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Research Letters |

Chronic Localized Fibrosing Vasculitis of the Eyelid FREE

Sheeja S. John, FRCS; Swetha S. Philip, MS; Anne J. Premkumar, MD; Sarada David, MS
Arch Ophthalmol. 2009;127(10):1396-1397. doi:10.1001/archophthalmol.2009.245.
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Chronic localized fibrosing vasculitis (CLFV) is a rare entity of unknown etiology. To our knowledge, it has never been reported to occur in the eyelid.

A 42-year-old man had gradually progressive, painless swelling in the left lower eyelid for 2 years. There was no history of systemic illness, drug intake, or insect bite. On examination, there was a nontender, firm, nodular mass in the left lower eyelid (Figure 1). The palpebral conjunctiva appeared normal. There were no features of orbital involvement. Ocular examination did not reveal any abnormalities in either eye, and best-corrected visual acuities were 20/20 OU. Systemic examination results were normal.

Place holder to copy figure label and caption
Figure 1.

Painless, slowly growing mass in the lower eyelid of a 42-year-old, otherwise healthy man. The differential diagnoses considered were lymphoma, pseudotumor, tuberculosis, sarcoidosis, and Wegener granulomatosis.

Graphic Jump Location

Computed tomographic scan of the orbit showed a homogeneous, minimally enhancing, relatively well-defined soft-tissue lesion in the eyelid with no evidence of orbital extension. Paranasal sinuses were normal. Results of a detailed systemic workup performed to rule out autoimmune or infectious systemic disease were within normal limits.

Incisional biopsy of the lesion was performed. Histopathological examination showed features of chronic leukocytoclastic vasculitis with a mild to moderate, diffuse infiltrate consisting predominantly of lymphocytes and fewer, scattered neutrophils, eosinophils, and plasma cells (Figure 2A). The inflamed stroma contained granulation tissue, marked proliferation of small vessels, and an extensive onion-skin pattern of fibrosis of blood vessels (Figure 2B). This picture was punctuated by foci of active leukocytoclastic vasculitis, characterized by fibrinoid necrosis of vessel walls in which eosinophils, neutrophils, and nuclear debris were present (Figure 2C). No lymphoid hyperplasia or granulomas were identified. These histological features were consistent with CLFV. The patient was started on topical tacrolimus ointment and is currently being followed up.

Place holder to copy figure label and caption
Figure 2.

Histopathological analysis of the eyelid lesion (hematoxylin-eosin). A, Diffuse infiltration of inflammatory cells with fibrosed blood vessels (original magnification ×50). B, Onion-skin pattern (arrows) of fibrosis of the vessels (original magnification ×200). C, Lymphocytes and nuclear debris (arrows) in the walls of blood vessels (original magnification ×200).

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Chronic localized fibrosing vasculitis, an entity that was first described by Carlson and LeBoit in 1997,1 is characterized by a nonspecific inflammatory reaction with vasculitis of small vessels with a distinctive concentric pattern of fibrosis.

Although CLFV may be considered a variant of inflammatory pseudotumor of the skin,1 there are distinct differences. The presence of vasculitis and leukocytoclasis (nuclear debris) and the absence of dense plasma cell infiltrate in our case clearly distinguished it from cutaneous inflammatory pseudotumor. The distinction between the 2 entities may also have prognostic implications as CLFV (classified as vasculopathic reaction pattern2) may be cured by local excision,1 whereas idiopathic pseudotumor of the skin (classified as tumorlike proliferations2) is now considered part of the spectrum of myofibroblastic proliferations3 with a potential for recurrence and localized persistent growth.4

Chronic localized fibrosing vasculitis has been considered a variant of granuloma faciale, which usually occurs as 1 or more plaques, papules, or nodules on the face.5 Histologically, granuloma faciale has dense inflammation with predominant eosinophils and plasma cells, has dilated blood vessels, and often has extravasated red blood cells and toxic hyaline resembling fibrinoid material in the vessel wall, unlike the picture in our case. Targetoid, angiocentric, concentric fibrosis is also generally not seen in granuloma faciale.

Other pathological differential diagnoses are erythema elevatum diutinum, eosinophilic angiocentric fibrosis,6 and angiolymphoid hyperplasia with eosinophilia.5 The clinicopathological criteria that we used to diagnose CLFV in our case were as follows: solitary lesion; negative results on systemic evaluation for vasculitis; chronic inflammatory infiltrate of predominant lymphocytes; foci of acute leukocytoclastic vasculitis with fibrinoid necrosis, neutrophils, eosinophils, and nuclear debris; proliferation of small blood vessels and granulation tissue; concentric fibrosis of blood vessels; and no lymphoid hyperplasia or granulomas.

The clinical differential diagnosis of a nodular lesion in the eyelid as in our case would be lymphoma, pseudotumor, and granulomatous lesions like tuberculosis, syphilis, sarcoidosis, and Wegener granulomatosis. Despite its rarity, CLFV should be considered in the differential diagnosis of an eyelid mass.

Correspondence: Dr John, Department of Ophthalmology, Christian Medical College, Vellore 632001, India (sheejajohn@rediffmail.com).

Financial Disclosure: None reported.

Carlson  JALeBoit  PE Localized chronic fibrosing vasculitis of the skin: an inflammatory reaction that occurs in settings other than erythema elevatum diutinum and granuloma faciale. Am J Surg Pathol 1997;21 (6) 698- 705
PubMed
Weedon  D Skin Pathology. 2nd ed. Edinburgh, Scotland Churchill Livingstone2002;
Mentzel  T Myofibroblastic sarcomas: a brief review of sarcomas showing a myofibroblastic line of differentiation and discussion of the differential diagnosis. Curr Diagn Pathol 2001;7 (1) 17- 2410.1054/cdip.2000.0057
Coffin  CMWatterson  JPriest  JRDehner  LP Extrapulmonary inflammatory myofibroblastic tumor (inflammatory pseudotumor): a clinicopathologic and immunohistochemical study of 84 cases. Am J Surg Pathol 1995;19 (8) 859- 872
PubMed
Ortonne  NWechsler  JBagot  MGrosshans  ECribier  B Granuloma faciale: a clinicopathologic study of 66 patients. J Am Acad Dermatol 2005;53 (6) 1002- 1009
PubMed
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- 1225
PubMed

Figures

Place holder to copy figure label and caption
Figure 1.

Painless, slowly growing mass in the lower eyelid of a 42-year-old, otherwise healthy man. The differential diagnoses considered were lymphoma, pseudotumor, tuberculosis, sarcoidosis, and Wegener granulomatosis.

Graphic Jump Location
Place holder to copy figure label and caption
Figure 2.

Histopathological analysis of the eyelid lesion (hematoxylin-eosin). A, Diffuse infiltration of inflammatory cells with fibrosed blood vessels (original magnification ×50). B, Onion-skin pattern (arrows) of fibrosis of the vessels (original magnification ×200). C, Lymphocytes and nuclear debris (arrows) in the walls of blood vessels (original magnification ×200).

Graphic Jump Location

Tables

References

Carlson  JALeBoit  PE Localized chronic fibrosing vasculitis of the skin: an inflammatory reaction that occurs in settings other than erythema elevatum diutinum and granuloma faciale. Am J Surg Pathol 1997;21 (6) 698- 705
PubMed
Weedon  D Skin Pathology. 2nd ed. Edinburgh, Scotland Churchill Livingstone2002;
Mentzel  T Myofibroblastic sarcomas: a brief review of sarcomas showing a myofibroblastic line of differentiation and discussion of the differential diagnosis. Curr Diagn Pathol 2001;7 (1) 17- 2410.1054/cdip.2000.0057
Coffin  CMWatterson  JPriest  JRDehner  LP Extrapulmonary inflammatory myofibroblastic tumor (inflammatory pseudotumor): a clinicopathologic and immunohistochemical study of 84 cases. Am J Surg Pathol 1995;19 (8) 859- 872
PubMed
Ortonne  NWechsler  JBagot  MGrosshans  ECribier  B Granuloma faciale: a clinicopathologic study of 66 patients. J Am Acad Dermatol 2005;53 (6) 1002- 1009
PubMed
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- 1225
PubMed

Correspondence

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