Multicentric reticulohistiocytosis: a unique case with pulmonary fibrosis.

Published

Journal Article

BACKGROUND: Multicentric reticulohistiocytosis (MRH) is a rare disease of uncertain etiology that most commonly presents as a papulonodular cutaneous eruption accompanied by erosive polyarthritis. Although MRH is considered a systemic disorder in that it targets skin and joints, involvement of thoracic and visceral organs is uncommon. OBSERVATIONS: A woman presented with diffuse cutaneous nodules, and skin biopsy findings revealed classic features of MRH. However, she also manifested severe pulmonary symptoms. A lung biopsy specimen showed prominent histiocytic infiltrates exhibiting the same characteristic morphologic features as those seen in her skin. Furthermore, the lung biopsy findings were significant for a pattern of usual interstitial pneumonia accompanied by notable lymphoid aggregates, a pattern of interstitial lung disease typical of systemic autoimmune and inflammatory conditions. CONCLUSIONS: These findings are notable because a histiocytic pulmonary infiltrate suggestive of direct pulmonary involvement by MRH is a rare event. In addition, presentation of MRH in the setting of usual interstitial pneumonia is unique. These observations document a new clinical and histopathologic presentation of MRH that is significant for expanding the idea of MRH as a systemic disease while supporting the notion that MRH is promoted by an inflammatory milieu.

Full Text

Duke Authors

Cited Authors

  • West, KL; Sporn, T; Puri, PK

Published Date

  • February 2012

Published In

Volume / Issue

  • 148 / 2

Start / End Page

  • 228 - 232

PubMed ID

  • 22351825

Pubmed Central ID

  • 22351825

Electronic International Standard Serial Number (EISSN)

  • 1538-3652

Digital Object Identifier (DOI)

  • 10.1001/archdermatol.2011.1331

Language

  • eng

Conference Location

  • United States