ABSTRACT
INTRODUCTION: Immunglobulin G4 (IgG4)-related disease was recently described and represents a systemic lymphoproliferative disorder. The orbital form of the disease manifests as chronic lid swelling and proptosis. Visual disturbances may occur due to apical orbital lesions. CASE REPORT: A 65-year old pacient presents with the impossibility of maintaining his right eye open due to a progressive swelling of the upper lid. The general clinical examination shows adenopathy at 3 lymph node stations of the head. On ophthalmologic examination, a large tumor of the upper lid is observed in the right eye and proptosis and central retinal vein occlusion are noted in the left eye. The serum levels of the IgG are very high. A lymph node biopsy was performed. DISCUSSION: The differential diagnosis between the IgG4-related orbital disease and non-Hodgkin lymphoma is discussed. CONCLUSION The diagnosis criteria for IgG4-related disease are both the high serum levels of IgG4 and the specific immunohistochemistry stains.
Subject(s)
Eyelid Neoplasms/diagnosis , Hypergammaglobulinemia/diagnosis , Immunoglobulin G/blood , Immunologic Factors/blood , Lymphoma, Non-Hodgkin/diagnosis , Orbital Neoplasms/diagnosis , Aged , Biomarkers/blood , Diagnosis, Differential , Exophthalmos/diagnosis , Eyelid Neoplasms/blood , Eyelid Neoplasms/immunology , Humans , Hypergammaglobulinemia/blood , Hypergammaglobulinemia/immunology , Lymphoma, Non-Hodgkin/blood , Lymphoma, Non-Hodgkin/immunology , Male , Orbital Neoplasms/blood , Orbital Neoplasms/immunology , Retinal Vein Occlusion/diagnosisABSTRACT
PURPOSE: To evaluate the effects of steroid injections for periocular capillary hemangioma on adrenal function and body composition. DESIGN: Noncomparative, interventional case series. PARTICIPANTS: Four patients with periocular hemangioma. METHODS: Four white female infants with sight-threatening periocular hemangiomata received a combination of steroid injections of triamcinolone and betamethasone. In the first 2 cases, injections were perilesional and in the other 2, intralesional. MAIN OUTCOME MEASURES: The infants were monitored by serial measurements of basal serum cortisol concentrations, responses to the Synacthen stimulation test, measurement of growth and of weight gain, and, in one case, more detailed anthropometric measures of body composition. RESULTS: Prolonged suppression of circulating serum cortisol concentrations and cortisol responses to the Synacthen stimulation test were noted in 3 cases, and marked failure to thrive was noted in all 4 cases. CONCLUSIONS: Adrenal suppression after steroid injection for periocular capillary hemangioma is a potentially life-threatening complication. Failure to thrive is also a frequent side effect of treatment. Ophthalmologists should undertake the above treatment in consultation with a pediatric endocrinologist.
Subject(s)
Adrenal Insufficiency/chemically induced , Eyelid Neoplasms/drug therapy , Failure to Thrive/chemically induced , Glucocorticoids/adverse effects , Hemangioma, Capillary/drug therapy , Adrenal Insufficiency/blood , Betamethasone/adverse effects , Betamethasone/therapeutic use , Eyelid Neoplasms/blood , Failure to Thrive/blood , Female , Glucocorticoids/therapeutic use , Hemangioma, Capillary/blood , Humans , Hydrocortisone/blood , Infant , Injections, Intralesional , Triamcinolone Acetonide/adverse effects , Triamcinolone Acetonide/therapeutic useSubject(s)
Carcinoma, Basal Cell/blood , Eyelid Neoplasms/blood , Adult , Aged , Aged, 80 and over , Biomarkers, Tumor/blood , Carcinoembryonic Antigen/blood , Ferritins/blood , Humans , Middle Aged , Pregnancy-Specific beta 1-Glycoproteins/analysis , Radioimmunoassay , alpha-Fetoproteins/biosynthesisABSTRACT
Two infants with symptomatic periocular hemangiomas received 1-ml intralesional injections of a 50:50 mixture of triamcinolone acetonide (40 mg/ml) and betamethasone (8 mg/ml). Both developed adrenal suppression as evidenced by the immediate depression of their serum cortisol and adrenocorticotrophic hormone levels. One patient remained adrenal suppressed for five months and, concurrently, her growth rate and weight dropped from normal to below the fifth percentile. Adrenal suppression should be added to the growing list of complications caused by the corticosteroid injection of periocular hemangiomas.
