Dataset Information

An unusual orbital tumor in an adult: Granuloma annulare.

ABSTRACT:

Purpose

Granuloma annulare (GA) is a rare clinical entity that does not classically arise from the peri-orbital area in adults. The purpose of this case report is to present a 69-year-old female with GA of the orbit. As well, the pathological and immunohistochemical features of these tumors will be discussed.

Observations

One case of GA of the orbit was identified from a tertiary ophthalmology referral centre. Clinical and histopathological features of the case were reviewed. Other cases of GA were also retrieved from the literature and addressed in this report.

Conclusion and importance

Granuloma annulare is a rare orbital lesion in adults. It is known to typically arise on the hands and feet of children. This lesion must be distinguished from necrobiotic xanthogranuloma (NXG), which is a progressive peri-orbital dermatosis seen in middle age men and women. GA is thought to be a benign, often self-resolving condition, whereas NXG tends to be linked to other systemic conditions and may have a poorer prognosis.Differentiating this rare orbital tumor from necrobitotic xanthogranuloma (NXG) is essential, as both a systemic work-up and follow-up must be appropriately arranged. A comprehensive description of pathognomonic microscopic features of GA and NXG is reviewed to achieve the correct diagnosis.

SUBMITTER: Bergeron S

PROVIDER: S-EPMC6247445 | biostudies-literature | 2019 Mar

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

An unusual orbital tumor in an adult: Granuloma annulare.

Bergeron Sabrina S Sanft Debra-Meghan DM Zoroquiain Pablo P Esposito Evangelina E Arthurs Bryan B Burnier Miguel N MN

American journal of ophthalmology case reports 20181114

<h4>Purpose</h4>Granuloma annulare (GA) is a rare clinical entity that does not classically arise from the peri-orbital area in adults. The purpose of this case report is to present a 69-year-old female with GA of the orbit. As well, the pathological and immunohistochemical features of these tumors will be discussed.<h4>Observations</h4>One case of GA of the orbit was identified from a tertiary ophthalmology referral centre. Clinical and histopathological features of the case were reviewed. Othe ...[more]

PMID: 30505978

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Project description:BACKGROUND:Granuloma annulare is a chronic noninfectious granulomatous skin condition with variable clinical presentations. Generalized granuloma annulare, defined as widespread disease with >10 skin lesions, accounts for 15% of all cases. Numerous associated diseases have been controversially discussed, most importantly diabetes mellitus, dyslipidemia, thyroid disease, malignancy and systemic infections. OBJECTIVES:The objective of our study is to describe disease characteristics, treatment outcome and associated diseases in patients treated at the Department of Dermatology of the University Hospital Zurich during the last 20 years. METHODS:The hospital database was searched for patients with generalized granuloma annulare in the last 20 years (January 1, 1998, to December 31, 2017). Overall, 61 patients, 14 males and 47 females, were included in our study. The mean age was 58 years at first consultation. The diagnosis was verified clinically and histologically. RESULTS:Generalized granuloma annulare occurred at a mean age of 55 years, more commonly in females. Pruritus was absent in 51% of all patients. Metabolic diseases including diabetes mellitus, hypercholesterinemia and hypertriglyceridemia were present in 10.5, 8.2 and 4.9%, respectively. Thyroid disease was present in 9.8% and malignant disease in 23%, including colorectal cancer, lymphoproliferative disease, squamous cell carcinoma of the esophagus, basal cell carcinoma and gynecological malignancy. Therapy was initiated in 92%, while second- and third-line therapy was performed in 70 and 39%, respectively. Benefit during therapy (e.g., full and partial remission) was achieved in 39.3% during first-line, in 39.4% during second-line and in 33.8% during third-line treatment. Topical corticosteroids were the most commonly prescribed treatment, mostly leading to stable disease (46.6%). Combined full and partial remission occurred in a large proportion of patients receiving UVA1 (45%), PUVA (63.6%) and intralesional triamcinolone acetonide (100%). CONCLUSIONS:Generalized granuloma annulare is a mostly asymptomatic and benign disease with a strong tendency for treatment resistance. We suggest to screen all patients for dyslipidemia, thyroid disease and malignant disease. While randomized trials are needed, we suggest topical corticosteroids as the first-line treatment, intralesional triamcinolone acetonide for persistent solitary lesions and, if further treatment is needed, UVA1 or PUVA.

Project description:ImportanceAlthough granuloma annulare (GA) has been associated with several other conditions, these studies have been limited by single-center designs and small sample sizes.ObjectiveTo evaluate whether there is an association between GA and type 2 diabetes, hyperlipidemia, autoimmune conditions, and hematologic malignant neoplasms, using a large population-based cohort study.Design, setting, and participantsThis retrospective cohort study conducted between January 1, 2016, and June 30, 2019, used deidentified data from the US Optum Clinformatics Data Mart Database. A total of 5137 patients with GA were matched by age and sex with up to 10 randomly selected controls (n = 51 169) with a diagnosis of a nevus or seborrheic keratosis.Main outcomes and measuresLogistic regression was used to evaluate for potential associations between GA and diabetes, hyperlipidemia, autoimmune conditions, and hematologic malignant neoplasms. All analyses were adjusted for race/ethnicity, income, and educational level.ResultsThis study included 5137 individuals with GA (3760 women [73.2%]; mean [SD] age, 57.7 [19.0] years) and 51 169 controls (37 456 women [73.2%]; mean [SD] age, 57.7 [19.0] years). Those with GA were more likely than controls to have baseline diabetes (1086 [21.1%] vs 6780 [13.3%]; adjusted odds ratio [aOR], 1.67; 95% CI, 1.55-1.80), hyperlipidemia (1669 [32.5%] vs 14 553 [28.4%]; aOR, 1.15; 95% CI, 1.08-1.23), hypothyroidism (727 [14.2%] vs 5780 [11.3%]; aOR, 1.24; 95% CI, 1.15-1.36), and rheumatoid arthritis (62 [1.2%] vs 441 [0.9%]; aOR, 1.34; 95% CI, 1.02-1.75). Those with GA were more likely to have incident diabetes (144 [2.8%] vs 1061 [2.1%]; aOR, 1.31; 95% CI, 1.10-1.57), hypothyroidism (41 [0.8%] vs 252 [0.5%]; aOR, 1.59; 95% CI, 1.14-2.22), systemic lupus erythematosus (21 [0.4%] vs 65 [0.1%]; aOR, 3.06; 95% CI, 1.86-5.01), and rheumatoid arthritis (26 [0.5%] vs 122 [0.2%]; aOR, 2.05; 95% CI, 1.34-3.13). There was no association between GA and an increased risk of hematologic malignant neoplasms.Conclusions and relevanceThis population-based cohort study identified associations between GA and baseline diabetes and hyperlipidemia as well as between GA and both baseline and incident autoimmune conditions. These findings suggest that diabetes and hyperlipidemia may be risk factors for the development of GA and that autoimmunity may be an important factor in the pathogenesis of GA.

Dataset Information

An unusual orbital tumor in an adult: Granuloma annulare.

Purpose

Observations

Conclusion and importance

Publications

An unusual orbital tumor in an adult: Granuloma annulare.

Similar Datasets

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