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Article Abstract

Polyglandular auto-immune syndromes are uncommon constellation of organ specific auto-immune diseases, characterised by the existence of two or more endocrinopathies. Polyglandular autoimmune type II syndrome also known as Schmidt's syndrome is more common, comprising Addison's disease, auto-immune thyroid disease, type 1 diabetes mellitus and/or hypogonadism, pernicious anaemia, coeliac disease, vitiligo, hypophysitis, etc. Here a case of a 56-year-old man is reported with a prior history of hypothyroidism who presented with adrenal crisis. Further laboratory investigations revealed primary auto-immune hypothyroidism, primary adrenal insufficiency, hypogonadism and he was diagnosed as a case of polyglandular auto-immune type II syndrome or Schmidt's syndrome. Early recognition of the syndrome and replacement therapy can be life saving, particularly when there is adrenal or thyroid insufficiency.

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Aims And Objectives: Clinical, biochemical, and radiological profiles of Addison's disease and to assess the various etiological spectrum of primary adrenal insufficiency (PAI) in adults.

Materials And Methods: A retrospective cohort study was carried out in the Department of Endocrinology, Madurai Medical College, Madurai between January 2014 and January 2021 over a 7-year period.

Inclusion Criteria: All the patients with clinical symptoms and or signs of suspected PAI, such as hyperpigmentation, weight loss, persistent nausea or vomiting, fatigue, and hypotension, were recruited.

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We report an interesting case of a 38-year-old woman presenting with reverse Takotsubo syndrome (TTS) secondary to an Addisonian crisis, her second such episode. A few years prior, she had presented with typical TTS in the setting of Addisonian crisis; diagnostic work-up revealing Auto-Immune Polyglandular Syndrome Type II (APS II). We believe this to be the first case report of typical and variant phenotypes of TTS in a patient with APS II.

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[The challenge for dermatologists of early APECED diagnosis].

Ann Dermatol Venereol

April 2014

Service de dermatologie, CHU Jean Minjoz, 3, boulevard Alexandre-Fleming, 25030 Besançon cedex, France; EA3081, SFR FED 4234 IBCT, université de Franche-Comté, 25030 Besançon cedex, France.

Background: Polyglandular auto-immune syndrome type 1 (PAS-1) or auto-immune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is a rare autosomal recessive disorder linked to auto-immune regulator (AIRE) gene mutations. Herein, we report the case of a 3-year-old boy with APECED emphasizing the wide phenotypic variability and the extent of skin lesions.

Patients And Methods: A 3-year-old boy with a history of auto-immune hepatitis was referred for a generalized pruriginous urticaria-like eruption present for one month.

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