This Article Full Text Full Text (PDF) All Versions of this Article: 92/6/2007    most recent Author Manuscript (PDF) Submit a related Letter to the Editor Purchase Article View Shopping Cart Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Copyright Permission Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Haroche, J. Articles by Piette, J.-C. Search for Related Content PubMed PubMed Citation Articles by Haroche, J. Articles by Piette, J.-C. Related Collections Adrenal and Hypertension

Bilateral Adrenal Infiltration in Erdheim-Chester Disease. Report of Seven Cases and Literature Review

Service de Médecine Interne (J.H., Z.A., B.W., J.-C.P.), Service d’Endocrinologie et Médecine de la Reproduction (P.T.), Service de Neuropathologie (D.S.), and Service de Radiologie (C.G., P.C., P.A.G.), Hôpital Pitié-Salpêtrière, 75013 Paris, and Université Pierre et Marie Curie, Paris 6, France; and Service de Néphrologie-Immunologie Clinique (B.B.), Hôpital Bretonneau, 37044 Tours, France

Address all correspondence and requests for reprints to: Julien Haroche, M.D., Ph.D., Service de Médecine Interne, Hôpital Pitié-Salpêtrière, 47-83 Bld de l’Hôpital, 75013 Paris, France. E-mail: julien.haroche{at}psl.aphp.fr.

Context: Erdheim-Chester disease (ECD) is a rare non-Langerhans form of histiocytosis characterized by xanthomatous tissue infiltration with CD68⁺ CD1a^– foamy histiocytes. Endocrine problems, such as diabetes insipidus and hypogonadotropic hypogonadism, frequently occur in ECD, but bilateral adrenal infiltration has rarely been reported in this disease.

Objective: The aim of the study was to describe adrenal and periadrenal infiltration in ECD.

Patients: A total of 22 patients with ECD undergoing systematic computed tomography to search for signs of adrenal enlargement participated in the study.

Results: Of the 22 patients with ECD, seven (31.8%) displayed adrenal infiltration on computed tomography. In one case, autopsy confirmed that the adrenal enlargement was due to foamy histiocyte infiltration in the adrenal glands. Possible adrenal insufficiency was assessed in five of the seven patients. One developed signs of adrenal insufficiency, which was confirmed by adrenocorticotropin stimulation tests. Adrenal involvement was reported in only 15 of the 240 ECD cases published up to May 2006. This frequency is significantly lower than that in our series (P = 0.0008; Fisher’s exact test).

Conclusion: Physicians should be aware of ECD as a possible cause of morphological changes in adrenal size and infiltration.