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Hand-Schuller-Christian disease

MedGen UID:
1804593
•Concept ID:
C5574737
•
Disease or Syndrome
Synonym: Hand-Schuller-Christian syndrome
SNOMED CT: Hand-Schüller-Christian disease (39795003); Langerhans cell histiocytosis, multifocal (128811003); Schüller-Christian syndrome (39795003); Multifocal and unisystemic Langerhans cell histiocytosis (39795003); Schuller-Christian syndrome (39795003); Hand-Schuller-Christian disease (39795003); Chronic differentiated progressive histiocytosis (39795003); Chronic disseminated histiocytosis X (39795003); Chronic histiocytosis X (39795003); Generalized histiocytosis of bones (39795003); Chronic idiopathic xanthomatosis (39795003)
 
Monarch Initiative: MONDO:0020519

Definition

A multifocal, unisystem form of Langerhans-cell histiocytosis. There is involvement of multiple sites in one organ system, most frequently the bone. Patients are usually young children presenting with multiple destructive bone lesions. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

Professional guidelines

PubMed

Diagnosis and management of langerhans cell histiocytosis.
DiCaprio MR, Roberts TT
J Am Acad Orthop Surg 2014 Oct;22(10):643-52. doi: 10.5435/JAAOS-22-10-643. PMID: 25281259
Experience of prolonged treatment of hand-Schüller-Christian disease with prednisolone.
Laczi F, Czako L, László FA
Endokrinologie 1977;70(3):331-9. PMID: 608461
Hormonal treatment of Hand-Schüller-Christian disease: report on a case with disappearance of the bone lesions.
FLOSI AZ, ASSIS LM, COELHO NETO AS, BLOISE W, CINTRA AB, BARROS RP
J Clin Endocrinol Metab 1959 Feb;19(2):239-47. doi: 10.1210/jcem-19-2-239. PMID: 13620751

Recent clinical studies

Etiology

Diagnosis and management of langerhans cell histiocytosis.
DiCaprio MR, Roberts TT
J Am Acad Orthop Surg 2014 Oct;22(10):643-52. doi: 10.5435/JAAOS-22-10-643. PMID: 25281259
Hand-Schüller-Christian disease and Erdheim-Chester disease: coexistence and discrepancy.
Yin J, Zhang F, Zhang H, Shen L, Li Q, Hu S, Tian Q, Bao Y, Jia W
Oncologist 2013;18(1):19-24. Epub 2013 Jan 8 doi: 10.1634/theoncologist.2012-0234. PMID: 23299772Free PMC Article
Hand-Schüller-Christian disease.
Bhargava D, Bhargava K, Hazarey V, Ganvir SM
Indian J Dent Res 2012 Nov-Dec;23(6):830-2. doi: 10.4103/0970-9290.111273. PMID: 23649074
Multisystem Langerhans' cell histiocytosis (Hand-Schüller-Christian disease) in an adult: a case report and review of the literature.
Scolozzi P, Lombardi T, Monnier P, Jaques B
Eur Arch Otorhinolaryngol 2004 Jul;261(6):326-30. Epub 2003 Oct 10 doi: 10.1007/s00405-003-0690-z. PMID: 14551790
Idiopathic differentiated histiocytosis.
Daneshbod K, Kissane JM
Am J Clin Pathol 1978 Sep;70(3):381-9. doi: 10.1093/ajcp/70.3.381. PMID: 309279

Diagnosis

Langerhans cell histiocytosis in the oral and maxillofacial region: An update.
Faustino ISP, Fernandes PM, Pontes HAR, Mosqueda-Taylor A, Santos-Silva AR, Vargas PA, Lopes MA
J Oral Pathol Med 2021 Jul;50(6):565-571. Epub 2021 Jun 20 doi: 10.1111/jop.13207. PMID: 34091963
Diagnosis and management of langerhans cell histiocytosis.
DiCaprio MR, Roberts TT
J Am Acad Orthop Surg 2014 Oct;22(10):643-52. doi: 10.5435/JAAOS-22-10-643. PMID: 25281259
Hand-Schüller-Christian disease.
Bhargava D, Bhargava K, Hazarey V, Ganvir SM
Indian J Dent Res 2012 Nov-Dec;23(6):830-2. doi: 10.4103/0970-9290.111273. PMID: 23649074
Histiocytosis X.
Favara BE, McCarthy RC, Mierau GW
Hum Pathol 1983 Aug;14(8):663-76. doi: 10.1016/s0046-8177(83)80138-5. PMID: 6347865
Hand-Schuller-Christian disease.
Praharaj KC, Mohanta KD, Nanda CN, Naik UP
Indian Pediatr 1968 Oct;5(10):456-64. PMID: 5715221

Therapy

A rare condition of Hand-Schüller-Christian disease.
Becelli R, Carboni A, Gianni C, Alterio A, Renzi G
J Craniofac Surg 2002 Nov;13(6):759-61. doi: 10.1097/00001665-200211000-00008. PMID: 12457089
Hand-Schüller-Christian disease with occult diabetes insipidus, cardiac failure and renal dysfunction.
Kimura T, Ota K, Shoji M, Inoue M, Sato K, Ohta M, Yamamoto T, Endo K, Osaki H, Aoyama H
Jpn J Med 1990 Jul-Aug;29(4):405-10. doi: 10.2169/internalmedicine1962.29.405. PMID: 2148780
Hand-Schüller-Christian disease in a septuagenarian.
Dolezal JF, Thomson ST
Arch Dermatol 1978 Jan;114(1):85-7. PMID: 619789
Hand-Schüller-Christian disease.
Hayduk JW
Oral Surg Oral Med Oral Pathol 1967 Jan;23(1):29-35. doi: 10.1016/0030-4220(67)90480-x. PMID: 5224921
Hand-Schüller-Christian disease treated with cortisone.
SEWELL RH
Proc R Soc Med 1954 Jan;47(1):10-1. PMID: 13134156Free PMC Article

Prognosis

Diagnosis and management of langerhans cell histiocytosis.
DiCaprio MR, Roberts TT
J Am Acad Orthop Surg 2014 Oct;22(10):643-52. doi: 10.5435/JAAOS-22-10-643. PMID: 25281259
Hand-Schüller-Christian disease and Erdheim-Chester disease: coexistence and discrepancy.
Yin J, Zhang F, Zhang H, Shen L, Li Q, Hu S, Tian Q, Bao Y, Jia W
Oncologist 2013;18(1):19-24. Epub 2013 Jan 8 doi: 10.1634/theoncologist.2012-0234. PMID: 23299772Free PMC Article
Langerhans cell histiocytosis: literature review and descriptive analysis of oral manifestations.
Madrigal-Martínez-Pereda C, Guerrero-Rodríguez V, Guisado-Moya B, Meniz-García C
Med Oral Patol Oral Cir Bucal 2009 May 1;14(5):E222-8. PMID: 19218906
Histiocytosis X.
Favara BE, McCarthy RC, Mierau GW
Hum Pathol 1983 Aug;14(8):663-76. doi: 10.1016/s0046-8177(83)80138-5. PMID: 6347865
Idiopathic differentiated histiocytosis.
Daneshbod K, Kissane JM
Am J Clin Pathol 1978 Sep;70(3):381-9. doi: 10.1093/ajcp/70.3.381. PMID: 309279

Clinical prediction guides

Multidisciplinary approach in a case of Hand-Schüller-Christian disease with maxillary involvement.
Cazzolla AP, Testa NF, Favia G, Lacaita MG, Ciavarella D, Zhurakivska K, Troiano G, Lo Muzio L
Spec Care Dentist 2018 Mar;38(2):107-111. Epub 2018 Feb 8 doi: 10.1111/scd.12273. PMID: 29418009
Hand-Schüller-Christian disease and Erdheim-Chester disease: coexistence and discrepancy.
Yin J, Zhang F, Zhang H, Shen L, Li Q, Hu S, Tian Q, Bao Y, Jia W
Oncologist 2013;18(1):19-24. Epub 2013 Jan 8 doi: 10.1634/theoncologist.2012-0234. PMID: 23299772Free PMC Article
Eosinophilic granuloma of the bone in Hand-Schüller-Christian disease: extensive in vivo eosinophil degranulation and subsequent binding of released eosinophil peroxidase (EPO) to other inflammatory cells.
Zabucchi G, Soranzo MR, Menegazzi R, Cattin L, Vecchio M, Lanza F, Patriarca P
J Pathol 1991 Mar;163(3):225-31. doi: 10.1002/path.1711630307. PMID: 1707451
Growth hormone responses to growth hormone-releasing hormone in Hand-Schüller-Christian Disease.
Gelato MC, Loriaux DL, Merriam GR
Neuroendocrinology 1989 Sep;50(3):259-64. doi: 10.1159/000125231. PMID: 2507952
Pathology of orbital bones. The XXXII Edward Jackson Memorial Lecture.
Blodi FC
Am J Ophthalmol 1976 Jan;81(1):1-26. doi: 10.1016/0002-9394(76)90186-0. PMID: 814817

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