Granulomatous lesions affecting the reticuloendothelial system produce a group of disorders having certain striking clinical features. The condition known as Hand-Schuller-Christian disease is one of this group, and the well-known SchiillerChristian triad of exophthalmos, diabetes insipidus, and multiple lesions in the skull was for many years considered to be pathognomonic. More recently Otani...

A 1-year-old boy presented with recurrent pyoderma-like lesions and purulent ear discharge of 6 months duration. The biopsy helped to confirm Letterer Siwe disease. Purpuric lesion on palms and soles which is a reputedly lethal sign of the disease was also present in the child who died within a few days.

In 1933 Siwe laid down the criteria of the syndrome now called Letterer-Siwe disease, and in the literature before and after that date the different features are discussed and related to the reported cases. The following case illustrates in particular the peripheral blood changes which may occur in this condition, and a table is appended summarizing the clinical and pathological features of 18 ...

Langerhans cell histiocytosis (LCH) as a term encompasses eosinophilic granuloma and two clinical syndromes: Letterer-Siwe disease and Hand-Schüller-Christian. All these syndromes seem to represent similar processes in which the proliferating cells have the structural and functional features of Langerhans cells. In reviewing the literature found that this disease does indeed occur in some famil...