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A Case of Critical Lower-Limb Ischemia in a 29-Year-Old Man with Autoimmune Polyglandular Syndrome Type 1 (APS-1)

Rare disease

Raed M. Ennab, Nesreen A. Saadeh

Jordan Department of Clinical Sciences, Yarmouk University, Irbid, Jordan

Am J Case Rep 2020; 21:e924705

DOI: 10.12659/AJCR.924705

Available online: 2020-09-10

Published: 2020-10-17


#924705

BACKGROUND: Autoimmune polyglandular syndrome type 1 (APS-1) is an extremely rare autoimmune disorder with an autosomal recessive inheritance pattern. Its manifestations present in chronological sequence of the components mucocutaneous candidiasis, Addison disease, and hypoparathyroidism. Vascular calcification is a very rare manifestation of the disease, and it may be severe, causing critical lower-limb ischemia and significant morbidity. To the best of our knowledge, this is the first such case to be reported in Jordan and the Arab region.
CASE REPORT: We present the case of a 29-year-old patient diagnosed with autoimmune polyglandular syndrome type 1 (APS-1). He has Addison disease, hypoparathyroidism, and mucocutaneous candidiasis. He presented with features of critical lower-limb ischemia and bacterial infection of the left foot. The patient underwent a successful angioplasty, and received management of his bacterial and fungal infections and the chronic endocrinopathies.
CONCLUSIONS: Autoimmune polyglandular syndrome type 1 (APS-1) is a very rare disorder. Recognizing its syndromic nature will facilitate an active search for the component diseases and the possible complications, which would allow early diagnosis and management. This applies to the rare vascular complications, which can lead to significant morbidity.

Keywords: Addison Disease, Angioplasty, Candidiasis, Chronic Mucocutaneous, hypoparathyroidism, peripheral arterial disease, Polyendocrinopathies, Autoimmune



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