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      Recovery from alopecia areata in a patient with autoimmune polyglandular syndrome type 3

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          Summary

          Recovery from alopecia is rare in autoimmune polyglandular syndrome (APS). A 41-year-old male was admitted to our hospital with hyperglycemia. He developed alopecia areata (AA) 5 months before admission and developed thirst, polyuria, and anorexia in 2 weeks. His plasma glucose level upon admission was 912 mg/dl (50.63 mmol/l) and HbA1c was 13.7%. Although urinary and plasma C-peptide levels showed that insulin secretion was not depleted, anti-insulinoma-associated antigen 2 antibody was present. In addition, measurement of thyroid autoantibodies revealed the presence of Hashimoto's thyroiditis. These findings suggested a diagnosis of APS type 3. The patient has showed signs of improvement with the continuation of insulin therapy. During the successful control of diabetes, he had total hair regrowth within 2–3 months. Human leukocyte antigen typing showed that DRB1*1501-DQB1*0602 and DQB1*0301 were present. Similar cases should be accumulated to clarify the association of APS type 3 with recovery from AA.

          Learning points

          • Alopecia in diabetic patients is a suspicious manifestation of autoimmune type 1 diabetes.

          • Patients with autoimmune type 1 diabetes specifically manifesting alopecia should be further examined for diagnosis of APS.

          • Insulin-mediated metabolic improvement may be a factor, but not the sole factor, determining a favorable outcome of alopecia in patients with autoimmune type 1 diabetes.

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          Most cited references12

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          Alopecia areata: a long term follow-up study of 191 patients.

          The prognosis of alopecia areata (AA) is difficult to predict. Few studies report long-term follow-up of AA patients. The purpose of this study is to better assess the long-term evolution of AA and the possible relationship between disease severity and treatment response with long-term prognosis. One hundred ninety-one patients with AA who presented with a new diagnosis of AA between 1983 and 1990 were subsequently contacted by phone. Patients were queried regarding current disease status, treatments, and disease course. Severity of AA at first consultation ranged from mild (128 patients) to severe (63 patients). Fifty-five of 191 patients were affected by concomitant autoimmune or related inflammatory disease. Sixty-six of 191 patients were presently disease free (follow-up duration, 15-22 years; mean 17.74 years). These include 41 of 60 patients with S1 disease (68.3%), 22 of 68 patients with S2 disease (32.3%), 1 of 11 patients with S3 disease (9%), 1 of 14 patients with S4 disease (7.1%), and 1 of 11 patients with alopecia totalis (AT) (9.1%). Sixty-nine of 191 patients (36-1%) were presently affected by AT or alopecia universalis. There was a statistically significant tendency of severe patterns of AA to worsen over time. In children, 18 of 39 (13 with or =S3 disease) with AA had developed AT or alopecia universalis at long-term follow-up. In children, however, this trend was not statistically significant. Patients with severe AA who responded to topical immunotherapy seem to have a better prognosis than nonresponders. Follow-up was only performed by phone. Severity of AA at time of first consultation is an important prognostic factor. Response to therapy (topical immunotherapy) may be associated with better prognosis. In children, the prognosis is worse; our study found that AA worsens over time.
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            Two types of autoimmune Addison's disease associated with different polyglandular autoimmune (PGA) syndromes.

            A review of 295 patients with autoimmune Addison's disease which occurred as part of a polyglandular autoimmune syndrome is presented. Information of 41 cases was obtained from our clinics and from the examination of medical records, while 254 cases were culled from the literature. We report that autoimmune Addison's disease in association with other autoimmune diseases occurs in at least two distinct types. Addison's disease occurring in Type I polyglandular autoimmune disease (PGA) is associated with chronic mucocutaneous candidiasis and/or acquired hypoparathyroidism. The age of onset is predominately in childhood or in the early adult years. Type I PGA syndrome is also frequently associated with chronic active hepatitis, malabsorption, juvenile onset pernicious anemia, alopecia and primary hypogonadism. Insulin requiring diabetes and/or autoimmune thyroid disease are infrequent. In contrast, Addison's disease in Type II PGA is associated with insulin requiring diabetes and/or autoimmune thyroid disease(s). Although the age of onset of Addison's disease in Type II PGA syndrome is not confined to any age group or any specific sex, it occurs predominately in the middle years of life in females. The associated autoimmune diseases found in Type I disease, such as chronic active hepatitis, etc. (see table II) are rare in Type II PGA disease except for a low frequency of gonadal failure. We provide evidence to support the concept that the Addison's diseases in Type I and II PGA syndromes have different genetic bases, as related to HLA haplotypes, and possibly have different underlying pathogeneses.
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              Diagnostic criteria for acute‐onset type 1 diabetes mellitus (2012): Report of the Committee of Japan Diabetes Society on the Research of Fulminant and Acute‐onset Type 1 Diabetes Mellitus

              Abstract Type 1 diabetes is a disease characterized by destruction of pancreatic β‐cells, which leads to absolute deficiency of insulin secretion. Depending on the manner of onset and progression, it is classified as fulminant, acute‐onset or slowly progressive type 1 diabetes. Here, we propose the diagnostic criteria for acute‐onset type 1 diabetes mellitus. Among the patients who develop ketosis or diabetic ketoacidosis within 3 months after the onset of hyperglycemic symptoms and require insulin treatment continuously after the diagnosis of diabetes, those with anti‐islet autoantibodies are diagnosed with ‘acute‐onset type 1 diabetes mellitus (autoimmune)’. In contrast, those whose endogenous insulin secretion is exhausted (fasting serum C‐peptide immunoreactivity <0.6 ng/mL) without verifiable anti‐islet autoantibodies are diagnosed simply with ‘acute‐onset type 1 diabetes mellitus’. Patients should be reevaluated after certain periods in case their statuses of anti‐islet autoantibodies and/or endogenous insulin secretory capacity are unknown.

                Author and article information

                Journal
                Endocrinol Diabetes Metab Case Rep
                Endocrinol Diabetes Metab Case Rep
                edm
                EDM Case Reports
                Endocrinology, Diabetes & Metabolism Case Reports
                Bioscientifica Ltd (Bristol )
                2052-0573
                1 February 2015
                2015
                : 2015
                : 140084
                Affiliations
                [1]Department of Internal Medicine, Osaka Gyomeikan Hospital , 5-4-8 Nishikujo, Konohana-ku, Osaka, 554-0012, Japan
                Author notes
                Correspondence should be addressed to S Makino Email: makinos@ 123456ares.eonet.ne.jp
                Article
                EDM140084
                10.1530/EDM-14-0084
                4342500
                c8720905-14bb-4c06-b1b2-fccaa1f6506f
                © 2015 The authors

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License.

                History
                : 26 November 2014
                : 29 January 2015
                Categories
                Unique/Unexpected Symptoms or Presentations of a Disease

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