inflammation seen in GF.5Growth of GF plaques is slow, insidious, and usually asymptomatic. Lesions occasionally cause mild pruritus or pain, while larger lesions may cause a localized mass effect. Grossly, the lesions appear as red to reddish brown, well-circumscribed, elevated plaques that vary from a few millimeters to several centimeters in diameter. Spontaneous involution of GF lesions is rare. Granuloma faciale does not have any malignant potential, although there is 1 reported case of coincident basal cell carcinoma and GF.6 The clinical differential diagnosis is extensive, encompassing autoimmune and inflammatory disorders such as discoid lupus erythematosus, sarcoidosis, cutaneous amyloidosis, and rosacea. Erythema elevatum diutinum is a type of chronic leukocytoclastic vasculitis that is characterized by red, brown, or yellow plaques or nodules and has a similar histologic appearance except that it has a higher proportion of neutrophils to eosinophils and a lack of a well-defined grenz zone.2Lesions are classically distributed over the dorsum of the joints of the hands and elbows. Although GF does not explicitly resemble either basal cell carcinoma or squamous cell carcinoma, GF lesions may be confused with a malignancy owing to an occasionally similar gross appearance. This underscores the necessity for a biopsy. Definitive diagnosis requires biopsy of the lesion. Microscopy demonstrates a normal-appearing epidermis and a grenz zone of uninvolved dermis overlying a mixed leukocytic infiltrate consisting of numerous eosinophils and neutrophils concentrated around dilated blood vessels. Lymphocytes, histiocytes, and, occasionally, mast cells may also be noted. Extravasated erythrocytes and hemosiderin deposits are often seen, which may contribute to the gross reddish brown appearance. Immunoflorescence reveals IgG deposits along the basement membrane of blood vessels.7Fibrosis may or may not be seen, depending on the duration of the lesion. The fibrosis generally develops in concentric circles around small vessels. Granuloma faciale is often resistant to medical therapy. A 585-nm pulsed-dye laser has been shown to produce resolution of the lesion without scarring.8Topical and intralesional corticosteroid therapies, dermabrasion, argon and carbon dioxide lasers, and cryotherapy have all been used with varying degrees of success. Dapsone has shown efficacy in treating GF, via its anti-inflammatory properties. Although the exact mechanism has yet to be fully elucidated, it is thought that dapsone interferes with the oxidative damage caused by an activated myeloperoxidase in neutrophils.4,9,10Unfortunately, many of these treatments result in significant scarring. Surgical excision may produce acceptable short-term results, but there is a substantial risk for recurrence depending on the size and location of the lesion.10Variables such as size and location of the lesion, as well as patient expectations regarding cosmesis and cure, should be considered before surgery is undertaken. After thorough explanation of procedural risks and benefits, our patient elected to undergo staged excision of the lesion under local anesthesia. Return to Quiz Case 2. References 1. Pedace FJPerry HO Granuloma faciale: a clinical and histopathologic review. Arch Dermatol 1966;94 (4) 387- 395PubMedGoogle ScholarCrossref 2. Lever WFLeeper RW Eosinophilic granuloma of the skin: report of cases representing the two different diseases described as eosinophilic granuloma. Arch Derm Syphilol 1950;62 (1) 85- 96PubMedGoogle ScholarCrossref 3. Sears JKGitter DGStone MS Extrafacial granuloma faciale. Arch Dermatol 1991;127 (5) 742- 743PubMedGoogle ScholarCrossref 4. Guill MAAton JK Facial granuloma responsive to dapsone therapy. Arch Dermatol 1982;118 (5) 332- 335PubMedGoogle ScholarCrossref 5. Gauger ARonet CSchnopp C et al. High local interleukin 5 production in granuloma faciale (eosinophilicum): role of clonally expanded skin-specific CD4+ cells. Br J Dermatol 2005;153 (2) 454- 457PubMedGoogle ScholarCrossref 6. Kamalpour LGammon BLarsen FCashman RCockerell C A finding of granuloma faciale associated with basal cell carcinoma. J Cutan Pathol 2009;36 (10) 1103- 1104PubMedGoogle ScholarCrossref 7. Nieboer CKalsbeek GL Immunofluorescence studies in granuloma eosinophilicum faciale. J Cutan Pathol 1978;5 (2) 68- 75PubMedGoogle ScholarCrossref 8. Sewell LElston D Extrafacial granuloma faciale successfully treated with 595-nm pulse dye laser. J Am Acad Dermatol 2008;58 (2) AB141Google ScholarCrossref 9. Zacarian SA Cryosurgery effective for granuloma faciale. J Dermatol Surg Oncol 1985;11 (1) 11- 13PubMedGoogle ScholarCrossref 10. Maillard HGrognard CToledano CJan VMachet LVaillant L Granuloma faciale: efficacy of cryosurgery in 2 cases. Ann Dermatol Venereol 2000;127 (1) 77- 79PubMedGoogle Scholar

Journal

Archives of Otolaryngology - Head & Neck SurgeryAmerican Medical Association

Published: Apr 18, 2011

Keywords: granuloma faciale,neutrophils

References

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