Macular arteritis: a pole of the spectrum of cutaneous polyarteritis nodosa?

early diagnosis (often based on the detection of angiokeratomas) is important, as timely treatment can prevent the development of serious, irreversible complications. Dermatologists can play a key role in this respect. Enzyme replacement therapy (with agalsidase alpha) is the only specific treatment available for Fabry disease, and has been shown to stabilize renal function and reduce left ventricular hypertrophy. In our series, treatment did not improve any of the skin manifestations present before treatment initiation. In conclusion, the detection of angiokeratomas, regardless of their location or distribution, should alert the dermatologist to a possible diagnosis of Fabry disease and prompt the performance of relevant tests to confirm or rule out this serious, treatable disease. Young relatives of newly diagnosed patients should also be studied, with a particular focus on symptoms that may have been missed or interpreted incorrectly.