Fahr's syndrome with dermatological manifestation
Department of Dermatology and Venereology, Ibn Rochd University Hospital, Casablanca, Morocco.
Letter to the Editor
World Journal of Advanced Research and Reviews, 2024, 21(03), 862–864
Article DOI: 10.30574/wjarr.2024.21.3.0741
Publication history:
Received on 26 January 2024; revised on 04 March 2024; accepted on 07 March 2024
Abstract:
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.
Keywords:
Fahr’s syndrome; Amicrobial pustulosis; Neuropsychiatric symptoms; Hypoparathyroidism; Central gray nuclei calcification
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