[Infrequent clinical manifestations in a patient with eosinophilic granuloma]

We report the case of a 54 year old male with diabetes insipidus and diabetes mellitus who developed, twenty years later, signs of hypogonadotropic hypogonadism, secondary adrenal insufficiency and primary hypothyroidism with positive serum antithyroglobulin antibodies. At that time, an osteolytic lesion on the left temporal bone was localized by radiologic studies. A bone biopsy confirmed the diagnosis of eosinophilic granuloma. Laboratory tests also showed anemia, hypergammaglobulinemia and accelerated eritrosedimentation rate which constitute atypical manifestations of this rare disease in adults. The probable etiopathogenic mechanisms are discussed: while some authors have described an autoimmune primary defect, others have suggested a primary viral infection as the cause of Hand-Schuller-Christian disease. The association of direct and indirect evidence of immunologic alterations in our patient is suggestive of an autoimmune origin.