FRI644 A Rare Case Of Autoimmune Polyglandular Disease Type 3, With Scleredema Adultorum Of Buschke, Type 1 Diabetes And Graves Disease

Abstract

Abstract Disclosure: C. Pérez-Castañeda: None. G.A. González-Villaseñor: None. M. Mercado: None. A. Pozos-Ochoa: None. H.J. Medina: None. E. Gamez: None. M.T. Muñoz-Magallanes: None. Background: Scleredema Adultorum of Buschke is an uncommon skin disease, characterized by hardening and stiffness of the subcutaneous tissues, which typically starts at the neck; affecting the upper back causing immobilization; it may develop both in type 1 and type 2 diabetes mellitus and it is related with prolonged exposure to hyperglycemia, obesity and poor metabolic control. Treatment options include topical as well as systemic treatments and physical modalities; but therapy is often reported as unsuccessful. We present a case of scleredema of Buschke occurring in a patient with Autoimmune Polyglandular Disease Type 3 involving thyroid autoimmune disease and type one diabetes which to our knowledge it´s the first time to be described in literature. Clinical Case: A 42 year old male presented with history of a long standing type 1 diabetes and hypothyroidism after radioiodine treatment for refractory Graves´ disease. He complained of skin thickening and hardening in the nape, back and shoulder areas which has been slowly progressing over the last six years. Physical examination revealed a symmetric, painless, poorly defined non-pitting induration of the skin, with hyperpigmentation and peau d´ orange appearance, involving the back of the scalp, neck, upper back and both shoulders; at palpation there was a woody 1 hardness and thickness of the skin associated with failure of either rolling nor pinching the skin. There was a significant decreased range of motion in both shoulders. We performed an ultrasound which demonstrated an increased thickness of the dermis with a pronounced posterior acoustic shadow and presence of multiple echogenic dots. A magnetic resonance was subsequently conducted, revealing a thickening of the skin at subcutaneous tissue primarily through the interscapular region and a hyperintense band at the dermis. Biopsy specimen showed collagen bundles separated by gaps that correspond to mucopolysaccharide deposits. The patient was followed at the outpatient clinic, we initiated physiotherapy and we adjusted his diabetes treatment in order to achieve adequate control, the patient now refers a steady state on the clinical picture. Conclusion: Scleredema is a rare disease, treatment options include topical as well as systemic treatments and physical modalities; but therapy is often reported as unsuccessful, probably associated with the few cases published in the literature; so there is no standard therapeutic protocol. The course is usually unpredictable and the disease itself is refractory. This is the first case reporting the rare association of type one diabetes, Grave s disease and scleredema of Buschke. 2 Presentation: Friday, June 16, 2023