Vitiligo associated with other autoimmune diseases: Polyglandular autoimmune syndrome types 3B + C and 4

Department of Dermatology, University of Chieti-Pescara, Italy.
Clinical and Experimental Dermatology (Impact Factor: 1.09). 10/2006; 31(5):746-9. DOI: 10.1111/j.1365-2230.2006.02171.x
Source: PubMed


Vitiligo is a common skin disease characterized by depigmented maculae resulting from a reduction of the number and function of melanocytes. Many studies suggest that vitiligo might be an autoimmune disease. Vitiligo has been frequently described in association with other autoimmune diseases. Among the diseases described in association with vitiligo are the so-called autoimmune polyglandular syndromes (APS). Vitiligo can be present in all types of APS but the most frequent association appears to be in APS-3. APS-3 was defined as the association between autoimmune thyroiditis and another autoimmune disease. Here we report one patient with thyroiditis, vitiligo and autoimmune gastritis (APS-3B+C), one patient with chronic autoimmune thyroiditis, vitiligo and alopecia (APS-3C), and one case of a young patient with type 1 diabetes mellitus and vitiligo (APS-4), according to the newest classification. We stress the importance of a thorough assessment for autoimmune diseases in selected patients with vitiligo.

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    • "Betterle and Zanchetta distinguish four subtypes (A, B, C and D) on the basis of the different organ-specific and non-organ-specific autoimmune disease associated, excluding Addison's disease and/or hypoparathyroidism. 10 The only case of APS type 3B+C was described by Amerio et al in 2006. 11 It involved a 36-year-old woman with a 20-year history of generalised vitiligo, chronic autoimmune thyroiditis and autoimmune gastritis with high titres of antiparietal cell antibodies and pernicious anaemia. "
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    ABSTRACT: The clinical and instrumental findings revealed a case of autoimmune polyendocrine syndrome (APS) type 3B+C in a 41-year-old Caucasian woman with unexplained refractory iron-deficiency anaemia secondary to autoimmune atrophic gastritis and a long-term history of two different autoimmune diseases-Hashimoto's disease and vitiligo. Indeed the occurrence in the same patient of three or more autoimmune diseases defines APS. The first classification of APS was suggested by Neufeld and Blizzard in 1980 and it included four main types of APS on the basis of clinical features. The only case of APS type 3B+C was described by Amerio et al in 2006.
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    ABSTRACT: Autoimmune diseases constitute a heterogeneous group of disorders characterized by the loss of immune tolerance to self-antigens. Despite their distinct clinical picture, there is growing evidence that common molecular mechanisms may contribute to the whole spectrum of autoimmune diseases. This theory is strongly supported by the existence of the autoimmune polyendocrine syndromes (APS). Thus, the clinical diagnosis of APS1 is made in an individual who presents with at least two out of three cardinal symptoms, namely autoimmune Addison's disease, autoimmune hypoparathyroidism, and mucocutaneous candidiasis. APS1 is a rare autosomal recessive syndrome caused by mutations in the autoimmune regulator (AIRE) gene. APS2, which occurs at a much higher frequency, is classically defined as the coexistence of autoimmune Addison's disease, autoimmune thyroid disease, and/or type 1 diabetes. In contrast to APS1, the precise modes of inheritance and the genetic causes underlying APS2 remain unknown. Identification of genetic factors predisposing to this syndrome may contribute to our understanding of common mechanisms involved in autoimmunity.
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