Acrodermatitis chronica atrophicans (ACA) was diagnosed in 15 patients from the southern and eastern part of Friesland (the Netherlands). Twelve patients had one leg affected; three had more than one extremity involved. Frequent complaints were fatigue, paraesthesia, swelling and blue discoloration. The symptoms persisted for many years in most cases. The inflammatory stage was observed most frequently (13/15): violet-blue erythema, oedema, firm swelling and nodules. Atrophy (4/15) was observed once in the form of a scleroderma-like lesion. Neuropathy was found in five patients. Histological investigation showed infiltration with lymphocytes and plasma cells (13/15) and atrophy (2/15). Spirochaetes were demonstrated in biopsies of 13 patients. Specific antibodies against Borrelia burgdorferi were found in all patients. ACA appears to be not infrequent and must be distinguished from other inflammatory and vascular diseases, such as chronic venous insufficiency.