417
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S-747￭
Rapid progression of systemic sclerosis precipitated by a pheochromocytoma
순천향대학교서울병원 내과
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이종택, 정지수, 김현숙
Systemic sclerosis (SSc), also called scleroderma, is an immune-mediated rheumatic disease that is characterised by fibrosis of the skin and internal or- gans and vasculopathy. SScis known to be related with an increased risk of cancer. Pheochromocytomas are rare tumors derived from the chromaffin cells of the embryonic neural crest. A 57-year-old man had raynaud's phenomenon, sclerodactyly fingers and symptoms rapidly progressed to neck and both upper arms for 3 months. The anti - Scl 70 antibody positive. The initial modified Rodnan Skin Score (mRSS) was 15 points and the nailfold capil- lary test showed moderate avascularity and dilated capillaries. There was no abnormal finding in the blood test except for a slight increase in ESR. A 5.6 cm sized a left suprarenal mass in the HRCT of the patient was used to perform abdominal pelvic CT in the next step. Adrenal hamangioma, adreno- cortical carcinoma and pheochromocytma were suspected. Positron emission tomography-computed tomography showed a 5.4 cm sized hypometabolic mass in the left adrenal gland. The patient underwent a left hand-assisted laparoscopicadrenalectomy and the results of immunohistochemical stains consistent with pheochromocytoma rather than malignant tumor. In our case, the pateints syptom of SSc progressed rapidly and pheochromocytoma has been found incidentially. In such patients, raynaud’s phenomenon may be prolonged and can result in ischemic digital ulceration or infarction.
Significant negative correlations between plasma catecholamine concentration and dermal blood flow have been demonstrated in patients with pheochromocytoma. But the very high levels of catecholamine production may explain the persistent arterial vasospasm and critical peripheral ischemia. In this case, we report a patient of showing rapid progression of known SSc when he was concomitantly diagnosed pheochromocytoma which was incidentally found simultaneously may be related to the progression of disease.
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S-748￭
Rapid change of the capillary morphology in dermatomyositis with treatment response
순천향대학교서울병원 내과학교실
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박성조, 김윤석, 김현숙
Dermatomyositis (DM) is an idiopathic inflammatory myopathy with characteristic cutaneous findings that frequently affects the skin and proximal muscle weakness. A muscle biopsy is the gold standard for diagnosis. But there is no accurate method for estimate the treatment response. DM shows nailfold capillaroscopy (NFC) changes similar to scleroderma like pattern, but unlikely with scleroderma, capillary morphology has tendency for im- provement and deterioration with disease activity. The following case will shows this. A 75-year-old male was referred with proximal arm swelling and facial rash. The patient had been treated for hypertension, diabetes mellitus, gout, and had a low anterior resection due to rectal cancer 12 years before.
A magnetic resonance imaging revealed signal change in both proximal arm, and it was compatible with inflammatory myositis. NFC was also per- formed and it showed avascularity, moderate dilated capillaries, and decreased blood flow of left 3rd finger. Biopsies of skin and triceps brachii muscle were performed. Findings of muscle biopsy were perivascular and interfascicular inflammatory CD4+ lymphocyte infiltrates with adjoining groups of muscle fiber regeneration. These were consistent with histologic findings of DM. After diagnosis of DM, treatment commenced with high-dose of the methylprednisolone (500 mg/day) infusion for 3 days; and the dose of methylprednisolone was tapered gradually. After 3 months, facial rash and both upper extremities edema were improved. We performed NFC repeatedly, and it showed compatible of improvement mild avascularity, mild dilated ca- pillaries, and no tortuous capillaries. The relationship between NFC change and disease activity is still unclear. However, people with less disease prog- ress have been shown to have fine NFC results. As observed in this case, considering the characteristic NFC change observed in DM patients, NFC im- provement can be expected in DM improvement. Until now, there has not been enough tests to monitor the improvement of disease activity during treat- ment of DM. In this case, NFC was also rapidly changed. Therefore, NFC could be an alternative method to monitor the clinical outcome or suspect treatment response.
