Langerhans cell histiocytosis is a rare disease in adults with a myriad of clinical presentations. A case of multisystemic Langerhans cell histiocytosis with involvement of bone, skin, lungs, and the hypothalamic-pituitary-axis is reported. The possibility of a disseminated disease should be considered in the diagnosis of Langerhans cell histiocytosis, and these patients should undergo a careful evaluation and follow up with early institution of treatment.
NezelofCFrileux-HerbetFCronier-SachotJ. Disseminated histiocytosis X: Analysis of prognostic factors based on a retrospective study of 50 cases. Cancer. 1979 Nov;44(5):1824–38.
2.
Histiocytosis syndromes in children. Writing Group of the Histiocyte Society. Lancet. 1987;1(8526):208–9.
3.
FavaraBEFellerACPauliMJaffeESWeissLMAricoM. Contemporary classification of histiocytic disorders. The WHO Committee On Histiocytic/Reticulum Cell Proliferations. Reclassification Working Group of the Histiocyte Society. Med Pediatr Oncol. 1997 Sep;29(3):157–66.
BaumgartnerIvon HochstetterABaumertBLuetolfUFollathF. Langerhans'-cell histiocytosis in adults. Med Pediatr Oncol. 1997;28(1):9–14.
6.
AricoMGirschikofskyMGénéreauTKlersyCMcClainKGroisN. Langerhans cell histiocytosis in adults. Report from the International Registry of the Histiocyte Society. Eur J Cancer. 2003 Nov;39(16):2341–8.
7.
SchmitzLFavaraBE. Nosology and pathology of Langerhans cell histiocytosis. Hematol Oncol Clin North Am. 1998 Apr;12(2):221–46.
8.
MittheiszESeidlRPrayerDWaldenmairMNeophtouBPötschgerU. Central nervous system-related permanent consequences in patients with Langerhans cell histiocytosis. Pediatr Blood Cancer. 2007;48(1):50–6.
9.
HauptRNanduriVCalevoMGBernstrandCBraierJLBroadbentV. Permanent consequences in Langerhans cell histiocytosis patients: A pilot study from the Histiocyte Society-Late Effects Study Group. Pediatr Blood Cancer. 2004;42(5):438–44.
VassalloRRyuJHSchroederDRDeckerPALimperAH. Clinical outcomes of pulmonary Langerhans'-cell histiocytosis in adults. N Engl J Med. 2002 Feb 14;346(7):484–90.
14.
GotzGFichterJ. Langerhans'-cell histiocytosis in 58 adults. Eur J Med. Res2004 Nov 29;9(11):510–4.
15.
VassalloRJensenEAColbyTVRyuJHDouglasWWHartmanTE. The overlap between respiratory bronchiolitis and desquamative interstitial pneumonia in pulmonary Langerhans cell histiocytosis: High-resolution CT, histologic, and functional correlations. Chest. 2003 Oct;124(4):1199–205.
16.
BassetFCorrinBSpencerHLacroniqueJRothCSolerP. Pulmonary histiocytosis X. Am Rev Respir Dis. 1978 Nov;118(5):811–20.
17.
LacroniqueJRothCBattestiJPBassetFChretienJ. Chest radiological features of pulmonary histiocytosis X: A report based on 50 adult cases. Thorax. 1982;37:104–9.
18.
MooreADGodwinJDMullerNLNaidichDPHammarSPBuschmanDL. Pulmonary histiocytosis X: Comparison of radiographic and CT findings. Radiology1989 Jul;172(1):249–54.
19.
KulwiecELLynchDAAguayoSMSchwarzMIKingTEJr. Imaging of pulmonary histiocytosis X. Radiographics1992 May;12:515–26.
20.
RyuJHMossJBeckGJLeeJCBrownKKChapmanJT. The NHLBI lymphangioleiomyomatosis registry: Characteristics of 230 patients at enrollment. Am J Respir Crit Care Med. 2006;173(1):105–11.
21.
CarsilloTAstrinidisAHenskeEP. Mutations in the tuberous sclerosis complex gene TSC2 are a cause of sporadic pulmonary lymphangioleiomyomatosis. Proc Natl Acad Sci U S A. 2000 May;97(11):6085–90.
22.
AstrinidisAHenskeEP. Tuberous sclerosis complex: Linking growth and energy signaling pathways with human disease. Oncogene. 2005 Nov;24(50):7475–81.
23.
KellyJMossJ. Lymphangioleiomyomatosis. Am J Med Sci. 2001 Jan;321(1):17–25.
GlassbergMK. Lymphangioleiomyomatosis. Clin Chest Med. 2004 Sep;25(3):573–82, vii.
26.
JohnsonSRCordierJFLazorRCottinVCostabelUHarariS. European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis. Eur Respir J. 2010 Jan;35(1):14–26.
27.
ChuSCHoribaKUsukiJAvilaNAChenCCTravisWD. Comprehensive evaluation of 35 patients with lymphangioleiomyomatosis. Chest. 1999 Apr; 115(4):1041–52.
28.
BonelliFSHartmanTESwensenSJSherrickA. Accuracy of high-resolution CT in diagnosing lung diseases. AJR Am J Roentgenol. 1998 Jun;170(6):1507–12.