Angiolymphoid hyperplasia with eosinophilia: a disease that may be confused with malignancy

Twelve new cases of an unusual, benign vasoproliferative and inflammatory disorder of unknown etiology, angiolymphoid hyperplasia with eosinophilia (ALHE), are described and contrasted clinically and pathologically with those appearing in the literature. Only recently recognized in the United States, the disease is of singular importance because the vascular component may be confused histologically with angiosarcoma, thereby resulting in unwarranted aggressive therapeutic measures. ALHE characteristically affects adults and presents in the head and neck region as either solitary or multiple cutaneous tumors. The lesions are pruritic, frequently bleed after minor trauma, and may be associated with peripheral eosinophilia and regional lymphadenopathy. On rare occasions, the disease may masquerade as a salivary-gland tumor, cause stenosis of the external auditory canal, or present as an osseous lesion of the skull. Extrafacial tumors are uncommon. Excision is the most frequent form of therapy; however, local irradiation, corticosteroids, electrodessication with curettage, and chemotherapy have also had varying degrees of success.