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Hand-Schuller-Christian Syndrome
Post your experienceHand-Schuller-Christian Syndrome (HSC) is one variant of Langerhan's cell histiocytosis (LCH) which was previously known as Histiocytosis X.1
Langerhan's cell histiocytosis covers a spectrum of disorders that have been separately classified in the past as:
- Eosinophilic granuloma (localised bony lesions, single or multiple)
- Hand-Schuller-Christian syndrome itself (multiple bony lesions affecting the skull, associated with proptosis and hypothalamic infiltration) and
- Letterer-Siwe disease (aggressive systemic variant with involvement of soft tissue only).2
These categories are not exclusive and overlap with progression between types.
All are diseases of abnormal Langerhan's cell proliferation, probably due to defective immunoregulation. Although no longer believed to be a truly malignant condition (most cases appear to be self-limiting), LCH does sometimes behave aggressively and its response to chemotherapy places it within oncologists' practice.
LCH is characterised by :
- Presence of clonal proliferation of Langerhans cell (monocytes lineage)
- Contain Birbeck's granule (tennis racket- shaped)
- Express surface antigens S100 and CD11 and 14 surface monoclonal antibodies
- Variable numbers of eosinophils, lymphocytes and giant cells.
With Hand-Schuller-Christian syndrome, there is a recognised triad of bone lesions, diabetes insipidus and exophthalmos. However, this full triad is only seen in about 12%.
Possible clinical features (listed by systems) and complications are:
- General features:
- Fever
- Weight loss or failure to thrive
- Raised ESR
- Bone lesions:
- Cranial lesions are very common in HSC, and may cause:
- Otitis media
- Diabetes insipidus (due to lesions affecting the pituitary or its stalk)
- Exophthalmos
- Dental lesions, e.g. loose teeth and bleeding gums
- Cranial lesions are very common in HSC, and may cause:
- Haematological:
- Skin:5,9
- Pink crusted papules, nodules, mouth or genital ulceration, seborrheic rashes
- Neurological:
- Skull lesions may impinge on CNS; bony lesions can affect spinal cord
- Transverse myelitis
- Other organ involvement:
- Lung
- Liver
- Renal
- GI tract
Tissue biopsy, FBC, renal and liver function tests, coagulation studies, bone marrow aspirates, skeletal survey, chest X-ray and urine osmolality, hearing tests. CT or MRI may be useful, e.g. for cranial involvement.
Current recommendations are that patients be risk-stratified according to the number of organs involved, the degree of organ dysfunction and which organ system is involved.10
- Mild symptoms may not require treatment.
- Where a single system affected e.g. bone, lymphatics or skin use minimum intervention to stop progression of the lesion, e.g. curettage, excision, or intralesional steroid injection for skin or bone lesions.
- Possible treatment of skin lesions includes:5
- Local treatments, e.g. topical steroids, antiseptics or antibiotics; curettage or intralesional injection of steroids
- Phototherapy (PUVA)
- Topical nitrogen mustard (mechlorethamine)
- Thalidomide
- Where more than one system involved, steroids and/or chemotherapy:
- For children, oral corticosteroids may be the first option5
- Other chemotherapy agents are: methotrexate, vinblastine, etoposide or mercaptopurine.
- Management of progressive disease not responding to above: Possible use of allogenic marrow transplantation or stem cell transplantation (if match), or antithymocytic globulin, prednisolone and ciclosporin (where no donor).
Some cases resolve or regress spontaneously.12 Neonates with isolated cutaneous lesions have an excellent prognosis.10Prognosis varies the number of organ systems involved.8,13
For example:
- If age > 2 years of age when diagnosed, with no involvement of the blood, liver, lungs or spleen, patients often do well and/or are likely to respond to a single chemotherapy drug.5
- Younger children with involvement of blood, liver, lungs or spleen may require more complicated chemotherapy regimens with multiple drugs.5
- Initial response to chemotherapy is also a prognostic indicator.10
- Some patients with multiple organ involvement may not respond to treatment.
- About 20% of patients with multisystem involvement may have a progressive form of the disease.13
Document references
- Favara BE, Feller AC, Pauli M, et al; 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. [abstract]
- Nezelof C, Basset F; Langerhans cell histiocytosis research. Past, present, and future. Hematol Oncol Clin North Am. 1998 Apr;12(2):385-406. [abstract]
- Kimura T, Ota K, Shoji M, et al; Hand-Schuller-Christian disease with occult diabetes insipidus, cardiac failure and renal dysfunction.; Jpn J Med. 1990 Jul-Aug;29(4):405-10. [abstract]
- Scolozzi P, Lombardi T, Monnier P, et al; Multisystem Langerhans' cell histiocytosis (Hand-Schuller-Christian disease) in an adult: a case report and review of the literature. Eur Arch Otorhinolaryngol. 2004 Jul;261(6):326-30. Epub 2003 Oct 10. [abstract]
- DermNet NZ; Langerhans cell histiocytosis. Updated March 2008.
- Shahla A, Parvaneh V, Hossein HD; Langerhans cells histiocytosis in one family. Pediatr Hematol Oncol. 2004 Jun;21(4):313-20. [abstract]
- Kanold J, Vannier JP, Fusade T, et al; Langerhans-cell histiocytosis in twin sisters. Arch Pediatr. 1994 Jan;1(1):49-53. [abstract]
- Stull MA, Kransdorf MJ, Devaney KO; Langerhans cell histiocytosis of bone. Radiographics. 1992 Jul;12(4):801-23. [abstract]
- Selim A, Shea R; Langerhans Cell Histiocytosis. eMedicine, February 2007.
- Satter EK, High WA; Langerhans cell histiocytosis: a case report and summary of the current recommendations of the Histiocyte Society. Dermatol Online J. 2008 Mar 15;14(3):3. [abstract]
- Textbook of Paediatrics, 6th Edition Forfar and Arneil 2003 Churchill Livingstone ISBN 0443071926
- Larralde M, Rositto A, Giardelli M, et al; Congenital self-healing histiocytosis (Hashimoto-Pritzker). Int J Dermatol. 1999 Sep;38(9):693-6. [abstract]
- Howarth DM, Gilchrist GS, Mullan BP, et al; Langerhans cell histiocytosis: diagnosis, natural history, management, and outcome. Cancer. 1999 May 15;85(10):2278-90. [abstract]
Internet and further reading
- Satter EK, High WA. Langerhans Cell Histiocytosis: A case report and summary of the current recommendations of the Histiocyte Society. Dermatol Online J. 2008 Mar 15;14(3):3
DocID: 847
Document Version: 21
DocRef: bgp1383
Last Updated: 20 Nov 2008
Review Date: 20 Nov 2010
The authors and editors of this article are employed to create accurate and up to date content reflecting reliable research evidence, guidance and best clinical practice. They are free from any commercial conflicts of interest. Find out more about updating.
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