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References in periodicals archive ?
LCH encompasses a spectrum of clinical manifestations ranging from a solitary chronic lesion, known as an eosinophilic granuloma, to a fulminant multisystem process called Letterer-Siwe disease. An intermediate form, Hand-Shuller-Christian disease, is composed of the triad of calvarial lesions, diabetes insipidus, and exophthalmos.
In the past, LCH was subdivided into three categories: Letterer-Siwe disease (acute disseminated disease), Hand-Schuller Christian disease (multifocal or uni-focal disease), and Eosinophilic granuloma (usually uni-focal disease) (Table).
In Letterer-Siwe disease, there is multisystem involvement.
SAN DIEGO -- The most aggressive variant of Langerhans cell histiocytosis, Letterer-Siwe disease is characterized by cutaneous involvement, organomegaly, and thrombocytopenia, Joseph P.