Clinical Features and Results of Steroid Therapy for Orbital Inflammatory Pseudotumor

PURPOSE: To evaluate the clinical features and treatment outcomes of steroid therapy for orbital inflammatory pseudotumor. METHODS: Sixty-four patients diagnosed with orbital inflammatory pseudotumor were reviewed retrospectively. Patients with a follow-up period of less than 6 months were excluded from the study. The pseudotumor was classified into myositic, lacrimal, anterior, diffuse, or apical type according to orbital computed tomography findings. All patients were initially treated with systemic corticosteroids and evaluated for response to the treatment. Treatment outcome was considered a "success" if the patient had complete relief of symptoms with no recurrence, and a "failure" if the patient had no or only partial relief of symptoms or showed relapse. Factors affecting the treatment outcome were analyzed. RESULTS: The most frequent lesion subtype was myositis. Periorbital edema was the most common symptom and was evident in 53.1% of the patients. Thirty-eight patients (59.4%) showed treatment success. Age, sex, bilaterality, and mean follow-up length did not correlate with the treatment outcome. A short interval from symptom onset to treatment time and apical subtype were significantly associated with good steroid response (p < 0.05). CONCLUSIONS: In orbital inflammatory pseudotumor, myositis was the most common subtype. A short interval from symptom onset to treatment time and apical subtype were associated with good steroid response.

Morbihan Disease: A Rare Variant of Rosacea / 대한피부과학회지

Morbihan disease, also known as 'rosacea lymphedema' or 'solid persistent facial edema' was first reported by Degos, in 1957, for describing persistent lymphedema on the upper half of the face in association with rosacea. Although the pathogenesis of Morbihan disease is not established well, two hypotheses were proposed; firstly, the hypothesis of lymphatic obstruction by destruction of vessel supporting connective tissue (e.g. elastin), and secondly, the hypothesis of mast cell-induced fibrosis. Herein, we report a 71-year-old man who had 9-months history of persistent bilateral edema of the eyelids. The histopathologic exam from edematous eyelid revealed marked dermal edema, dilated vascular structure with perivascular mixed cellular inflammatory infiltration. Moreover, focal granulomatous infiltration adjacent to a vascular structure and numerous mast cells were also identified. This case is the first reported case of Morbihan disease in Korean dermatological literature. Furthermore, the histopathologic findings of this case support both of the two hypotheses for development of Morbihan disease.

A Case of Systemic Lupus Erythromatosus Presenting with Periorbital Edema / 대한류마티스학회지

Periorbital edema is a rare manifestation of systemic lupus erythematosus (SLE). We describe here a patient with bilateral periorbital edema as a presenting symptom of SLE. A 23-year-old woman was admitted for a sudden onset of severe periorbital edema that developed 7 days previously. On the basis of the malar rash, arthritis of the right hand and left wrist, leukopenia, proteinuria and the positive anti-nuclear and anti-dsDNA antibodies, she was diagnosed with SLE. Kidney biopsy revealed lupus nephritis (WHO class IV). After treatment with high dose of steroids, intravenous cyclophosphamide and daily hydroxychloroquine, her symptoms resolved, including the periorbital edema. She was discharged with prescriptions for prednisolone and hydroxychloroquine and she remains well.

A Case of Drug Eruption with Periorbital Edema and Exfoliative Dermatitis Induced by Imatinib Mesylate (STI571, Gleevec(TM)) / 대한피부과학회지

STI571 (imatinib mesylate, Gleevec(TM)), a selective inhibitor of the bcr-abl, c-kit, platelet-derived growth factor receptor tyrosine kinases, is a new anticancer drug used for chronic myelogenous leukemia and gastrointestinal stromal tumors. Cutaneous adverse reactions of periorbital edema and exfoliative dermatitis related to STI571 are rare and there have been no previous reports in the Korean literature. We herein report a case of periorbital edema and exfoliative dermatitis due to STI571 and discuss the possible mechanism of periorbital edema related to STI571.