Letterer-Siwe syndrome

Epidemiology: 1) children & young adults 2) predominance of males Pathology: Multiple sites, multiple organ systems. Clinical manifestations: 1) systemic illness resembling an infection or malignancy 2) hepatosplenomegaly 3) petechiae 4) purpura 5) generalized skin eruption Management: 1) prognosis: a) commonly fulminant & fatal b) spontaneous remissions uncommon c) worst prognosis is in the very young, with multifocal involvement & organ dysfunction 2) pharmaceutical agents a) vinblastine b) combination chemotherapy

General

genetic syndrome (multisystem disorder) Langerhans cell histiocytosis (LCH); histiocytosis X malignant histiocytosis; true histiocytic lymphoma; reticulosarcoma; histiocytic medullary reticulosis

References

Color Atlas & Synopsis of Clinical Dermatology, Common & Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY, 1997, pg 556-561