A rare association of polyglandular syndrome type 2 with other autoimmune conditions

Abstract

Abstract: Autoimmune polyglandular syndrome (APS) is defined as a heterogeneous group of polyendocrine diseases, which can be classified into type I and II (APS-2). APS-2 is a rare polygenic condition represented by the presence of primary autoimmune adrenal insufficiency, associated with type 1 diabetes mellitus and/or autoimmune thyroid disease. The coexistence of these three diseases, present in 10% of APS-2 cases, is called Carpenter syndrome. In APS-2, other complications can coexist that make this condition even more uncommon. In this report, we present a very rare case of a 41-year-old woman with APS-2 and the classic triad of autoimmune diseases that characterize this morbidity, associated with other autoimmune conditions, namely: vitiligo, autoimmune panniculitis, Sjögren’s syndrome, autoimmune gastritis and celiac disease. Together, all alterations present in our patient represent less than 1% of the population with APS- 2. Thus, we remember that certain signs and symptoms present in the same patient may be part of a set of different autoimmune diseases that coexist with each other.