Hand-Schuller-Christian diseaseD96.5

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 26.10.2022

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HistoryThis section has been translated automatically.

Hand 1893; Schüller 1915; Christian 1920

DefinitionThis section has been translated automatically.

Disease belonging to the Langerhans cell histiocytoses with granulomatous growths on the skeleton, skull, orbit (exophthalmos) and pituitary region(diabetes insipidus).

ManifestationThis section has been translated automatically.

Mostly occurring in childhood.

LocalizationThis section has been translated automatically.

Mainly capillitium, neck, armpits, flanks, eyelids, mucous membranes (oral cavity, respiratory tract, genitals)

Clinical featuresThis section has been translated automatically.

Skin lesions in 30% of cases. Polymorphous picture reminiscent of seborrheic eczema. Dry, pale brownish skin, petechiae, disseminated to tuberous infiltrates covered with scales or crusts, disseminated xanthomas, unilateral or bilateral exophthalmos, diabetes insipidus. X-rays of the skull show multiple, bony skull defects.

HistologyThis section has been translated automatically.

Reticulohistiocytic granulation tissue: Histiocytes, giant cells, plasma cells, lymphocytes, eosinophil granulocytes, Langerhans cells and foam cells.

TherapyThis section has been translated automatically.

By internists or pediatrics. No standardized therapy known.

External therapyThis section has been translated automatically.

Symptomatic therapy of skin symptoms according to clinic. Inflammatory sites with glucocorticoids such as 0.1% triamcinolone acetonide cream(Triamgalen, R259). For superinfection, antiseptic externals such as polihexanide (Serasept, Prontoderm) or 10% polyvidone iodine solution R203. For hyperkeratotic skin lesions, try keratolytic ointments such as 3% salicylic acid ointment(e.g., Salicylvaseline Lichtenstein, R228 ) or oil. Monitor for bacterial and mycotic secondary infections, especially if intertriginous spaces are affected.

Radiation therapyThis section has been translated automatically.

Isolated skin foci can be treated with soft tissue X-rays if necessary.

Internal therapyThis section has been translated automatically.

For early childhood, relatively good experience has been reported with the use of a glucocorticoid-cytostatic combination. Prednisolone (Decortin H) 2 mg/kg bw/day combined with cytostatic drugs (e.g. vincristine, see also cytostatic drugs). In adults, initially rather monotherapy with high-dose glucocorticoids, e.g. prednisone 2 mg/kg bw/day. If necessary, cytostatic monotherapy or combination therapy with agents such as vinblastine, methotrexate, 6-mercaptopurine or cyclophosphamide. If there is no improvement, glucocorticoids can be used in combination with vinblastine or, if necessary, bone marrow transplantation. The life-prolonging effect of cytostatic treatment is not certain, good response to glucocorticoid therapy, also response to short-duration and low-dose radiotherapy. Adiuretin can be used in the presence of diabetes insipidus .

Operative therapieThis section has been translated automatically.

Isolated skin foci can be excised. In case of multiple skin lesions, PUVA therapy or an experiment with 20% mechloroethamine may be possible.

LiteratureThis section has been translated automatically.

  1. Becelli R et al (2002) A rare condition of Hand-Schuller-Christian disease. J Craniofac Surgery 13: 759-761
  2. Chatelain R et al (1994) Langerhans cell histiocytosis (Hand-Schüller-Christian disease). Progress in practical dermatology and venereology 1994: 372-375
  3. Christian H (1919) Defects in membranous bones, exophthalmos, and diabetes insipidus; an unusual syndrome of dyspituitarism In: Contributions to medical and biological research, dedicated to Sir William Osler. New York, P. B. Hoeber 1: 390-401
  4. Christian H (1920) Defects in membranous bones, exophthalmos, and diabetes insipidus; an unusual syndrome of dyspituitarism. Medical Clinics of North America (Philadelphia) 3: 849-871
  5. Hand A (1893) Polyuria and tuberculosis. Proceedings of the Pathological Society of Philadelphia 16: 282-284 Archives of Pediatrics (New York) 10: 673-675
  6. Rowland RS (1928) Xanthomatosis and the reticuloendothelial system. Correlation of an unidentified group of cases described as defects in mambraneous bones, exophthalmos and diabetes insipidus (Christian's syndrome). Archives of Internal Medicine (Chicago) 42: 611-674
  7. Schüller A (1915) On peculiar skull defects in adolescence (map skull). Progress in the field of X-rays 23: 12-18
  8. Schüller A (1926) Dysostosis hypophysaria. British Journal of Radiology (London) 31: 156-158
  9. Smith T (1865) Skull-cap showing congenital deficiency of bone. Transactions of the Pathological Society of London 16: 224-225
  10. Schultze A (1999) Oral manifestations of Langerhans' cell histiocytosis. Therapeutic strategies involving oral and maxillofacial surgery. Oral maxillofacial screen 3: 158-164
  11. Sundar KM et al (2003) Pulmonary Langerhans cell histiocytosis: emerging concepts in pathobiology, radiology, and clinical evolution of disease. Chest 123: 1673-1683
  12. Vassallo R et al (2002) Clinical outcomes of pulmonary Langerhans'-cell histiocytosis in adults. N Engl J Med 346: 484-490

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Last updated on: 26.10.2022