Abstract: We report a case of cutaneous angiosarcoma of the head and face with atypical manifestations. An 81-year-old male patient presented with progressive swelling of the unilateral eyelid for 8 months and infiltrative, painless erythema on the face for 6 months. He was diagnosed with “periodontal disease and lymphadenitis” at another hospital 3 months prior. After anti-inflammatory treatment and tooth extraction, the skin lesions showed no obvious improvement, and similar lesions appeared on the contralateral face. He was admitted to our hospital 6 months after onset. Cutaneous well-differentiated angiosarcoma was diagnosed by histopathology and immunohistochemistry. This case indicates that angiosarcoma of the head and face may present only with eyelid swelling and facial erythema, lacking typical purpuric lesions, and is easily misdiagnosed. For elderly patients with painless erythema and progressive soft tissue swelling of the head and face that is unresponsive to anti-inflammatory therapy, high suspicion and early pathological biopsy are warranted for a definite diagnosis.

Key words: cutaneous angiosarcoma, atypical presentation, facial erythema