Paraneoplastic multicentric reticulohistiocytosis: a clinicopathologic challenge

Indian J Dermatol Venereol Leprol. 2011 May-Jun;77(3):318-20. doi: 10.4103/0378-6323.79704.

Abstract

Multicentric Reticulohistiocytosis (MRH) is a rare, systemic non-Langerhans cell histiocytosis (non-LCH) with prominent joint and skin manifestations. It is mostly self limiting. However, 15-30% of the cases are associated with malignancy and carry a poor prognosis. We report the case of a 42-year-old man who presented with multiple reddish-brown papules that on biopsy showed aggregates of oncocytic histiocytes with several multinucleate giant cells. Immunostains were positive for CD 68, CD 45 and were negative for S-100, CD1a. An impression of multicentric reticulohistiocytosis (MRH) was made, with the recommendation to screen for malignancy. Electron microscopy of the skin lesions showed features consistent with non-Langerhans cell histiocytosis. The patient was later diagnosed with acute myeloid leukemia at a follow-up visit several months later. Thus, it appears prudent to screen and follow-up adults with MRH, to identify an underlying malignant condition.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Biopsy
  • Diagnosis, Differential
  • Histiocytosis, Non-Langerhans-Cell / pathology*
  • Humans
  • Leukemia, Myeloid, Acute / complications*
  • Male
  • Paraneoplastic Syndromes / etiology*
  • Paraneoplastic Syndromes / pathology*