Psychoneuroendocrine disturbances in a patient with a rare granulomatous disease

Summary

Erdheim-Chester disease (ECD) and Langerhans' cell histiocytosis (LCH) are rare granulomatosis-like diseases of unknown etiology which are characterized by lipoidgranulomatous infiltrates in various organs. Contrary to LCH, endocrine and cerebral lesions were infrequently observed in ECD. We report on a patient with the clinical diagnosis of ECD displaying endocrine and cerebral manifestations and skeletal, pulmonary and soft tissue involvement. Disturbance of the endocrine system was revealed by enlargement of the pituitary, partial deficiency of growth hormone (GH), hyperprolactinemia and testosterone deficiency. Cerebral involvement included sinus vein thrombosis, pathologic acoustic evoked potentials, persistence of gadolinium enhancement after magnetic resonance imaging and hypomania. These findings emphasize the importance to assess endocrine and cerebral function in patients with rare granulomatous diseases like ECD and multiorgan involvement.

Key words:

Erdheim-Chester - Langerhans' cell histiocytosis - Xanthogranulomatosis - Central nervous system - Endocrine

References

• 1 Bancroft L W, Berquist T H. Erdheim-Chester disease: radiographic findings in five patients.  Skeletal Radiol. 1998;  27 127-132
• 2 Bohlega S, Alwatban J, Tulbah A, Bakheet S M, Powe J. Cerebral manifestation of Erdheim-Chester disease: clinical and radiologic findings.  Neurology. 1997;  49 1702-1705
• 3 Ferro J M, Correia M, Pontes C, Baptista M V, Pita F. Cerebral vein and dural sinus thrombosis in Portugal: 1980-1998.  Cerebrovasc Dis. 2001;  11 177-182
• 4 Grote H J, Moesenthin M, Foss H D, Kekow J, Roessner A. Osseous manifestation of Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy). A case report and review of the literature.  Gen Diagn Pathol. 1998;  143 341-345
• 5 Grothe C, Urbach H, Bös M, Ko Y, Schröder R. Zerebelläres Syndrom, Exophtalmus und sekundärer Hypogonadismus bei Erdheim-Chester-Erkrankung.  Nervenarzt. 2001;  72 449-452
• 6 Hindman B W, Thomas R D, Young L W, Yu L. Langerhans cell histiocytosis: unusual skeletal manifestations observed in thirty-four cases.  Skeletal Radiol. 1998;  27 177-181
• 7 Kaltsas G A, Powles T B, Evanson J, Plowman P N, Drinkwater J E, Jenkins P J, Monson J P, Besser G M, Grossman A B. Hypothalamo-pituitary abnormalities in adult patients with Langerhans' cell histiocytosis: clinical, endocrinological, and radiological features and response to treatment.  J Clin Endocrinol Metab. 2000;  85 1370-1376
• 8 Kenn W, Stabler A, Zachoval R, Zietz C, Raum W, Wittenberg G. Erdheim-Chester disease: a case report and literature overview.  Eur Radiol. 1999;  9 153-158
• 9 Kujat C, Martin J, Puschel W. Erdheim-Chester disease.  Radiologe. 1991a;  31 297-306
• 10 Kujat C, Junk B, Hermes M, Martin J, Dewes W. Cerebral manifestations of Erdheim-Chester disease.  Radiologe. 1991b;  31 307-309
• 11 Miller R L, Sheeler L R, Bauer T W, Bukowski R M. Erdheim-Chester disease. Case report and review of the literature.  Am J Med. 1986;  80 1230-1236
• 12 Pautas E, Cherin P, Pelletier S, Vidailhet M, Herson S. Cerebral Erdheim-Chester disease: report of two cases with progressive cerebellar syndrome with dentate abnormalities on magnetic resonance imaging.  J Neurol Neurosurg Psychiatry. 1998;  65 597-599
• 13 Ruiz-Hernandez G, Tajahuerce-Romera G M, Latorre-Ibanez M D, Vila-Fayos V, Lara-Pomares A. Epiphyseal involvement in Erdheim-Chester disease: radiographic and scintigraphic findings in a case with lytic lesions.  Nuklearmedizin. 2000;  39 N72-N73
• 14 Sandrock D, Merino M J, Scheffknecht B H, Neumann R D. Scintigraphic findings and follow up in Erdheim-Chester disease.  Eur J Nucl Med. 1990;  16 55-60
• 15 Serratrice J, Granel B, De Roux C, Pellissier J F, Swiader L, Bartoli J M, Disdier P, Weiller P J. “Coated aorta”: a new sign of Erdheim-Chester disease.  J Rheumatol. 2000;  27 1550-1553
• 16 Tien R D, Brasch R C, Jackson D E, Dillon W P. Cerebral Erdheim-Chester disease: persistent enhancement with Gd-DTPA on MR images.  Radiology. 1989;  172 791-792
• 17 Tritos N A, Weinrib S, Kaye T B. Endocrine manifestations of Erdheim-Chester disease (a distinct form of histiocytosis).  J Intern Med. 1998;  244 529-535
• 18 Vassallo R, Ryu J H, Colby T V, Hartman T, Limper A H. Pulmonary Langerhans'-cell histiocytosis.  N Engl J Med. 2000;  342 1969-1978
• 19 Veyssier-Belot C, Cacoub P, Caparros-Lefebvre D, Wechsler J, Brun B, Remy M, Wallaert B, Petit H, Grimaldi A, Wechsler B, Godeau P. Erdheim-Chester disease. Clinical and radiologic characteristics of 59 cases.  Medicine (Baltimore). 1996;  75 157-169
• 20 Wallaschofski H, Donner M, Hentschel B, Faber R, Stepan H, Koksch M, Lohmann T. Prolactin as a novel cofactor for platelet aggregation.  Exp Clin Endocrinol Diabetes. 2001;  109 A13
• 21 Wittenberg K H, Swensen S J, Myers J L. Pulmonary involvement with Erdheim-Chester disease: radiographic and CT findings.  Am J Roentgenol. 2000;  174 1327-1331
• 22 Wright R A, Hermann R C, Parisi J E. Neurological manifestations of Erdheim-Chester disease.  J Neurol Neurosurg Psychiatry. 1999;  66 72-75