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Indian Journal of Dermatology, Venereology and Leprology
Medknow Publications on behalf of The Indian Association of Dermatologists, Venereologists and Leprologists (IADVL)
ISSN: 0378-6323
EISSN: 0973-3922
Vol. 77, No. 3, 2011, pp. 318-320
Bioline Code: dv11092
Full paper language: English
Document type: Case Report
Document available free of charge

Indian Journal of Dermatology, Venereology and Leprology, Vol. 77, No. 3, 2011, pp. 318-320

 en Paraneoplastic multicentric reticulohistiocytosis: A clinicopathologic challenge
Tirumalae, Rajalakshmi; Rout, Pritilata; Jayaseelan, Elizabeth; Shet, Arun; Devi, Shanthala & Kumar, Karuna Ramesh

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.

Keywords
Multicentric reticulohistiocytosis, non-Langerhans cell histiocytosis, paraneoplastic syndrome

 
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