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Hypercalcemia in Necrobiotic Xanthogranuloma: First Reported Case and Insight Into Treatment
Author(s) -
Sfeir Jad G,
Zogala Richard J,
Popii Violeta B
Publication year - 2017
Publication title -
journal of bone and mineral research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.882
H-Index - 241
eISSN - 1523-4681
pISSN - 0884-0431
DOI - 10.1002/jbmr.3047
Subject(s) - medicine , granulomatous disease , pathophysiology , disease , chronic granulomatous disease , first line , dermatology , metabolic bone disease , bone disease , pathology , immunology , osteoporosis
Necrobiotic xanthogranuloma (NXG) is a rare systemic and progressive granulomatous disease first described in 1980. Given no established first‐line therapy, treatment focuses on the control of skin lesions and associated complications. Despite it being a granulomatous disease, NXG has not been associated with hypercalcemia. About 140 cases of NXG have been reported to date but, to our knowledge, this is the first case to be complicated by hypercalcemia. Our case confirms a granulomatous disease–mediated production of 1α‐hydroxylase leading to increased synthesis of 1,25‐dihydroxyvitamin D and subsequent hypercalcemia. Based on this pathophysiology, we elected to start systemic glucocorticoids, titrated to clinical and metabolic response. Steroid‐sparing agents need to be considered to avoid long‐term complications but continue controlling this granulomatous disease. © 2017 American Society for Bone and Mineral Research.