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.

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...

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...

The lungs may be involved in patients with histiocytosis X as part of the generalised disease, but histiocytosis X confined to the lungs without extrapulmonary involvement is rare. This report describes two cases of primary pulmonary histiocytosis arising in patients with Hodgkin's disease. It is impossible to state with certainty whether the pulmonary histiocytosis arose as a response to the d...

eosinophilic granuloma,8,9 Hand-Schuller-Christian10-12 and (Abt)-Letterer-Siwe13-15 diseases were not independent diseases. On the contrary, he felt that they shared pathogenesis and enough clinical aspects to be encompassed under the term histiocytosis X.16 This was a breakthrough and the first step towards understanding that LCH is a unique nosologic entity with heterogeneous clinical manife...