Letterer-Siwe Disease: Report of A Case.
- Author:
Chang Jo KOH
;
Chung Koo CHO
- Publication Type:Original Article
- MeSH:
Adrenal Cortex Hormones;
Anemia;
Biopsy;
Bone Marrow;
Dermatitis, Seborrheic;
Diarrhea;
Edema;
Eosinophilic Granuloma;
Extremities;
Histiocytosis, Langerhans-Cell*;
Humans;
Infant;
Male;
Prognosis;
Skin;
Skin Tests;
Skull;
Thrombocytopenia
- From:Korean Journal of Dermatology
1975;13(2):139-143
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Letterer-Siwe disease is a non-lipid reticuloendotheliosis of unknown etiology and usually grouped with Hand-Schuller-Christian disease and eosinophilic granuloma as components of histiocytosis-X. It occurs mostly in infancy and, clinically it is the most generalliaed and serious. In recent years, the prognosis of infants with Letterer-Siwe disease has improved, primarily due to more aggressive therapy with corticosteroids and antimetabolic agents. The 14 months old male patient was admitted into the Severance Hospital on 15th, July 1974 for evaluation of a chronic and refractory skin eruptions, abdominal distension with diarrhea and edema on extremities. He had been well until 3 months old age when he developed seborrhea-like skin eruptions which did not improve on treatment for seborrhea. Vigorous treatment was started for the baby from the beginning of admission day. Routine laboratory tests, skin biopsy, bone marrow aspiration and radiologic studies for bony structures wer done. All those studies had showed severe anemia, thrombocytopenia, multiple bony defects in skull and numerous non-lipid histiocytic infiltration in skin and bone marrow all those were consist with Letterer-Siwe disease.
