Clinical, morphological, ultrastructural and immunological studies were performed in a case of congenital self-healing non-Langerhans cell histiocytosis. The patient showed several aspects that have not been published before: a large nodule in the vulvar region, vesiculobullous elements and pneumoni
Spontaneous healing of Langerhans cell histiocytosis (histiocytosis X)
โ Scribed by L. Corbeel; E. Eggermont; J. Desmyter; I. Surmont; R. Vos; C. Wolf-Peeters; C. Cobbaert; A. Eykens
- Publisher
- Springer
- Year
- 1988
- Tongue
- English
- Weight
- 278 KB
- Volume
- 148
- Category
- Article
- ISSN
- 0340-6997
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โฆ Synopsis
A 3-month-old male infant presented with pallor, hepatomegaly (4.5 cm), splenomegaly (1.5 cm), anaemia (Hb 6 g/dl) and thrombocytopenia (16 X 10(9)/l). A liver biopsy was diagnostic for Langerhans cell histiocytosis (histiocytosis X). The patient's lymphocytes, co-cultured with neonatal lymphocytes, were positive for virus-like particles without reverse transcriptase activity. The hepatomegaly diminished after 6 months and a second liver biopsy showed decreased histiocytic infiltration. A second viral blood culture remained negative. After 14 months, the hepatomegaly had disappeared completely and there were no more abnormal haematological or clinical findings.
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## SUMMARY Langerhans cell histiocytosis is a rare condition that can affect any organ of the body. Patients of all ages may present to the dermatologist and it is important to make the diagnosis as quickly as possible, because time from presentation to diagnosis is of prognostic importance in adul