Background Multicentric reticulohistiocytosis (MR) is a rare histiocytic systemic disease mainly affecting the skin and synovia.
Objective To present the main and newest aspects of MR.
Method A review of the current medical literature, composing a retrospective study of 96 case reports published world-wide since 1977.
Results Facial and hand skin nodules with symmetric arthritis were the most frequent clinical presentation. The pathognomonic sign of ‘coral beads’ and vermicular erythematous lesions bordering nostrils, seems to be very characteristic of MR. There is a significant association with cancer. Histiocytic cells of MR are best characterized on immunohistochemistry by its immunoreactivity for vimentin, CD68 and CD45 and non-reactivity for S-100 protein, CD34 and factor XIIIa. Although the outcome of the disease is usually very limiting for the patients, treatment with immunosuppressive drugs may be useful.
Conclusion MR is a unique histiocytic proliferative disease with a peculiar immunophenotypic pattern and distinctive clinical and histopathological aspects.