Hand-Schuller-Christian disease D96.5

Hand 1893; Schüller 1915; Christian 1920

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

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.

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.

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 .

1. Becelli R et al (2002) A rare condition of Hand-Schuller-Christian disease. J Craniofac Surgery 13: 759-761
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