Histiocytic medullary reticulosis is a clinicopathologic syndrome characterized by its acute onset and relentless progression to death within a few months. Major clinical features are fever, malaise, weakness, weight loss, lymphadenopathy, hepatosplenomegaly, jaundice, and purpura. Common laboratory findings are severe anemia, leukopenia, and thrombocytopenia. Cardinal pathologic features are systemized proliferation of atypical, neoplastic, erythrophagocytic histiocytes. Treatment is of no avail. Two cases of histiocytic medullary reticulosis, with papular and nodular skin lesions suggestive of lymphomatous infiltrates as early signs, are presented. Review of 83 previously reported cases indicates that 10% of cases may have these lesions, which can be an aid in diagnosis. Although there are similarities of histiocytic medullary reticulosis to Hodgkin's disease, Letterer-Siwe disease, and lymphomatous diseases, we think that histiocytic medullary reticulosis is a distinct clinicopathologic type of malignant histiocytosis.
Abele DC, Griffin TB. Histiocytic Medullary ReticulosisReport of Two Cases and Review of the Literature. Arch Dermatol. 1972;106(3):319-329. doi:10.1001/archderm.1972.01620120007002