Multicentric Reticulohistiocytosis

 SCR presents as single papular or nodular cutaneous lesion


image GCR is characterized by eruption of multiple small cutaneous lesions


• Juvenile xanthogranuloma
image Typically single lesion occurring in children but can be multiple, & some cases occur in adults

• Langerhans cell histiocytosis
image Strongly positive for S100 & CD1a



image
Low Magnification of Multicentric Reticulohistiocytosis
Low magnification of multicentric reticulohistiocytosis (MCR) shows a nodular proliferation of enlarged histiocytic cells in the dermis. There is a very thin grenz zone image, and the epidermis shows flattening of the rete ridges.


image
Higher Magnification of Multicentric reticulohistiocytosis
Higher magnification of MCR shows a proliferation of numerous, enlarged, histiocytic-appearing cells image associated with a background mixed inflammatory infiltrate containing scattered eosinophils image.

image
Multicentric Reticulohistiocytosis With Large Multinucleated Cell
High magnification of a cellular MCR shows a very large, central multinucleated cell containing fragments of phagocytosed erythrocytes image.

image
Hypocellular Variant of Multicentric Reticulohistiocytosis
A hypocellular example of MCR shows a superficial, dermal-based proliferation of large epithelioid histiocytes image, many of which are multinucleated, and a mild associated lymphocytic infiltrate image.


TERMINOLOGY


Abbreviations




• Multicentric reticulohistiocytosis (MCR)


Synonyms




• Giant cell reticulohistiocytosis

• Reticulohistiocytosis of skin & synovia


Definitions




• Proliferation of large histiocytes with dense eosinophilic cytoplasm involving multiple organs


ETIOLOGY/PATHOGENESIS


Associated Conditions




• Autoimmune disorders & internal malignancies


CLINICAL ISSUES


Epidemiology




• Age
image Most cases occur in adults > 40 years


Site




• Can affect multiple sites, including hands, face, juxtaarticular regions, oral, nasal/pharyngeal mucosa


Presentation




• Multiple nodules & arthropathy
image Most cases present with multiple cutaneous/mucocutaneous papulonodules & severe arthropathy, other visceral symptoms

Only gold members can continue reading. Log In or Register to continue

Related posts:

  1. Inverted Type A (Clonal) Nevi
  2. Pigmented Spindle Cell Nevus (Reed Nevus)
  3. Spindle Cell/Pleomorphic Lipoma
  4. Lymphoepithelioma-Like Carcinoma of Skin

Stay updated, free articles. Join our Telegram channel
Tags:
Apr 24, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Multicentric Reticulohistiocytosis

Full access? Get Clinical Tree
Get Clinical Tree app for offline access