Cutaneous Letterer-Siwe Disease
- 1 February 1969
- journal article
- research article
- Published by American Medical Association (AMA) in Archives of Pediatrics & Adolescent Medicine
- Vol. 117 (2) , 236-238
- https://doi.org/10.1001/archpedi.1969.02100030238022
Abstract
Letterer-Siwedisease is a nonlipid reticuloendotheliosis of unknown etiology and is usually grouped with Hand-Schüller-Christian disease and eosinophilic granuloma as components of histiocytosis X.1It typically occurs in infancy and, clinically, is the most generalized and serious of these conditions.2In recent years, the prognosis of infants with Letterer-Siwe disease has improved, primarily due to more aggressive therapy with corticosteroids and antimetabolic agents.3,4The patient described below is of interest because of her prolonged course and because involvement has apparently been confined to the skin. Report of a Case The patient (JHH No. 121-91-97) was admitted to The Johns Hopkins Hospital in October 1966 for evaluation of a chronic and refractory skin eruption. She had been well until 8 months of age when she developed "cradle cap" which did not improve on treatment for seborrhea. By 16 months of age, there was diffuse involvement of the neckKeywords
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