Generalized non-Langerhans cell histiocytosis: four cases illustrate a spectrum of disease

The proliferation of non-Langerhans cell histiocytes is a poorly understood process of unknown cause. Variation in the clinical features and/or histopathology of histiocytic proliferation has led to subclassification of the general category of non-Langerhans cell histiocytes. Although the current classification may provide some useful generalizations in regard to the anticipated clinical course, wide variability in presentation and outcome make this classification less than optimal when dealing with individual patients. The objectives of the study were to present four cases of generalized non-Langerhans cell histiocytosis.

Medical records and slides of four patients diagnosed with non-Langerhans cell histiocytosis at the Cleveland Clinic are reviewed.

The patients exhibit features of more than one subtype of non-Langerhans cell histiocytosis.

The overlap among the clinical and histologic features of the generalized cutaneous non-Langerhans cell histiocytic disorders suggests that they represent one disease entity with a wide spectrum of presentations rather than many distinct disorders.