Abstract
Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of bone marrow-derived antigen-presenting cells that home to diverse organs and tissues, predominantly the skin. LCH is a rare disease that mainly manifests in the pediatric age group, with several in part overlapping phenotypes. Clinically and morphologically, LCH is divided into unifocal LCH, single-system LCH, multifocal LCH, multiorgan/disseminated LCH, Langerhans cell sarcoma, and Langerhans tumors. All forms and variants are characterized by a progressive and monoclonal proliferation of medium-sized, CD1a-reactive, and langerin-/CD207-reactive cells that often contain Birbeck granules. Langerhans cell infiltrates are regularly accompanied by infiltrates of eosinophils, lymphoid cells, and macrophages. Lesions with a high density of eosinophils were previously termed eosinophilic granuloma. Hepatobiliary involvement in LCH presents with a characteristic spectrum of lesions that include a disseminated form, focal forms (microscopic and macroscopic), and a form associated with bile ducts (LCH cholangiopathy). A small subset of Langerhans cell proliferations present as tumorous lesions or Langerhans cell sarcoma. In addition to LCH, there are a group of so-called self-healing Langerhans cell and related disorders that can involve the hepatobiliary tract.
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Abdallah M, Généreau T, Donadieu J, Emile JF, Chazouillères O, Gaujoux-Viala C et al (2011) Langerhans’ cell histiocytosis of the liver in adults. Clin Res Hepatol Gastroenterol 35:475–481
Abla O, Egeler RM, Weitzman S (2010) Langerhans cell histiocytosis: current concepts and treatments. Cancer Treat Rev 36:354–359
Abt AF, Denenholz EJ (1936) Letterer-Siwe disease: splenomegaly associated with widespread hyperplasia of nonlipoid-storing macrophages; discussion of the so-called reticulo-endothelioses. Am J Dis Child 51:499–522
Adamson HG (1905) Society intelligence: the Dermatological Society of London. Br J Dermatol 17:222
Al Ghamdi KM, Al Suwaidan SN (2010) Penile juvenile xanthogranuloma and neurofibromatosis type-1: risk association with juvenile myelomonocytic leukaemia? East Mediterr Health 16:451–454
Al-Abbadi M, Masih A, Braylan RC, Almasri NM (1997) Soft tissue Langerhans’ cell histiocytosis in an adult. A case presentation with flow cytometric analysis and literature review. Arch Pathol Lab Med 121:169–172
Alexander AS, Turner R, Uniate L, Pearcy RG (2005) Xanthoma disseminatum: a case report and literature review. Br J Radiol 78:153–157
Allen CE, McClain KL (2007) Langerhans cell histiocytosis: a review of past, current and future therapies. Drugs Today (Barc) 43:627–643
Almanaseer IY, Kosova L, Pellettiere EV (1986) Composite lymphoma with immunoblastic features and Langerhans’ cell granulomatosis (histiocytosis X). Am J Clin Pathol 85:111–114
Altman J, Winkelmann RK (1962) Xanthoma disseminatum. Arch Dermatol 86:582–596
Aparicio G, Mollet J, Batralot R, Bodet D, Heras C, Bassas P, Viros A, Garcia-Patos V (2008) Eruptive xanthogranuloma associated with relapsing acute lymphoblastic leukemia. Pediatr Dermatol 25:487–488
Arachchillage DR, Carr TF, Kerr B, Hawkins K, Kelsey A, Judge M, Wynn RF (2010) Juvenile myelomonocytic leukemia presenting with features of neonatal hemophagocytic lymphohistiocytosis and cutaneous juvenile xanthogranulomata and successfully treated with allogeneic hemopoietic stem cell transplant. J Pediatr Hematol Oncol 32:152–155
Arakawa A, Matsukawa T, Yamashita Y, Yoshimatsu S, Ohtsuka N, Miyazaki T, Yamamoto H, Harada M, Ishimaru Y, Takahashi M (1994) Periportal fibrosis in Langerhans’ cell histiocytosis mimicking multiple liver tumors: US, CT, and MR findings. J Comput Assist Tomogr 18:157–159
Arico M, Egeler RM (1998) Clinical aspects of Langerhans cell histiocytosis. Hematol Oncol Clin North Am 12:247–257
Arico M, Girschikofsky M, Genereau T, Klersy C, McClain K, Grois N, Emile JF, Lukina E, De Juli E, Danesino C (2003) Langerhans cell histiocytosis in adults. Report from the International Registry of the Histiocyte Society. Eur J Cancer 39:2341–2348
Aterman K, Remmele W, Smith M (1988) Karl Touton and his “xanthelasmic giant cell.” A selective review of multinucleated giant cells. Am J Dermatopathol 10:257–269
Avery ME, McAfee JG, Guld HG (1957) The course and prognosis of reticuloendotheliosis (eosinophilic granuloma, Schüller-Christian disease and Letterer-Siwe disease): a study of forty cases. Am J Med 22:636–652
Azorin D, Torrelo A, Lassaletta A, de Prada I, Colmenero I, Contra T, Gonzalez-Mediero I (2009) Systemic juvenile xanthogranuloma with fatal outcome. Pediatr Dermatol 26:709–712
Badalian-Very G, Vergilio JA, Degar BA, MacConail LE, Brandner B, Calicchio ML et al (2010) Recurrent BRAF mutations in Langerhans cell histiocytosis. Blood 116:1919–1923
Badalian-Very G, Vergilio JA, Degar BA, Rodriguez-Galindo C, Rollins BJ (2012) Recent advances in the understanding of Langerhans cell histiocytosis. Br J Haematol 156:163–172
Badalian-Very G, Vergilio JA, Fleming M, Rollins BJ (2013) Pathogenesis of Langerhans cell histiocytosis. Annu Rev Pathol 8:1–20
Bains A, Parham DM (2011) Langerhans cell histiocytosis preceding the development of juvenile xanthogranuloma: a case and review of recent developments. Pediatr Dev Pathol 14:480–484
Bank MI, Rengtved P, Carstensen H, Petersen BL (2003) Langerhans cell histiocytosis: an evaluation of histopathological parameters, demonstration of proliferation by Ki-67 and mitotic bodies. APMIS 111:300–308
Batista AC, Mendonça EF, Arantes Elias LS, Andrade BA, Almeida OP, Leon JE (2012) Nonlipidized juvenile xanthogranuloma: an unusual variant with a potential diagnostic pitfall. Int J Pediatr Otorhinolaryngol 76:295–299
Batson R, Shapiro JL, Christie A, Riley HD (1955) Acute nonlipid disseminated reticuloendotheliosis. AMA J Dis Child 90:323–343
Battaglini J, Olsen TG (1984) Disseminated xanthosiderohistiocytosis, a variant of xanthoma disseminatum, in a patient with a plasma cell dyscrasia. J Am Acad Dermatol 11:750–755
Belhadjali H, Mohamed M, Mahmoudi H, Youssef M, Moussa A, Chouchane S, Chouchane A et al (2008) Self-healing Langerhans cell histiocytosis (Hashimoto-Pritzker disease): two Tunisian cases. Acta Dermatovenerol Alp Panonica Adriat 17:188–192
Berger TG, Lane AT, Headington JT, Hartmann K, Burrish G, Levin MW, Disantagnese PA, Schwartz JL, Lambert JS (1986) A solitary variant of congenital self-healing reticulohistiocytosis: solitary Hashimoto-Pritzker disease. Pediatr Dermatol 3:230–236
Bonifazi E, Caputo R, Ceci A, Meneghini C (1982) Congenital self-healing histiocytosis. Arch Dermatol 118:267–272
Braier J, Ciocca M, Latella A, de Davila MG, Drajer M, Imventarza O (2002) Cholestasis, sclerosing cholangitis, and liver transplantation in Langerhans cell histiocytosis. Med Pediatr Oncol 38:178–182
Broadbent V, Egeler RM, Nesbit ME (1994) Langerhans cell histiocytosis – clinical and epidemiological aspects. Br J Cancer Suppl 23:S11–S16
Brown NA, Furtado LV, Betz BL, Kiel MJ, Weigelin HC, Lim MS, Elenitoba-Johnson KS (2014) High prevalence of somatic MAP2K1 mutations in BRAF V600E-negative Langerhans cell histiocytosis. Blood 124:1655–1658
Burgdorf WH, Zelger B (1996) The non-Langerhans’ cell histiocytoses in childhood. Cutis 58:201–207
Butler DF, Ranatunge BD, Rapini RP (2001) Urticating Hashimoto-Pritzker Langerhans cell histiocytosis. Pediatr Dermatol 18:41–44
Cabrera RA, Lacerda A, Lemos MM, Nunes JM, Mendonça ME (2005) Multiple visceral and subcutaneous nodules in a 4-month infant. Cytopathology 16:309–311
Calero G, Vallarino J, Centeno J (1986) Illig-Fanconi disease. First case described in Ecuador (in Spanish). Med Cutan Ibero Lat Am 14:1–4
Calverly DC, Wismer J, Rosenthal D, deSa D, Barr RD (1995) Xanthoma disseminatum in an infant with skeletal and marrow involvement. J Pediatr Hematol Oncol 17:61–65
Cambazard F, Kanitakis J, Zambruno G, Barre P, Jacquemier D, Thivolet J (1988) Hashimoto-Pritzker congenital self-healing histiocytosis (in French). Pediatrie 43:213–217
Campourcy M, Moreau-Cabarrot A, Gorguet B, Samalens G, Daste B, Eclache F, Bazex J (1997) Acquired regressive cutaneous non-Langerhans-cell histiocytosis in an infant (in French). Ann Dermatol Venereol 124:167–170
Caputo R, Veraldi S, Grimalt R, Gianotti R, Tosti A, Varotti C, de Kaminsky AR (1995) The various clinical patterns of xanthoma disseminatum. Considerations on seven patients and review of the literature. Dermatology 190:19–24
Caputo R, Passoni E, Cavicchini S (2003) Papular xanthoma associated with angiokeratoma of Fordyce: considerations on the nosography of this rare non-Langerhans cell histiocytoxanthomatosis. Dermatology 206:165–168
Caruso S, Miraglia R, Maruzzelli L, Luca A, Gridelli B (2008) Biliary wall calcification in Langerhans cell histiocytosis: report of two cases. Pediatr Radiol 38:791–794
Caruso S, Miraglia R, Spada M, Luca A, Gridelli B (2009) Biliary dilatation secondary to lithiasis in a child affected by Langerhans’ cell histiocytosis. J Clin Ultrasound 37:366–368
Castro EC, Blazquez C, Boyd J, Correa H, de Chadarevian JP, Felgar RE, Graf N et al (2010) Clinicopathologic features of histiocytic lesions following ALL, with a review of the literature. Pediatr Dev Pathol 13:225–237
Cavazza A, Pasquinelli G, Carlinfante G, Cenini E, Bonvicini U, Gardini G (1999) Nodular Langerhans cell histiocytosis of the liver in an adult with colonic adenocarcinoma. Histopathology 34:273–275
Celic D, Rados J, Lipozencic J, Dobric I (2004) Xanthoma disseminatum: case report. Acta Dermatovenerol Croat 12:282–288
Chabrol E, Thépaut M, Dezutter-Dambuyant C, Vivès C, Marcoux J, Kahn R, Valladeau-Guillemond J, Vachettet P et al (2015) Alteration of the langerin oligomerization state affects Birbeck granule formation. Biophys J 108:666–677
Chakraborty R, Hampton OA, Shen X, Sinko S, Shih A, Abhyankar H, Lim KP, Covington K, Trevino L et al (2014) Mutually exclusive recurrent somatic mutations in MAP2K1 and BRAF support a central role for ERK activation in LCH pathogenesis. Blood 124:3007–3015
Cham E, Siegel D, Ruben BS (2010) Cutaneous xanthogranulomas, hepatosplenomegaly, anemia, and thrombocytopenia as presenting signs of juvenile myelomonocytic leukemia. Am J Clin Dermatol 11:67–71
Chan YL, Li CK, Lee CY (1997) Sonographic appearance of hepatic Langerhans cell histiocytosis. Clin Radiol 52:761–763
Chang MW (1999) Update on juvenile xanthogranuloma: unusual cutaneous and systemic variants. Semin Cutan Med Surg 18:195–205
Chantorn R, Wisuthsarewong W, Aanpreung P, Sanpakit K, Manonukul J (2008) Severe congenital systemic juvenile xanthogranuloma in monozygotic twins. Pediatr Dermatol 25:470–473
Chantranuwat C (2004) Systemic form of juvenile xanthogranuloma: report of a case with liver and bone marrow involvement. Pediatr Dev Pathol 7:646–648
Chaudhary A, Debnath J, Thulkar S, Seth T, Sinha A (2006) Imaging findings in hepatic Langerhans’ cell histiocytosis. Indian J Pediatr 73:1036–1038
Chen BW, Chang MH, Lin DT, Lin KH, Chuu WM, Lin KS (1989) Extrahepatic biliary obstruction caused by cancer of non-liver origin in children: report of 5 cases. Taiwan Yi Xue Hui Za Zhi 88:819–823
Childers JH, Price PC (1954) Letterer-Siwe’s disease; report of case. AMA Arch Pathol 58:142–150
Christian HA (1919) Defects in membranous bone, exophthalmos and diabetes insipidus. In: Contributions to medical and biological research: dedicated to Sir William Osler, Bart., M.D., F.R.S., in honor of his seventieth birthday, July 12, by his pupils and c-workers. New York, Paul B. Hoeber, vol. 1, pp 390–401
Chu, Jaffe (1994) PMID 7521202
Chun SI, Song MS (1992) Congenital self-healing reticulohistiocytosis – report of a case of the solitary type and review of the literature. Yonsei Med J 33:194–198
Chunharas A, Pabunruang W, Hongeng S (2002) Congenital self-healing Langerhans cell histiocytosis with pulmonary involvement: spontaneous regression. J Med Assoc Thai 85(Suppl 4):S1309–S1313
Coldiron BM, Cruz PD, Freeman RG, Sontheimer RD (1988) Benign non-X histiocytosis: a unique case bridging several of the non-X histiocytic syndromes. J Am Acad Dermatol 18:1282–1289
Collin M, Bigley V, McClain KL, Allen CE (2015) Cells(s) of origin of Langerhans cell histiocytosis. Hematol Oncol Clin North Am 29:825–838
Concepcion W, Esquival CO, Terry A, Nakazato P, Garcia-Kennedy R, Houssin D et al (1991) Liver transplantation in Langerhans’ cell histiocytosis (histiocytosis X). Semin Oncol 18:24–28
Contreras F, Fonseca E, Gamallo C, Burgos E (1990) Multiple self-healing indeterminate cell lesions of the skin in an adult. Am J Dermatopathol 12:396–401
Cooper PH, Frierson HF, Kayne AL, Sabio H (1984) Association of juvenile xanthogranuloma with juvenile myeloid leukemia. Arch Dermatol 120:371–375
Coppes-Zantinga A, Egeler RM (2002) Historical. review. The Langerhans cell histiocytosis X files revealed. Br J Haematol 116:3–9
Cugati G, Singh M, Pande A, Ramamurthi R, Vasudevan MC (2011) Hand Schuller Christian disease. Indian J Med Paediatr Oncol 32:183–184
Davies CWH, Marren P, Juniper MC, Gray W, Wojnorowska F, Benson MK (2000) Xanthoma disseminatum with respiratory tract involvement and fatal outcome. Thorax 55:170–172
De Graaf JH, Timens W, Tamminga RY, Molenaar WM (1992) Deep juvenile xanthogranuloma: a lesion related to dermal indeterminate cells. Hum Pathol 23:905–910
Debray D, Pariente D, Urvoas E, Hadchouel M, Bernard O (1994) Sclerosing cholangitis in children. J Pediatr 124:49–56
Dehner LP (2003) Juvenile xanthogranulomas in the first two decades of life: a clinicopathologic study of 174 cases with cutaneous and extracutaneous manifestations. Am J Surg Pathol 27:579–593
Delabie J, De Wolf-Peeters C, De Vos R, Vandenberghe E, Kennes K, De Jonge I, Desmet V (1991) True histiocytic neoplasm of Langerhans’ cell type. J Pathol 163:217–223
Di Blasi A, de Seta L, Marsilia GM, Coletta S, Siani P, de Rosa I (1993) Juvenile xanthogranuloma. Description of a case with liver involvement (in Italian). Pathologica 85:85–90
Diard F, Cadier L, Billaud C, Trojani M (1982) Neonatal juvenile xanthogranulomatosis with pulmonary, extrapleural and hepatic involvement. One case report (in French). Ann Radiol (Paris) 25:113–118
Dorfman DM, Hornick JL, Shahsafaei A, Freeman GJ (2010) The phosphatidylserine receptors, T cell immunoglobulin mucin proteins 3 and 4, are markers of histiocytic sarcoma and other histiocytic and dendritic cell neoplasms. Hum Pathol 41:1486–1494
Dorjsuren G, Kim HJ, Jung JY, Bae BG, Lee JH (2011) Solitary type of congenital self-healing reticulohistiocytosis. Ann Dermatol 23(Suppl 1):S4–S7
Duncan WK, Post AC, McCoy BP (1988) Eosinophilic granuloma. Oral Surg Oral Med Oral Pathol 65:736–741
El Demellawy D, Young JL, de Nanassy J, Chernetsova E, Nasr A (2015) Langerhans cell histiocytosis: a comprehensive review. Pathology 47:294–301
Ersoy-Evans S, Gursoy T, Yigit S, Akcoren Z, Sahin S (2006) Solitary congenital self-healing reticulohistiocytosis in monozygotic twins. Pediatr Dermatol 23:273–275
Fan R, Sun J (2011) Neonatal systemic juvenile xanthogranuloma with an ominous presentation and successful treatment. Clin Med Insights Oncol 5:157–161
Favara BE (1981) The pathology of “histiocytosis”. Am J Pediatr Hematol Oncol 3:45–56
Favara BE (1996) Histopathology of the liver in histiocytosis syndromes. Pediatr Pathol Lab Med 16:413–433
Favara BE, McCarthy RC, Meirau GW (1983) Histiocytosis X. Hum Pathol 14:663–676
Favara et al. (1997) PMID 9212839
Ferrando J, Estrach T, Bombi JA, Bassas S, Navarra E, Simon A (1982) Self-healing childhood histiocytosis X (Illig-Fanconi disease). Comments on ultrastructural aspects and etiopathogenic classification of histiocytosis (in Spanish). Med Cutan Ibero Lat Am 10:323–338
Ferrando J, Campo-Voegeli A, Soler-Carrillo J, Munoz E, Sole M, Palou J, Conill C et al (1998) Systemic xanthohistiocytoma: a variant of xanthoma disseminatum? Br J Dermatol 138:155–160
Finn LS, Jaffe R (1997) Langerhans cell granuloma confined to bile duct. Pediatr Pathol Lab Med 17:461–468
Fione and Rizzo (1959) PMID 13656855
Foschini MP, Milandri GL, Dina RE, Usellini L, Macchia S, Spongano P (1995) Benign regressing histiocytosis of the liver. Histopathology 26:363–366
Freyer DR, Kennedy R, Bostrom BC, Kohut G, Dehner LP (1996) Juvenile xanthogranuloma: forms of systemic disease and their clinical implications. J Pediatr 129:227–237
Gadner H, Minkov M, Grois N, Pötschger U, Thiem E, Arico M, Astigarraga I, Braier J et al (2013) Therapy prolongation improves outcome in multi-system Langerhans cell histiocytosis. Blood 121:5006–5014
Gee SN, Huang JT, Schmidt BA, Gellis SE (2013) Rapidly fatal multiorgan Langerhans cell histiocytosis in a neonate. Pediatr Dermatol 30:e85–e86
Gianotti F, Caputo R, Ranzi T (1968) Ultrastructural study of giant cells and “Langerhans cell granules” in cutaneous lesions and lymph-node and liver biopsies from four cases of subacute disseminated histiocytosis of Letterer-Siwe. Arch Klin Exp Dermatol 233:238–252
Gianotti R, Alessi E, Caputo R (1993) Benign cephalic histiocytosis: a distinct entity or a part of a wide spectrum of histiocytic proliferative disorders of children? A histopathologic study. Am J Dermatopathol 15:315–319
Granot E, Cohen P, Asli Y, Bar-Ziv J (1994) Histiocytosis X presenting as prolonged cholestatic jaundice in childhood. Eur J Gastroenterol Hepatol 6:275–279
Grosfeld JL, Fitzgerald JF, Wagner VM, Newton WA, Baehner RL (1976) Portal hypertension in infants and children with histiocytosis X. Am J Surg 131:108–113
Guilarte Lopez-Manas J, Bellot Garcia V, Fernandez Perez R, Caballero Morales T, Ardoy Ibanez FM, Gallego Rojo FJ (1997) Multifocal Langerhans-cell granulomatosis with hepatic lesions simulating tumors (in Spanish). Gastroenterol Hepatol 20:407–411
Guthery SL, Heubi JE (2001) Liver involvement in childhood histiocytic syndromes. Curr Opin Gastroenterol 17:474–478
Guthrie JA, Arthur RJ (1994) Case report: juvenile xanthogranuloma with pulmonary, subcutaneous and hepatic involvement. Clin Radiol 49:498–500
Hadzic et al (2000) PMID 11003364
Hand A (1893) Polyuria and tuberculosis. Arch Pediatr 10:673
Hand A (1921) Defects of membranous bones, exophthalmos and polyuria in childhood: is it dyspituitarism? Am J Med Sci 162:509
Hara T, Mizuno Y, Ishii E, Ueda K, Hirata T, Daimaru Y, Nakagawara A (1988) Histiocytosis X presenting as multiple intrahepatic nodules. Nippon Ketsueki Gakkai Zasshi 51:1059–1062
Hashimoto K, Pritzker MS (1973) Electron microscopic study of reticulohistiocytoma. Arch Dermatol 107:263–270
Hashimoto K, Bale GF, Hawkins HK, Langston C, Pritzker MS (1986) Congenital self-healing reticulohistiocytosis (Hashimoto-Pritzker type). Int J Dermatol 25:516–523
Hashimoto K, Schachner LA, Huneiti A, Tanaka K (1999) Pagetoid self-healing Langerhans cell histiocytosis in an infant. Pediatr Dermatol 16:121–127
Hatakeyama N, Hori T, Yamamoto M, Sogawa I, Inazawa N, Tsutsumi H, Suzuki N (2009) An infant with self-healing cutaneous Langerhans cell histiocytosis followed by isolated thymic relapse. Pediatr Blood Cancer 53:229–231
Haughton AM, Horii KA, Shao L, Daniel J, Nopper AJ (2008) Disseminated juvenile xanthogranulomatosis in a newborn resulting in liver transplantation. J Am Acad Dermatol 58(2 Suppl):S12–S15
Haupt R, Monkov M, Astigarraga I, Schäfer E, Nanduri V, Jubran R, Egeler RM, Janka G et al (2013) Langerhans cell histiocytosis (LCH): guidelines for diagnosis, clinical work-up, and treatment for patients till the age of 18 years. Pediatr Blood Cancer 60:175–184
Heitner R, Mouton S, Rabinowitz L, Rosen EU (1978) Type I histiocytosis X presenting as biliary atresia. A case report. S Afr Med J 53:768–770
Henderson DW, Sage RE (1973) Malignant histiocytosis with eosinophils. Cancer 32:1421–1428
Hernandez-Martin A, Baselga E, Drolet BA, Esterly NB (1997) Juvenile xanthogranuloma. J Am Acad Dermatol 36:355–367
Herzog KM, Tubbs RR (1998) Langerhans cell histiocytosis. Adv Anat Pathol 5:347–358
Heyn RM, Hamoudi A, Newton WA (1990) Pretreatment liver biopsy in 20 children with histiocytosis X: a clinicopathological correlation. Med Pediatr Oncol 18:110–118
Higgins CR, Tatnall FM, Leigh IM (1994) Vesicular Langerhans cell histiocytosis – an uncommon variant. Clin Exp Dermatol 19:350–352
Hu WK, Gilliam AC, Wiersma SR, Dahms BB (2004) Fatal congenital systemic juvenile xanthogranuloma with liver failure. Pediatr Dev Pathol 7:71–76
Hutter C, Kauer M, Simonitsch-Klupp I, Jug G, Schwentner R, Leitner J, Bock P, Steinberger P et al (2012) Notch is active in Langerhans cell histiocytosis and confers pathognomonic features on dendritic cells. Blood 120:5199–5208
Ibarrola de Andres C, Toscano R, Lahuerta JJ, Martinez-Gonzalez MA (1999) Simultaneous occurrence of Hodgkin’s disease, nodal Langerhans’ cell histiocytosis and multiple myeloma IgA(kappa). Virchows Arch 434:259–262
Imamura M, Sakamoto S, Hanazono H (1971) Malignant histiocytosis: a case of generalized histiocytosis with infiltration of Langerhans’ granule containing histiocytes. Cancer 28:267–275
Inuzuka M, Tomita K, Tokura Y, Takigawa M (2003) Congenital self-healing reticulohistiocytosis presenting with hemorrhagic bullae. J Am Acad Dermatol 48(Suppl 5):S75–S77
Itoh H, Miyaguni H, Kataoka H, Akiyama Y, Tateyama S, Marutsuka K, Asada Y, Ogata K, Koono M (2001) Primary cutaneous Langerhans cell histiocytosis showing malignant phenotype in an elderly woman: report of a fatal case. J Cutan Pathol 28:371–378
Iupati D, Chander S (2006) A case report of Langerhans histiocytosis presenting sequentially over a 21-year-period with Letterer-Siwe disease, Hand-Schuller-Christian disease and eosinophilic granuloma of bone. J Pediatr Hematol Oncol 28:746–749
Iwai M, Kashiwadani M, Okuno T, Takino T, Koshokawa T (1988) Cholestatic liver disease in a 20-year-old woman with histiocytosis X. Am J Gastroenterol 83:164–168
Jaffe R (2004) Liver involvement in the histiocytic disorder of childhood. Pediatr Dev Pathol 7:214–225
Jain A, Mathur K, Khatri S, Kasana S, Jain SK (2011) Rare presentation of juvenile xanthogranuloma in the thoracic spine of an adult patient: case report of literature review. Acta Neurochir (Wien) 153:1813–1818
Jang KA, Ahn SJ, Choi JH, Sung KJ, Moon KC, Koh JK (2000) Histiocytic disorders with spontaneous regression in infancy. Pediatr Dermatol 17:364–368
Janssen D, Harms D (2005) Juvenile xanthogranuloma in childhood and adolescence: a clinicopathologic study of 129 patients from the Kiel pediatric tumor registry. Am J Surg Pathol 29:21–28
Janssen D, Fölster-Holst R, Harms D, Klapper W (2007) Clonality in juvenile xanthogranuloma. Am J Surg Pathol 31:812–813
Jesenak M, Plamenova I, Plank L, Banovcin P (2013) Wiskott-Aldrich syndrome caused by a new mutation associated with multifocal dermal juvenile xanthogranulomas. Pediatr Dermatol 30:e91–e93
Jih DM, Salcedo SL, Jaworsky C (2002) Benign cephalic histiocytosis: a case report and review. J Am Acad Dermatol 47:908–913
Jones MB, Voet R, Pagani J, Lotysch M, O’Connell T, Koretz RL (1981) Multifocal eosinophilic granuloma involving the common bile duct: histologic and cholangiographic findings. Gastroenterology 80:384–389
Kanitakis J, Zambruno G, Schmitt D, Cambazard F, Jacquemier D, Thivolet J (1988) Congenital self-healing histiocytosis (Hashimoto-Pritzker). An ultrastructural and immunohistochemical study. Cancer 61:508–516
Kaplan KJ, Goodman ZD, Ishak KG (1999) Liver involvement in Langerhans’ cell histiocytosis: a study of nine cases. Mod Pathol 12:370–378
Kapur P, Erickson C, Rakheja D, Carder KR, Hoang MP (2007) Congenital self-healing reticulohistiocytosis (Hashimoto-Pritzker disease): ten-year experience at Dallas Children’s Medical Center. J Am Acad Dermatol 56:290–294
Karadeniz C, Sarialioglu F, Gogus S, Akyuz C, Kucukali T, Kutluk T, Buyukpamukcu M (1991) Multiple primary malignancy: a report on Langerhans cell histiocytosis associated with Hodgkin’s disease. Turk J Pediatr 33:185–190
Kato T, Matsuda M, Ando H, Sasaki H (1981) A case of multifocal proliferations of histiocytic cells containing Langerhans’ cell granules. Am J Clin Pathol 76:480–485
Kaufman A, Bukberg PR, Werlin S, Young IS (1976) Multifocal eosinophilic granuloma (“Hand-Schüller-Christian disease”). Report illustrating H-S-C chronicity and diagnostic challenge. Am J Med 60:541–548
Kaur MR, Brundler MA, Stevenson O, Moss C (2008) Disseminated clustered juvenile xanthogranuloma: an unusual morphological variant of a common condition. Clin Exp Dermatol 33:575–577
Kawai K, Harada T, Matsukuma H, Nakashita Y (1978) Letterer-Siwe disease with lipid-containing vacuolated cells – an autopsy case. Acta Pathol Jpn 28:787–796
Kawase T, Hamazaki M, Ogura M, Kawase Y, Murayama T, Mori Y, Nagai H, Tateno M et al (2005) CD56/NCAM-positive Langerhans cell sarcoma: a clinicopathologic study of 4 cases. Int J Hematol 81:323–329
Kay TW (1905) Acquired hydrocephalus with atrophic bone changes, exophthalmos and polyuria. Penn Med J 9:520
Kitahama S, Iitaka M, Shimizu T, Serizawa N, Fukasawa N, Miura S, Kawasaki S, Yamanaka K, Kawakami Y et al (1996) Thyroid involvement by malignant histiocytosis of Langerhans’ cell type. Clin Endocrinol (Oxford) 45:357–363
Knobler RM, Neumann RA, Gebhart W, Radaskiewicz T, Ferenci P, Widhalm K (1990) Xanthoma disseminatum with progressive involvement of the central nervous and hepatobiliary systems. J Am Acad Dermatol 23:341–346
Kodet R, Elleder M, De Wolf-Peeters C, Mottl H (1991) Congenital histiocytosis. A heterogeneous group of diseases, one presenting as so-called congenital self-healing histiocytosis. Pathol Res Pract 187:458–466
Kolde G, Bonsmann G (1992) Generalized lichenoid juvenile xanthogranuloma. Br J Dermatol 126:66–70
Komp DM (1987) Historical perspectives of Langerhans cell histiocytosis. Hematol Oncol Clin North Am 1:9–21
Konno S, Hizawa N, Betsuyaku T, Yasuo M, Yamamoto H, Koizumi T, Nishimura M (2007) Adult Langerhans cell histiocytosis with independently relapsing lung and liver lesions that was successfully treated with etoposide. Intern Med 46:1231–1235
Kraus et al (2001) PMID 11285404
Krutchkoff DJ, Jones CR (1984) Multifocal eosinophilic granuloma: a clinical pathologic conference. J Oral Pathol 13:472–488
Ladisch S (1998) Langerhans cell histiocytosis. Curr Opin Hematol 5:54–58
Landing BH (1987) Lymphohistiocytosis in childhood. Pathologic comparison with fatal Letterer-Siwe disease (disseminated visceral histiocytosis X). Perspect Pediatr Pathol 9:48–74
Langerhans p (1868) Ueber die nerven der menschlichen haut. Arch Pathol Anat Physiol Klin Med 44:325–337
Langfort R, Radzikowska E, Czarnowska E, Wiatr E, Grajkowska W, Blasinska-Przerwa K et al (2009) Langerhans cell sarcoma with pulmonary manifestation, mediastinum involvement and bronchoesophageal fistula. A rare location and difficulties in histopathological diagnosis. Pneumol Allergol Pol 77:327–334
Larralde M, Rositto A, Giardelli M, Gatti CF, Santos Munoz A (1999) Congenital self-healing histiocytosis (Hashimoto-Pritzker). Int J Dermatol 38:693–696
Larralde et al (2003) PMID 12653927
Laugier P, Hunziker N, Laut J, Orusco M, Osmos L (1975) Reticulohistiocytosis of benign evolution (Hashimoto-Pritzker type). Electron microscopic study (in French). Ann Dermatol Syphiligr (Paris) 102:21–31
Leblanc A, Hadchouel M, Jehan P, Odievre M, Alagille D (1981) Obstructive jaundice in children with histiocytosis X. Gastroenterology 80:134–139
Lee JS, Ko GH, Kim HC, Jang IS, Jeon KN, Lee JH (2006) Langerhans cell sarcoma arising from Langerhans cell histiocytosis: a case report. J Korean Med Sci 21:577–580
Lee RJ, Leung C, Lim EJ, Angus PW, Bhathal PS, Crowley P, Gonzales M, Stella D et al (2011) Liver transplantation in an adult with sclerosing cholangitis due to multisystem Langerhans cell histiocytosis. Am J Transplant 11:1755–1756
Letterer E (1924) Aleukämische Retikulose (ein Beitrag zu den proliferativen Erkrankungen des Retikuloendothelialapparates). Frankfurt Ztschr Path 30:377
Levy S, Capron D, Joly JP, Cordonnier C, Sevestre H, Delamarre J, Tribout B, Capron JP (1998) Hepatic nodules as single organ involvement in an adult with Langerhans cell granulomatosis. A case report. J Clin Gastroenterol 26:69–73
Li S, Borowitz MJ (2001) CD79a(+) T-cell lymphoblastic lymphoma with coexisting Langerhans cell histiocytosis. Arch Pathol Lab Med 125:958–960
Li H, Wang C, Guo G, Gao C, Wu Y, Chen Y (2012) The characteristic expression of B7-associated proteins in Langerhans cell sarcoma. Acta Histochem 114:733–743
Li J, Cao D, Guo G, Wu Y, Chen Y (2013) Expression and anatomical distribution of TIM-containing molecules in Langerhans cell sarcoma. J Mol Histol 44:213–220
Lichtenstein L (1953) Histiocytosis X; integration of eosinophilic granuloma of bone, Letterer-Siwe disease, and Schüller-Christian disease as related manifestations of a single nosologic entity. AMA Arch Pathol 56:84–102
Lichtenstein L, Jaffe HL (1940) Eosinophilic granuloma of bone. Am J Pathol 16:595–604
Lieberman PH, Jones CR, Dargeon HW, Begg CF (1969) A reappraisal of eosinophilic granuloma of bone. Hand-Schüller-Christian syndrome and Letterer-Siwe syndrome. Medicine (Baltimore) 48:375–400
Lieberman PH, Jones CR, Steinman RM, Erlandson RA, Smith J, Gee T, Huvos A, Garin-Chesa P et al (1996) Langerhans cell (eosinophilic) granulomatosis. A clinicopathologic study encompassing 50 years. Am J Surg Pathol 20:519–552
Liu DG, Zhang YX, Li F (2012) Multisystem Langerhans cell histiocytosis with liver dysfunction as the first presentation: a case report. Oncol Lett 3:391–394
Llamas-Velasco M, Cannata J, Dominguez I, Garcia-Noblejas A, Aragues M, Fraga J et al (2012) Coexistence of Langerhans cell histiocytosis, Rosai-Dorfman disease and splenic lymphoma with fatal outcome after rapid development of histiocytic sarcoma of the liver. J Cutan Pathol 39:1125–1130
Longakter M, Frieden I, Leboit P, Sherertz E (1994) Congenital “self-healing” Langerhans cell histiocytosis: the need for long term follow-up. J Am Acad Dermatol 31:910–916
Ma XL, Shen KL, Wang B (2012) A child with pulmonary and liver Langerhans’-cell histiocytosis. Chin Med J (Engl) 125:1675–1676
Ma J, Jiang Y, Chen X, Gong G (2014) Langerhans cell histiocytosis misdiagnosed as liver cancer and pituitary tumor in an adult: a case report and brief review of the literature. Oncol Lett 7:1602–1604
Maly E, Przyborska M, Rybczynska A, Konatkowska B, Nowak J, Januszkiewicz D (2012) Juvenile xanthogranuloma with clonal proliferation in the bone marrow. J Pediatr Hematol Oncol 34:222–225
Mandel VD, Ferrari C, Cesinaro AM, Pellacani G, Del Forno C (2014) Congenital “self-healing” Langerhans cell histiocytosis (Hashimoto-Pritzker disease): a report of two cases with same cutaneous manifestations but different clinical course. J Dermatol. doi:10.1111/1346-8138
Mascaro JM, Aliaga A, Mascaro-Galy C (1978) Auto-involutive congenital reticulosis (Hashimoto-Pritzker type) (in French). Ann Dermatol Venereol 105:223–227
Masouye I, Chavaz P, Salomon D, Balderer J, Carraux P, Saurat JH (1990) Solitary Langerhans cell histiocytoma: an unusual form of Hashimoto-Pritzker histiocytosis? Pediatr Dermatol 7:299–302
McClain KL, Laud P, Wu WS, Pollack MS (2003) Langerhans cell histiocytosis patients have HLA Cw7 and DR4 types associated with specific clinical presentations and no increased frequency in polymorphisms of the tumor necrosis factor alpha promoter. Med Pediatr Oncol 41:502–507
McDonagh JER (1912) A contribution to our knowledge of the naevo-xantho-endotheliomata. Br J Dermatol 24:85–99
Mieli-Vergani G, Vergani D (2001) Sclerosing cholangitis in the paediatric patient. Best Pract Res Clin Gastroenterol 15:681–690
Minkow M (2011) Multisystem Langerhans cell histiocytosis in children: current treatment and future directions. Paediatr Drugs 13:75–86
Minkow M, Steiner M, Pötschger U, Arico M, Braier J, Donadieu J, Grois N, Henter JI et al (2008) Reactivation in multisystem Langerhans cell histiocytosis: data of the international LCH registry. J Pediatr 153:700–705
Misery L, Godard W, Hamzeh H, Levigne V, Vinvent C, Perrot JL, Gentil-Perret A, Schmitt D, Cambazard F (2003) Malignant Langerhans cell tumor: a case with favorable outcome associated with the absence of blood dendritic cell proliferation. J Am Acad Dermatol 49:527–529
Montgomery H, Osterberg A (1938) Xanthomatous correlation of clinical, histopathological, and chemical studies of cutaneous xanthoma. Arch Dermatol Syphilol 37:373–402
Morgan KW, Callen JP (2001) Self-healing congenital Langerhans cell histiocytosis presenting as neonatal papulovesicular eruption. J Cutan Med Surg 5:486–489
Mosterd K, van Marion A, van Steensel MA (2008) Neonatal Langerhans’ cell histiocytosis: a rare and potentially life-threatening disease. Int J Dermatol 47(Suppl 1):10–12
Muirhead D, Stone MS, Syrbu SI (2009) The utility of PU.1 as an immunohistochemical marker for histiocytic and dendritic lesions of the skin. Am J Dermatopathol 31:432–435
Munir A, Leech N, Windebank KP, McLelland J, Jones GL, Mitra D, Jenkins A, Qionton R (2012) Langerhans cell histiocytosis: a multisystem disorder. JR Coll Physicians Edinb 42:311–313
Muñoz-Garcia JC, Chabrol E, Vivès RR, Thomas A, de Paz JL, Rojo J, Imberty A, Fieschi F, Nieto PM, Angulo J (2015) Langerin-heparin interaction: two binding sites for small and large ligands as revealed by a combination of NMR spectroscopy and cross-linking mapping experiments. J Am Chem Soc 137:4100–4110
Murakami I, Gogusev J, Fournet JC, Glorion C, Jaubert F (2002) Detection of molecular cytogenetic aberrations in Langerhans cell histiocytosis of bone. Hum Pathol 33:555–560
Muslimani A, Chisti MM, Blenc AM, Boxwala I, Micale MA, Jaiyesimi I (2012) Langerhans/dendritic cell sarcoma arising from hairy cell leukemia: a rare phenomenon. Ann Hematol 91:1485–1487
Nakahigashi K, Ohta M, Sakai R, Sugimoto Y, Ikoma Y, Horiguchi Y (2007) Late-onset self-healing reticulohistiocytosis: pediatric case of Hashimoto-Pritzker type Langerhans cell histiocytosis. J Dermatol 34:205–209
Nakatani T, Morimoto A, Kato R, Tokuda S, Sugimoto T, Tokiwa K, Tsuchihashi Y, Imashuku S (2004) Successful treatment of congenital systemic juvenile xanthogranuloma with Langerhans cell histiocytosis-based chemotherapy. J Pediatr Hematol Oncol 26:371–374
Narvaez-Moreno B, Pulpillo-Ruiz A, De Zulueta-Dorado T, Conjero-Mir J (2013) Disseminated juvenile xanthogranuloma associated with follicular lymphoma in a adult: successful treatment with chemotherapy and rituximab. A review of the literature. Actas Dermosifiliogr 104:242–246
Nezelof C, Basset F, Rousseau MF (1973) Histiocytosis X histogenetic arguments for a Langerhans cell origin. Biomedicine 18:365–371
Ngendahayo P, de Sant Aubain N (2012) Mitotically active xanthogranuloma: a case report with review of the literature. Am J Dermatopathol 34:e27–e30
Nguyen TT, Schwartz EJ, West RB, Warnke RA, Arber DA, Natkunam Y (2005) Expression of CD163 (hemoglobin scavenger receptor) in normal tissues, lymphomas, carcinomas, and sarcomas is largely restricted to the monocyte/macrophage lineage. Am J Surg Pathol 29:617–624
Oranje AP, Vuzevski VD, de Groot R, Prins ME (1988) Congenital self-healing non-Langerhans cell histiocytosis. Eur J Pediatr 148:29–31
Orii T, Takeda H, Kawata S, Maeda K, Yamakawa M (2010) Differential immunophenotypic analysis of dendritic cell tumours. J Clin Pathol 63:497–503
Otani S, Ehrlich JC (1940) Solitary granuloma of bone simulating primary neoplasm. Am J Pathol 16:479–490
Papadakis V, Volonaki E, Katsibardi K, Stefanaki K, Valari M, Anagnostakou M, Polychronopoulou S (2012) A rare case of neonatal systemic xanthogranulomatosis with severe hepatic disease and metachronous skin involvement. J Pediatr Hematol Oncol 34:226–228
Papasozomenos SC (1999) Hypothalamic Langerhans cell histiocytosis with no eosinophils. Clin Neuropathol 18:260–264
Parentin F, Ventura G, Pastore S, Kiren V, Bibalo C, Pensiero S, Lepore L (2011) A singular case of congenital self-healing histiocytosis with skin, liver and atypical eye involvement. Ocul Immunol Inflamm 19:337–339
Park IS, Park IK, Kim EK, Kim S, Jon SR, Huh JR, Suh CW (2012) Langerhans cell histiocytosis followed by Hodgkin’s lymphoma. Korean J Intern Med 27:459–462
Parker JW, Lichtenstein L (1963) Severe hepatic involvement in chronic disseminated histiocytosis X. Report of a case with necropsy. Am J Clin Pathol 40:624–632
Patel P, Vyas R, Blickman J, Katzman P (2010) Multi-modality imaging findings of disseminated juvenile xanthogranuloma with renal involvement in an infant. Pediatr Radiol 40(Suppl 1):S6–S10
Peng CT, Chow KC, Chang WC, Tsai CH, Lin TY, Lin SS, Chiu CF (1999) Expression of Fas ligand in Langerhans’ cell histiocytosis: a case report of a boy with multisystem involvement. Am J Hematol 61:256–261
Petersen BL, Rengtved P, Bank MI, Carstensen H (2003) High expression of markers of apoptosis in Langerhans cell histiocytosis. Histopathology 42:186–193
Pileri SA, Grogan TM, Harris NL, Banks P, Campo E, Chan JK, Favera RD, Delsol G, De Wolf-Peeters C, Falini B, Gascoyne RD et al (2002) Tumours of histiocytes and accessory dendritic cells: an immunohistochemical approach to classification from the International Lymphoma Study Group based on 61 cases. Histopathology 41:1–29
Pineles SL, Liu GT, Acebes X, Arruga J, Nasta S, Glaser R, Pramick M, Fogt F, Roux PL et al (2011) Presence of Erdheim-Chester disease and Langerhans cell histiocytosis in the same patient: a report of 2 cases. J Neuroophthalmol 31:217–223
Pirovino et al (1988) PMID 3264377
Pollono D, Rey G, Latella A, Rosso D, Chantada G, Braier J (2007) Reactivation and risk of sequelae in Langerhans cell histiocytosis. Pediatr Blood Cancer 48:696–699
Prasil P, Cayer S, Lemay M, Pelletier L, Cloutier R, Leclerc S (1999) Juvenile xanthogranuloma presenting as obstructive jaundice. J Pediatr Surg 34:1072–1073
Pujol RM, Moreno A, Lopez D, Alomar A, de Moragas JM (1988) Childhood self-healing histiocytosis X. Pediatr Dermatol 5:97–102
Ratei R, Hummel M, Anagnostopoulos I, Jähne D, Arnold R, Dörken B, Mathas S, Benter T et al (2010) Common clonal origin of an acute B-lymphoblastic leukemia and a Langerhans’ cell sarcoma: evidence for hematopoietic plasticity. Haematologica 95:1461–1466
Raygada M, Arthur DC, Wayne AS, Rennert OM, Toretsky JA, Stratakis CA (2010) Juvenile xanthogranuloma in a child with previously unsuspected neurofibromatosis type 1 and juvenile myelomonocytic leukemia. Pediatr Blood Cancer 54:173–175
Ricciardo B, Irvine A, McDermott M, Ryan J, Collins S (2011) A case of congenital solitary Langerhans cell histiocytoma. Australas J Dermatol 52:e1–e3
Rice AJ, Wyatt JI (2000) Solitary Langerhans cell histiocytosis in association with primary biliary cirrhosis. Histopathology 36:569–571
Ringel E, Moschella S (1962) Primary histiocytic dermatoses. Arch Dermatol 86:582–596
Rodig SJ, Payne EG, Degar BA, Rollins B, Feldman AL, Jaffe ES, Androkites A et al (2008) Aggressive Langerhans cell histiocytosis following T-ALL: clonally related neoplasms with persistent expression of constitutively active NOTCH1. Am J Hematol 83:116–121
Romao Z, Pontes J, Andrade P, Leitao MC, Donato A, Freitas D (2002) Secondary biliary cirrhosis in a patient with histiocytosis X (in Portuguese). Acta Med Port 15:55–59
Ruco LP, Remotti D, Monardo F, Uccini S, Cristiani ML, Modesti A, Baroni CD (1988) Letterer-Siwe disease: immunohistochemical evidence for a proliferative disorder involving immature cells of Langerhans lineage. Virchows Arch A Pathol Anat Histopathol 413:239–247
Sabib M, Ettair S, Erreimi N, Mouane N (2011) Sclerosing cholangitis revealing Langerhans cell histiocytosis in a 15-mont-old child (in French). Arch Pediatr 18:974–978
Sahm F, Capper D, Preusser M, Meyer J, Stenzinger A, Lasitschka F, Berghoff AS et al (2012) BRAFV600E mutant protein is expressed in cells of variable maturation in Langerhans cell histiocytosis. Blood 120:e28–e34
Saito F, Okabe Y, Suga H, Watanabe T, Arinaga T, Naito Y, Uchida S, Hisaka T, Toyonaga A et al (2008) A case of hepatic eosinophilic granuloma, which needs distinction with metastatic liver cancer (in Japanese). Nippon Shokakibyo Gakkai Zasshi 105:1509–1514
Sankilampi U, Huikko-Tarvainen S, Kärjä V, Pirinen E, Naukharinen A, Hollmén A (2008) Congenital Langerhans cell histiocytosis mimicking a “blueberry muffin baby”. J Pediatr Hematol Oncol 30:245–248
Satoh T, Smith A, Sarde A, Lu HC, Mian S, Trouillet C, Mufti G, Emile JF, Fraternali F et al (2012) B-RAF mutant alleles associated with Langerhans cell histiocytosis, a granulomatous pediatric disease. PLoS One 7:e33891
Savva-Bordalo J, Freitas-Silva M (2008) Langerhans cell histiocytosis involving the liver of a male smoker: a case report. J Med Case Reports 2:376
Schouten B, Egeler RM, Leenen PJ, Taminiau AH, van den Broek LJ, Hogendoorn PC (2002) Expression of cell cycle-related gene products in Langerhans cell histiocytosis. J Pediatr Hematol Oncol 24:727–732
Schüller A (1915) Ueber eigenartige Schädeldefekte im Jugendalter (“Landkartenschädel”). Fortschr Geb Röntgenstrahlen 23:12–18
Schüller A (1926) Dysostosis hypophysaria. Br J Radiol 31:156–158
Seward JL, Malone JC, Callen JP (2004) Generalized eruptive histiocytosis. J Am Acad Dermatol 50:116–120
Shao H, Xi L, Raffeld M, Feldman AL, Ketterling RP, Knudson R, Rodriguez-Canales J et al (2011) Clonally related histiocytic/dendritic cell sarcoma and chronic lymphocytic leukemia/small lymphocytic lymphoma: a study of seven cases. Mod Pathol 24:1421–1432
Shin HT, Harris MB, Orlow SJ (2004) Juvenile myelomonocytic leukemia presenting with features of hemophagocytic lymphohistiocytosis in association with neurofibromatosis and juvenile xanthogranulomas. J Pediatr Hematol Oncol 26:591–595
Shoo BA, Shinkai K, McCalmont TH, Fox LP (2008) Xanthogranulomas associated with hematologic malignancy in adulthood. J Am Acad Dermatol 59:488–493
Shuangshoti S, Seksarn P (1987) Histiocytosis X: overlapping form between Letterer-Siwe disease and Hand-Schüller-Christian disease with advanced thymic involvement and obstructive jaundice. J Med Assoc Thai 70:344–350
Shy SW, Lee WH, Chang YC, Wu W (1996) A solitary congenital self-healing histiocytosis. Report of a case and review of the literature. Pathol Res Pract 192:869–874
Sidwell RU, Francis N, Slater DN, Mayou SC (2005) Is disseminated juvenile xanthogranulomatosis benign cephalic histiocytosis? Pediatr Dermatol 22:40–43
Sisto A, Feldman P, Garel L, Seidman E, Brochu P, Morin CL et al (1987) Primary sclerosing cholangitis in children: study of five cases and review of the literature. Pediatrics 80:918–923
Sivapirabu G, Sugo E, Wargon O (2011) Juvenile xanthogranuloma: challenges in complicated cases. Australas J Dermatol 52:284–287
Siwe S (1933) Die Reticuloendotheliose – ein neues Krankheitsbild unter den Hepatosplenomegalien. Z Kinderheilk 55:212–247
Smith T (1864) Skull-cap showing congenital deficiencies of bone. Trans Path Soc Lond 16:224
Squires RH, Weinberg AG, Zwiener RJ, Winick N (1993) Langerhans’ cell histiocytosis presenting with hepatic dysfunction. J Pediatr Gastroenterol Nutr 16:190–193
Stables GI, Mackie RM (1992) Generalized eruptive histiocytoma. Br J Dermatol 126:196–199
Stieber AC, Sever C, Starzl TE (1990) Liver transplantation in patients with Langerhans’ cell histiocytosis. Transplantation 50:330–340
Sumida K, Yoshidomi Y, Koga H, Kuwahara N, Matsuishi E, Karube K, Oshima K, Gondo H (2008) Leukemic transformation of Langerhans cell sarcoma. Int J Hematol 87:527–531
Sundar KM, Gosselin MV, Chung HL, Cahill BC (2003) Pulmonary Langerhans cell histiocytosis: emerging concepts in pathobiology, radiology, and clinical evolution of disease. Chest 123:1673–1683
Tahan SR, Pastel-Levy C, Bhan AK, Mihm MC (1989) Juvenile xanthogranuloma. Clinical and pathologic characterization. Arch Pathol Lab Med 113:1057–1061
Tani M, Ishii N, Kumagai M, Ban M, Sasase A, Mishima Y (1992) Malignant Langerhans cell tumour. Br J Dermatol 126:398–403
Tardio JC, Aguado M, Borbujo J (2013) Self-regressing S100-negative CD1a-positive cutaneous histiocytosis. Am J Dermatopathol 35:e57–e59
Tay YK, Friednash MM, Weston WL, Aeling JL (1998) Solitary congenital nodule in an infant. Solitary congenital self-healing reticulohistiocytosis. Arch Dermatol 134:627
Terry J, Luchinotta F, Sanders S, Vergilio JA, Hyde S, Boyd TK (2013) Congenital Langerhans cell histiocytosis with placental involvement. Pediatr Dev Pathol 16:224–228
Thompson HH, Pitt HA, Lewin KJ, Longmire WP (1984) Sclerosing cholangitis and histiocytosis X. Gut 25:526–530
Touton K (1885) Über das Xanthom, insbesondere dessen Histiologie und Histiogenese. Vierteljahrsschr Dermatol Syphil 12:3–53
Tran DT, Wolgamot GM, Olerud J, Hurst S, Argenyi Z (2008) An ‘eruptive’ variant of juvenile xanthogranuloma associated with Langerhans cell histiocytosis. J Cutan Pathol 35(Suppl 1):50–54
Trochtenberg DS, Dessypris EN (1990) Reversible hepatomegaly and diabetes mellitus in an adult with disseminated histiocytosis X. Am J Med Sci 299:179–184
Unuvar E, Devecioglu O, Akcay A, Gulluoglu M, Uysal V, Oguz F, Sidal M (2007) Successful therapy of systemic xanthogranuloma in a child. J Pediatr Hematol Oncol 29:425–427
Vade A, Hayani A, Pierce KL (1993) Congenital histiocytosis X. Pediatr Radiol 23:181–182
Vollum DI (1979) Letterer-Siwe disease in the adult. Clin Exp Dermatol 4:395–406
Walia M, Paul P, Mishra S, Mehta R (2004) Congenital Langerhans cell histiocytosis: the self-healing variety. J Pediatr Hematol Oncol 26:398–402
Warnke RA, Weiss LM, Chan JKC, Cleary ML, Dorfman RF (1995) Tumors of the lymph nodes and spleen. Atlas of tumor pathology, 3rd series, fascicle 14. Armed Forces Institute of Pathology, Washington, DC
Wee SH, Kim HS, Chang SN, Kim DK, Park WH (2000) Generalized eruptive histiocytoma: a pediatric case. Pediatr Dermatol 17:453–455
Weidman FD, Freeman W (1924) Xanthoma tuberosum: two necropsies disclosing lesions of central nervous system and other tissues. Arch Dermatol Syphilol 9:149
Weiss N, Keller C (1993) Xanthoma disseminatum: a rare normolipemic xanthomatosis. Clin Investig 71:233–238
Wells GC (1979) The pathology of adult type Letterer-Siwe disease. Clin Exp Dermatol 4:407–412
Wheller L, Carman N, Butler G (2013) Unilesional self-limited Langerhans cell histiocytosis: a case report and review of the literature. J Cutan Pathol 40:595–599
Willman CL (1994) Detection of clonal histiocytes in Langerhans cell histiocytosis: biology and clinical significance. Br J Cancer Suppl 23:S29–S33
Willman CL, Busque L, Griffith BB et al (1994) Langerhans cell histiocytosis (histiocytosis X): a clonal proliferative disease. N Engl J Med 331:154–160
Windebank KP, Nanduri V (2009) Langerhans cell histiocytosis. Arch Dis Child 94:904–908
Winkelmann RK, Müller SA (1963) Generalized eruptive histiocytoma: a benign papular histiocytic reticulosis. Arch Dermatol 88:154–164
Wood C, Wood GS, Deneau DG, Oseroff A, Beckstead JH, Malin J (1984) Malignant histiocytosis X. Report of a rapidly fatal case in an elderly man. Cancer 54:347–352
Woollons A, Darley CR (1998) Xanthoma disseminatum: a case with hepatic involvement, diabetes insipidus and type IIb hyperlipidaemia. Clin Exp Dermatol 23:277–280
Writing Group of the Histiocyte Society (1987) Histiocytosis syndromes in children. Lancet 1:208–209
Wu WS, McClain KL (1997) DNA polymorphisms and mutations of the tumor necrosis factor-alpha (TNF-alpha) promoter in Langerhans cell histiocytosis (LCH). J Interferon Cytokine Res 17:631–635
Xu X, Liu WP, Yang QP, Wang WY, Liao DY, Zhao S, Bi CF, Lin L, Min M (2012a) Langerhans cell histiocytosis: a clinicopathologic and immunohistochemical analysis of 258 cases (in Chinese). Zhonghua Bing Li Xue Za Zhi 41:91–96
Xu Z, Padmore R, Faught C, Duffet L, Burns BF (2012b) Langerhans cell sarcoma with an aberrant cytoplasmic CD3 expression. Diagn Pathol 7:128
Yagita K, Iwai M, Yagita-Toguri M, Kimura H, Taniwaki M, Misawa S, Okanoue T, Kashima K, Tsuchihashi Y (2001) Langerhans cell histiocytosis of an adult with tumors in liver and spleen. Hepatogastroenterology 48:581–584
Yeh BM, Nobrega KT, Reddy GP, Qayyum A (2007) Juvenile xanthogranuloma of the heart and liver: MRI, sonographic, and CT appearance. AJR Am J Roentgenol 189:W202–W204
Yousem SA, Colby TV, Chen YY, Chen WG, Weiss LM (2001) Pulmonary Langerhans’ cell histiocytosis: molecular analysis of clonality. Am J Surg Pathol 25:630–636
Yu CP, Tseng HH, Tu YC (1990) Congenital Letterer-Siwe disease with intrauterine fetal death: a case report and review of the literature. J Formos Med Assoc 89:806–810
Yu RC, Chu C, Buluwela L, Chu AC (1994) Clonal proliferation of Langerhans cells in Langerhans cell histiocytosis. Lancet 343:767–768
Yurkovich M, Wong A, Lam JM (2013) Solitary congenital self-healing Langerhans cell histiocytosis: a benign variant of Langerhans cell histiocytosis? Dermatol Online J 19:3
Zaenglein AL, Stelle MA, Kamino H, Chang MW (2001) Congenital self-healing reticulohistiocytosis with eye involvement. Pediatr Dermatol 18:135–137
Zelger B, Cerio R, Orchard G, Fritsch P, Wilson-Jones E (1992) Histologic and immunohistochemical study comparing xanthoma disseminatum and histiocytosis X. Arch Dermatol 128:1207–1212
Zelger BW, Sidoroff A, Orchard G, Cerio R (1996) Non-Langerhans cell histiocytoses. A new unifying concept. Am J Dermatopathol 18:490–504
Zhao G, Luo M, Wu ZY, Liu Q, Zhang B, Gao RL, Zhang ZQ (2009) Langerhans cell sarcoma involving gallbladder and peritoneal lymph nodes: a case report. Int J Surg Pathol 17:347–353
Zyada MM (2009) Expression of matrix metalloproteinase-9 and significance of a macrophage assay in eosinophilic granuloma. Ann Diagn Pathol 13:367–372
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Zimmermann, A. (2017). Tumors of the Langerhans Cell System. In: Tumors and Tumor-Like Lesions of the Hepatobiliary Tract. Springer, Cham. https://doi.org/10.1007/978-3-319-26956-6_100
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Publisher Name: Springer, Cham
Print ISBN: 978-3-319-26954-2
Online ISBN: 978-3-319-26956-6
eBook Packages: MedicineReference Module Medicine