Hand-Schuller-Christian disease


Hand-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


1. Sore Oral Mucosa 2. Skin Eruption


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.


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


* 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 o Local treatments, e.g. topical steroids, antiseptics or antibiotics; curettage or intralesional injection of steroids o Phototherapy (PUVA) o Topical nitrogen mustard (mechlorethamine) o Thalidomide * Where more than one system involved, steroids and/or chemotherapy: o For children, oral corticosteroids may be the first option5 o 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). Prognosis11