Fahr's syndrome with dermatological manifestation

Fahr's syndrome is a rare condition characterized by the presence of calcifications in the basal ganglia, typically secondary to a disorder of phosphocalcic metabolism, primarily hypoparathyroidism. It is generally challenging to clinically suspect as it can remain asymptomatic or manifest with diverse symptoms not corresponding to any specific clinical pattern. The dermatological expression of this syndrome is exceptional and is linked to hypoparathyroidism and hypocalcemia. In a hypocalcemic environment, keratinocytes exhibit significant proliferative activity, which may explain the pustular rash in our patients. Diagnosis relies on the presence of bilateral and symmetrical calcifications in the basal ganglia. Treatment involves calcium and vitamin D supplementation. Therefore, it is important to consider this syndrome in the presence of any aseptic pustulosis associated with disorders of phosphocalcic metabolism, particularly in cases with associated neuropsychiatric pathologies. We retrospectively report observations from six patients with Fahr's syndrome revealed through dermatological manifestations over a five-year period.