In a previous study we found that only half of those patients presenting with cutaneous sarcoidal granulomas have evidence of systemic involvement. The current study was designed to determine whether abnormal angiotensin-converting enzyme (ACE) levels were predictive of multisystem disease. Serum ACE levels were determined in 15 patients with active cutaneous sarcoidal granulomas. The ACE levels were elevated in ten of the patients but did clearly differentiate those with systemic involvement. Three of the six patients with disease localized to the skin had elevated ACE levels, whereas seven of the nine patients with systemic disease had elevated ACE levels. The ACE levels did not correlate with the extent of cutaneous disease, or any individual or combined system involvement. A normal ACE level cannot be used to rule out a diagnosis of sarcoidosis and, conversely, an abnormal level does not confirm multisystem involvement.