Endocrine Abstracts

Cutaneous lymphoid hyperplasia (CLH) is a spectrum of benign conditions characterized by reactive B- and T-cell cutaneous lymphocytic infiltrates. The most common presentation of reactive CLH is as a solitary red skin papule, nodule, or plaque but 10–15% of patients present with more generalized or multifocal skin lesions. A 40-year old male, initially diagnosed with type 1 diabetes mellitus (DM), with a family history of autoimmune thyroid disease, vitiligo and type 2 DM, asked transfer to our service for specific treatment and monitoring. Based on detailed personal history, labs exams (positive antibodies and normal C-peptide value) and clinical evolution, the diagnosis was changed to latent autoimmune diabetes of the adult (LADA) in the context of a polyglandular autoimmune syndrome type 3 by association with vitiligo and Hashimoto thyroiditis. The physical examination revealed a round erythematous lesion on the right shoulder with positive Darier sign (sugestive for a mastocytoma), localized prurit after stimulation, 3 other circular lesions on the abdomen and telangiectasia on the epigastric area. The patient was directed to a university hematology center for further investigations, where the CHL diagnosis was established based on the tryptase and β-2 microglobulin within normal values, lack of the pAsp816Val mutation and the histopathological examination. Despite denying the diagnosis of systemic mastocytosis, the patient received treatment with an antihistamine agent and sodium cromoglycate with progressive titration up to 10 capsules/day with some improvements in gastrointestinal and urinary symptoms. The antihyperglycemic therapy consists of Metformin and basal insulin. To our knowledge, this is the first case of CLH in a patient with type 3 polyglandular autoimmune syndrome composed of LADA, Hashimoto thyroiditis and vitiligo.